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954_11 | The Fighter Staff committee was instrumental in bringing about the increased exploitation of slave labor in the war economy. The SS provided 64,000 prisoners for 20 separate projects from various concentration camps including Mittelbau-Dora. Prisoners worked for Junkers, Messerschmitt, Henschel and BMW, among others. To increase production, Speer introduced a system of punishments for his workforce. Those who feigned illness, slacked off, sabotaged production or tried to escape were denied food or sent to concentration camps. In 1944, this became endemic; over half a million workers were arrested. By this time, 140,000 people were working in Speer's underground factories. These factories were death-traps; discipline was brutal, with regular executions. There were so many corpses at the Dora underground factory, for example, that the crematorium was overwhelmed. Speer's own staff described the conditions there as "hell".
The largest technological advance under Speer's command came through the rocket program. It began in 1932 but had not supplied any weaponry. Speer enthusiastically supported the program and in March 1942 made an order for A4 rockets, the predecessor of the world's first ballistic missile, the V-2 rocket. The rockets were researched at a facility in Peenemünde along with the V-1 flying bomb. The V-2's first target was Paris on 8 September 1944. The program while advanced proved to be an impediment to the war economy. The large capital investment was not repaid in military effectiveness. The rockets were built at an underground factory at Mittelwerk. Labor to build the A4 rockets came from the Mittelbau-Dora concentration camp. Of the 60,000 people who ended up at the camp 20,000 died, due to the appalling conditions.
On 14 April 1944, Speer lost control of Organisation Todt to his Deputy, Franz Xaver Dorsch. He opposed the assassination attempt against Hitler on 20 July 1944. He was not involved in the plot, and played a minor role in the regime's efforts to regain control over Berlin after Hitler survived. After the plot Speer's rivals attacked some of his closest allies and his management system fell out of favor with radicals in the party. He lost yet more authority.
Defeat of Nazi Germany | [
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954_12 | Defeat of Nazi Germany
Losses of territory and a dramatic expansion of the Allied strategic bombing campaign caused the collapse of the German economy from late 1944. Air attacks on the transport network were particularly effective, as they cut the main centres of production off from essential coal supplies. In January 1945, Speer told Goebbels that armaments production could be sustained for at least a year. However, he concluded that the war was lost after Soviet forces captured the important Silesian industrial region later that month. Nevertheless, Speer believed that Germany should continue the war for as long as possible with the goal of winning better conditions from the Allies than the unconditional surrender they insisted upon. During January and February, Speer claimed that his ministry would deliver "decisive weapons" and a large increase in armaments production which would "bring about a dramatic change on the battlefield". Speer gained control over the railways in February, and asked Heinrich Himmler to supply concentration camp prisoners to work on their repair.
By mid-March, Speer had accepted that Germany's economy would collapse within the next eight weeks. While he sought to frustrate directives to destroy industrial facilities in areas at risk of capture, so that they could be used after the war, he still supported the war's continuation. Speer provided Hitler with a memorandum on 15 March, which detailed Germany's dire economic situation and sought approval to cease demolitions of infrastructure. Three days later, he also proposed to Hitler that Germany's remaining military resources be concentrated along the Rhine and Vistula rivers in an attempt to prolong the fighting. This ignored military realities, as the German armed forces were unable to match the Allies' firepower and were facing total defeat. Hitler rejected Speer's proposal to cease demolitions. Instead, he issued the "Nero Decree" on 19 March, which called for the destruction of all infrastructure as the army retreated. Speer was appalled by this order, and persuaded several key military and political leaders to ignore it. During a meeting with Speer on 28/29 March, Hitler rescinded the decree and gave him authority over demolitions. Speer ended them, though the army continued to blow up bridges. | [
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954_13 | By April, little was left of the armaments industry, and Speer had few official duties. Speer visited the Führerbunker on 22 April for the last time. He met Hitler and toured the damaged Chancellery before leaving Berlin to return to Hamburg. On 29 April, the day before committing suicide, Hitler dictated a final political testament which dropped Speer from the successor government. Speer was to be replaced by his subordinate, Karl-Otto Saur. Speer was disappointed that Hitler had not selected him as his successor. After Hitler's death, Speer offered his services to the so-called Flensburg Government, headed by Hitler's successor, Karl Dönitz. He took a role in that short-lived regime as Minister of Industry and Production. Speer provided information to the Allies, regarding the effects of the air war, and on a broad range of subjects, beginning on 10 May. On 23 May, two weeks after the surrender of German forces, British troops arrested the members of the Flensburg Government and brought Nazi Germany to a formal end.
Post-war
Nuremberg trial
Speer was taken to several internment centres for Nazi officials and interrogated. In September 1945, he was told that he would be tried for war crimes, and several days later, he was moved to Nuremberg and incarcerated there. Speer was indicted on four counts: participating in a common plan or conspiracy for the accomplishment of crime against peace; planning, initiating and waging wars of aggression and other crimes against peace; war crimes; and crimes against humanity.
The chief United States prosecutor, Robert H. Jackson, of the U.S. Supreme Court said, "Speer joined in planning and executing the program to dragoon prisoners of war and foreign workers into German war industries, which waxed in output while the workers waned in starvation." Speer's attorney, Hans Flächsner, presented Speer as an artist thrust into political life who had always remained a non-ideologue. | [
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954_14 | Speer was found guilty of war crimes and crimes against humanity, principally for the use of slave labor and forced labor. He was acquitted on the other two counts. He had claimed that he was unaware of Nazi extermination plans, and the Allies had no proof that he was aware. His claim was revealed to be false in a private correspondence written in 1971 and publicly disclosed in 2007. On 1 October 1946, he was sentenced to 20 years' imprisonment. While three of the eight judges (two Soviet and American Francis Biddle) advocated the death penalty for Speer, the other judges did not, and a compromise sentence was reached after two days of discussions.
Imprisonment
On 18 July 1947, Speer was transferred to Spandau Prison in Berlin to serve his prison term. There he was known as Prisoner Number Five. Speer's parents died while he was incarcerated. His father, who died in 1947, despised the Nazis and was silent upon meeting Hitler. His mother died in 1952. As a Nazi Party member, she had greatly enjoyed dining with Hitler. Wolters and longtime Speer secretary Annemarie Kempf, while not permitted direct communication with Speer in Spandau, did what they could to help his family and carry out the requests Speer put in letters to his wife—the only written communication he was officially allowed. Beginning in 1948, Speer had the services of Toni Proost, a sympathetic Dutch orderly to smuggle mail and his writings.
In 1949, Wolters opened a bank account for Speer and began fundraising among those architects and industrialists who had benefited from Speer's activities during the war. Initially, the funds were used only to support Speer's family, but increasingly the money was used for other purposes. They paid for Toni Proost to go on holiday, and for bribes to those who might be able to secure Speer's release. Once Speer became aware of the existence of the fund, he sent detailed instructions about what to do with the money. Wolters raised a total of DM158,000 for Speer over the final seventeen years of his sentence. | [
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954_15 | The prisoners were forbidden to write memoirs. Speer was able to have his writings sent to Wolters, however, and they eventually amounted to 20,000 pages. He had completed his memoirs by November 1953, which became the basis of Inside the Third Reich. In Spandau Diaries, Speer aimed to present himself as a tragic hero who had made a Faustian bargain for which he endured a harsh prison sentence.
Much of Speer's energy was dedicated to keeping fit, both physically and mentally, during his long confinement. Spandau had a large enclosed yard where inmates were allocated plots of land for gardening. Speer created an elaborate garden complete with lawns, flower beds, shrubbery, and fruit trees. To make his daily walks around the garden more engaging Speer embarked on an imaginary trip around the globe. Carefully measuring distance travelled each day, he mapped distances to the real-world geography. He had walked more than , ending his sentence near Guadalajara, Mexico. Speer also read, studied architectural journals, and brushed up on English and French. In his writings, Speer claimed to have finished five thousand books while in prison, a gross exaggeration. His sentence amounted to 7,300 days, which only allotted one and a half days per book.
Speer's supporters maintained calls for his release. Among those who pledged support for his sentence to be commuted were Charles de Gaulle and US diplomat George Wildman Ball. Willy Brandt was an advocate of his release, putting an end to the de-Nazification proceedings against him, which could have caused his property to be confiscated. Speer's efforts for an early release came to naught. The Soviet Union, having demanded a death sentence at trial, was unwilling to entertain a reduced sentence. Speer served a full term and was released at midnight on 1 October 1966. | [
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954_16 | Release and later life
Speer's release from prison was a worldwide media event. Reporters and photographers crowded both the street outside Spandau and the lobby of the Hotel Berlin where Speer spent the night. He said little, reserving most comments for a major interview published in Der Spiegel in November 1966. Although he stated he hoped to resume an architectural career, his sole project, a collaboration for a brewery, was unsuccessful. Instead, he revised his Spandau writings into two autobiographical books, and later published a work about Himmler and the SS. His books included Inside the Third Reich (in German, Erinnerungen, or Reminiscences) and Spandau: The Secret Diaries. Speer was aided in shaping the works by Joachim Fest and Wolf Jobst Siedler from the publishing house Ullstein. He found himself unable to re-establish a relationship with his children, even with his son Albert who had also become an architect. According to Speer's daughter Hilde Schramm, "One by one my sister and brothers gave up. There was no communication." He supported Hermann, his brother, financially after the war. However, his other brother Ernst had died in the Battle of Stalingrad, despite repeated requests from his parents for Speer to repatriate him.
Following his release from Spandau, Speer donated the Chronicle, his personal diary, to the German Federal Archives. It had been edited by Wolters and made no mention of the Jews. David Irving discovered discrepancies between the deceptively edited Chronicle and independent documents. Speer asked Wolters to destroy the material he had omitted from his donation but Wolters refused and retained an original copy. Wolters' friendship with Speer deteriorated and one year before Speer's death Wolters gave Matthias Schmidt access to the unedited Chronicle. Schmidt authored the first book that was highly critical of Speer. | [
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954_17 | Speer's memoirs were a phenomenal success. The public was fascinated by an inside view of the Third Reich and a major war criminal became a popular figure almost overnight. Importantly, he provided an alibi to older Germans who had been Nazis. If Speer, who had been so close to Hitler, had not known the full extent of the crimes of the Nazi regime and had just been "following orders", then they could tell themselves and others they too had done the same. So great was the need to believe this "Speer Myth" that Fest and Siedler were able to strengthen it—even in the face of mounting historical evidence to the contrary.
Death
Speer made himself widely available to historians and other enquirers. In October 1973, he made his first trip to Britain, flying to London to be interviewed on the BBC Midweek programme. In the same year, he appeared on the television programme The World at War. Speer returned to London in 1981 to participate in the BBC Newsnight programme. He suffered a stroke and died in London on 1 September.
He had remained married to his wife, but he had formed a relationship with a German woman living in London and was with her at the time of his death. His daughter, Margret Nissen, wrote in her 2005 memoirs that after his release from Spandau he spent all of his time constructing the "Speer Myth".
The Speer myth | [
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] |
954_18 | The Speer myth
The Good Nazi
After his release from Spandau, Speer portrayed himself as the "good Nazi". He was well-educated, middle class, and bourgeois, and could contrast himself with those who, in the popular mind, typified "bad Nazis". In his memoirs and interviews, he had distorted the truth and made so many major omissions that his lies became known as "myths". Speer took his myth-making to a mass media level and his "cunning apologies" were reproduced countless times in post-war Germany. Isabell Trommer writes in her biography of Speer that Fest and Siedler were co-authors of Speer's memoirs and co-creators of his myths. In return they were paid handsomely in royalties and other financial inducements. Speer, Siedler and Fest had constructed a masterpiece; the image of the "good Nazi" remained in place for decades, despite historical evidence indicating that it was false.
Speer had carefully constructed an image of himself as an apolitical technocrat who deeply regretted having failed to discover the monstrous crimes of the Third Reich. This construction was accepted almost at face value by historian Hugh Trevor-Roper when investigating the death of Adolf Hitler for British Intelligence and in writing The Last Days of Hitler. Trevor-Roper frequently refers to Speer as "a technocrat [who] nourished a technocrat's philosophy", one who cared only for his building projects or his ministerial duties, and who thought that politics was irrelevant, at least until Hitler's Nero Decree which Speer, according to his own telling, worked assiduously to counter. Trevor-Roper – who calls Speer an administrative genius whose basic instincts were peaceful and constructive – does take Speer to task, however, for his failure to recognize the immorality of Hitler and Nazism, calling him "the real criminal of Nazi Germany": | [
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954_19 | For ten years he sat at the very centre of political power; his keen intelligence diagnosed the nature and observed the mutations of Nazi government and policy; he saw and despised the personalities around him; he heard their outrageous orders and understood their fantastic ambitions; but he did nothing. Supposing politics to be irrelevant, he turned aside and built roads and bridges and factories, while the logical consequences of government by madmen emerged. Ultimately, when their emergence involved the ruin of all his work, Speer accepted the consequences and acted. Then it was too late; Germany had been destroyed.
After Speer's death, Matthias Schmidt published a book that demonstrated that Speer had ordered the eviction of Jews from their Berlin homes. By 1999, historians had amply demonstrated that he had lied extensively. Even so, public perceptions of Speer did not change substantially until Heinrich Breloer aired a biographical film on TV in 2004. The film began a process of demystification and critical reappraisal. Adam Tooze in his book The Wages of Destruction said Speer had manoeuvred himself through the ranks of the regime skillfully and ruthlessly and that the idea he was a technocrat blindly carrying out orders was "absurd". Trommer said he was not an apolitical technocrat; instead, he was one of the most powerful and unscrupulous leaders in the Nazi regime. Kitchen said he had deceived the Nuremberg Tribunal and post-war Germany. Brechtken said that if his extensive involvement in the Holocaust had been known at the time of his trial he would have been sentenced to death. | [
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954_20 | The image of the good Nazi was supported by numerous Speer myths. In addition to the myth that he was an apolitical technocrat, he claimed he did not have full knowledge of the Holocaust or the persecution of the Jews. Another myth posits that Speer revolutionized the German war machine after his appointment as Minister of Armaments. He was credited with a dramatic increase in the shipment of arms that was widely reported as keeping Germany in the war. Another myth centered around a faked plan to assassinate Hitler with poisonous gas. The idea for this myth came to him after he recalled the panic when car fumes came through an air ventilation system. He fabricated the additional details. Brechtken wrote that his most brazen lie was fabricated during an interview with a French journalist in 1952. The journalist described an invented scenario in which Speer had refused Hitler's orders and Hitler had left with tears in his eyes. Speer liked the scenario so much that he wrote it into his memoirs. The journalist had unwittingly collaborated in one of his myths.
Speer also sought to portray himself as an opponent of Hitler's leadership. Despite his opposition to the 20 July plot, he falsely claimed in his memoirs to have been sympathetic to the plotters. He maintained Hitler was cool towards him for the remainder of his life after learning they had included him on a list of potential ministers. This formed a key element of the myths Speer encouraged. Speer also falsely claimed that he had realised the war was lost at an early stage, and thereafter worked to preserve the resources needed for the civilian population's survival. In reality, he had sought to prolong the war until further resistance was impossible, thus contributing to the large number of deaths and the extensive destruction Germany suffered in the conflict's final months.
Denial of responsibility | [
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954_21 | Denial of responsibility
Speer maintained at the Nuremberg trials and in his memoirs that he had no direct knowledge of the Holocaust. He admitted only to being uncomfortable around Jews in the published version of the Spandau Diaries. More broadly, Speer accepted responsibility for the Nazi regime's actions. Historian Martin Kitchen states that Speer was actually "fully aware of what had happened to the Jews" and was "intimately involved in the 'Final Solution'". Brechtken said Speer only admitted to a generalized responsibility for the Holocaust to hide his direct and actual responsibility. Speer was photographed with slave laborers at Mauthausen concentration camp during a visit on 31 March 1943; he also visited Gusen concentration camp. Although survivor Francisco Boix testified at the Nuremberg trials about Speer's visit, Taylor writes that, had the photo been available, he would have been hanged. In 2005, The Daily Telegraph reported that documents had surfaced indicating that Speer had approved the allocation of materials for the expansion of Auschwitz concentration camp after two of his assistants inspected the facility on a day when almost a thousand Jews were massacred. Heinrich Breloer, discussing the construction of Auschwitz, said Speer was not just a cog in the work—he was the "terror itself".
Speer did not deny being present at the Posen speeches to Nazi leaders at a conference in Posen (Poznań) on 6 October 1943, but claimed to have left the auditorium before Himmler said during his speech: "The grave decision had to be taken to cause this people to vanish from the earth", and later, "The Jews must be exterminated". Speer is mentioned several times in the speech, and Himmler addresses him directly. In 2007, The Guardian reported that a letter from Speer dated 23 December 1971, had been found in a collection of his correspondence with Hélène Jeanty, the widow of a Belgian resistance fighter. In the letter, Speer says, "There is no doubt—I was present as Himmler announced on October 6, 1943, that all Jews would be killed."
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954_22 | Armaments "miracle"
Speer was credited with an "armaments miracle". During the winter of 1941–42, in the light of Germany's disastrous defeat in the Battle of Moscow, the German leadership including Friedrich Fromm, Georg Thomas and Fritz Todt had come to the conclusion that the war could not be won. The rational position to adopt was to seek a political solution that would end the war without defeat. Speer in response used his propaganda expertise to display a new dynamism of the war economy. He produced spectacular statistics, claiming a sixfold increase in munitions production, a fourfold increase in artillery production, and he sent further propaganda to the newsreels of the country. He was able to curtail the discussion that the war should be ended.
The armaments "miracle" was a myth; Speer had used statistical manipulation to support his claims. The production of armaments did go up; however, this was due to the normal causes of reorganization before Speer came to office, the relentless mobilization of slave labor and a deliberate reduction in the quality of output to favor quantity. By July 1943 Speer's armaments propaganda became irrelevant because a catalogue of dramatic defeats on the battlefield meant the prospect of losing the war could no longer be hidden from the German public. Brechtken writes that Speer knew Germany was going to lose the war and deliberately extended its length, thus causing the deaths of millions of people in the death camps and on the battlefield who would have otherwise lived. Kitchen said, "There can be no doubt that Speer did indeed help to prolong the war longer than many thought possible, as a result of which millions were killed and Germany reduced to a pile of rubble".
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954_23 | Architectural legacy
Little remains of Speer's personal architectural works, other than the plans and photographs. No buildings designed by Speer during the Nazi era are extant in Berlin, other than the 4 entrance pavilions and underpasses leading to the Victory Column or Siegessäule, and the Schwerbelastungskörper, a heavy load-bearing body built around 1941. The concrete cylinder, high, was used to measure ground subsidence as part of feasibility studies for a massive triumphal arch and other large structures proposed as part of Welthauptstadt Germania, Hitler's planned post-war renewal project for the city. The cylinder is now a protected landmark and is open to the public. The tribune of the Zeppelinfeld stadium in Nuremberg, though partly demolished, can also be seen.
During the war, the Speer-designed Reich Chancellery was largely destroyed by air raids and in the Battle of Berlin. The exterior walls survived, but they were eventually dismantled by the Soviets. Unsubstantiated rumors have claimed that the remains were used for other building projects such as the Humboldt University, Mohrenstraße metro station and Soviet war memorials in Berlin.
See also
Speer Goes to Hollywood
Downfall, 2004 German film where he was portrayed by actor Heino Ferch
Legion Speer
Transportflotte Speer
Transportkorps Speer
Hermann Giesler
References
Informational notes
Citations
Bibliography
Printed sources
Online sources
Further reading
Causey, Charles M. (2016). The Lion and the Lamb: The True Holocaust Story of a Powerful Nazi Leader and a Dutch Resistance Worker,
External links
Francisco Boix identifies Speer at Nuremberg
1905 births
1981 deaths
20th-century German architects
Articles containing video clips
Deaths from cerebrovascular disease
20th-century German male writers
German people convicted of crimes against humanity
Holocaust perpetrators
Karlsruhe Institute of Technology alumni
Members of the Reichstag of Nazi Germany
Architects in the Nazi Party
Nazi Germany ministers
Nazi Party officials
Neoclassical architects
Officials of Nazi Germany
People convicted by the International Military Tribunal in Nuremberg
Politicians from Mannheim
People from the Grand Duchy of Baden
Recipients of the Knights Cross of the War Merit Cross
Technical University of Berlin alumni
Technical University of Munich alumni
Architects from Mannheim
Neurological disease deaths in England | [
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956_0 | The family Asteraceae (), alternatively Compositae (), consists of over 32,000 known species of flowering plants in over 1,900 genera within the order Asterales. Commonly referred to as the aster, daisy, composite, or sunflower family, Compositae were first described in the year 1740. The number of species in Asteraceae is rivaled only by the Orchidaceae, and which is the larger family is unclear as the quantity of extant species in each family is unknown.
Most species of Asteraceae are annual, biennial, or perennial herbaceous plants, but there are also shrubs, vines, and trees. The family has a widespread distribution, from subpolar to tropical regions in a wide variety of habitats. Most occur in hot desert and cold or hot semi-desert climates, and they are found on every continent but Antarctica. The primary common characteristic is the existence of sometimes hundreds of tiny individual florets which are held together by protective involucres in flower heads, or more technically, capitula.
The oldest known fossils are pollen grains from the Late Cretaceous (Campanian to Maastrichtian) of Antarctica, dated to c. 76–66 million years (myr). It is estimated that the crown group of Asteraceae evolved at least 85.9 myr (Late Cretaceous, Santonian) with a stem node age of 88–89 myr (Late Cretaceous, Coniacian).
Asteraceae is an economically important family, providing food staples, garden plants, and herbal medicines. Species outside of their native ranges can be considered weedy or invasive.
Description
Members of the Asteraceae are mostly herbaceous plants, but some shrubs, vines, and trees (such as Lachanodes arborea) do exist. Asteraceae species are generally easy to distinguish from other plants because of their unique inflorescence and other shared characteristics, such as the joined anthers of the stamens. However, determining genera and species of some groups such as Hieracium is notoriously difficult (see "damned yellow composite" for example).
Roots
Members of the family Asteraceae generally produce taproots, but sometimes they possess fibrous root systems. Some species have underground stems in the form of caudices or rhizomes. These can be fleshy or woody depending on the species. | [
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956_1 | Stems
Stems are herbaceous, aerial, branched, and cylindrical with glandular hairs, generally erect, but can be prostrate to ascending. The stems can contain secretory canals with resin, or latex which is particularly common among the Cichorioideae.
Leaves
Leaves can be alternate, opposite, or whorled. They may be simple, but are often deeply lobed or otherwise incised, often conduplicate or revolute. The margins also can be entire or toothed. Resin or latex also can be present in the leaves.
Inflorescences
Nearly all Asteraceae bear their flowers in dense flower heads called capitula. They are surrounded by involucral bracts, and when viewed from a distance, each capitulum may appear to be a single flower. Enlarged outer (peripheral) flowers in the capitulum may resemble petals, and the involucral bracts may look like a calyx.
Floral heads
In plants of the family Asteraceae, what appears to be a single flower is actually a cluster of much smaller flowers. The overall appearance of the cluster, as a single flower, functions in attracting pollinators in the same way as the structure of an individual flower in some other plant families. The older family name, Compositae, comes from the fact that what appears to be a single flower is actually a composite of smaller flowers.
The "petals" or "sunrays" in a sunflower head are actually individual strap-shaped flowers called ray flowers, and the "sun disk" is made of smaller circular shaped individual flowers called disc flowers. The word "aster" means "star" in Greek, referring to the appearance of some family members, as a "star" surrounded by "rays". The cluster of flowers that may appear to be a single flower, is called a head. The entire head may move tracking the sun, like a "smart" solar panel, which maximizes reflectivity of the whole unit and can thereby attract more pollinators. | [
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956_2 | On the outside the flower heads are small bracts that look like scales. These are called phyllaries, and together they form the involucre that protects the individual flowers in the head before they open. The individual heads have the smaller individual flowers arranged on a round or dome-like structure called the receptacle. The flowers mature first at the outside, moving toward the center, with the youngest in the middle.
The individual flowers in a head have 5 fused petals (rarely 4), but instead of sepals, have threadlike, hairy, or bristly structures singularly called a pappus, plural pappi, which surround the fruit and can stick to animal fur or be lifted by wind, aiding in seed dispersal. The whitish fluffy head of a dandelion, commonly blown on by children, is made of pappi with tiny seeds attached at the ends. The pappi provide a parachute like structure to help the seed be carried away in the wind.
A ray flower is a 3-tipped (3-lobed), strap-shaped, individual flower in the head of some members of the family Asteraceae. Sometimes a ray flower is 2-tipped (2-lobed). The corolla of the ray flower may have 2 tiny teeth opposite the 3-lobed strap, or tongue, indicating evolution by fusion from an originally 5-part corolla. Sometimes, the 3:2 arrangement is reversed, with 2 tips on the tongue, and 0 or 3 tiny teeth opposite the tongue. A ligulate flower is a 5-tipped, strap-shaped, individual flower in the heads of other members. A ligule is the strap-shaped tongue of the corolla of either a ray flower or of a ligulate flower. A disk flower (or disc flower) is a radially symmetric (i.e., with identical shaped petals arranged in circle around the center) individual flower in the head, which is ringed by ray flowers when both are present. Sometimes ray flowers may be slightly off from radial symmetry, or weakly bilaterally symmetric, as in the case of desert pincushions Chaenactis fremontii. | [
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956_3 | A radiate head has disc flowers surrounded by ray flowers. A ligulate head has all ligulate flowers. When a sunflower family flower head has only disc flowers that are sterile, male, or have both male and female parts, it is a discoid head. Disciform heads have only disc flowers, but may have two kinds (male flowers and female flowers) in one head, or may have different heads of two kinds (all male, or all female). Pistillate heads have all female flowers. Staminate heads have all male flowers. Sometimes, but rarely, the head contains only a single flower, or has a single flowered pistillate (female) head, and a multi-flowered male staminate (male) head.
Floral structures
The distinguishing characteristic of Asteraceae is their inflorescence, a type of specialised, composite flower head or pseudanthium, technically called a calathium or capitulum, that may look superficially like a single flower. The capitulum is a contracted raceme composed of numerous individual sessile flowers, called florets, all sharing the same receptacle.
A set of bracts forms an involucre surrounding the base of the capitulum. These are called "phyllaries", or "involucral bracts". They may simulate the sepals of the pseudanthium. These are mostly herbaceous but can also be brightly coloured (e.g. Helichrysum) or have a scarious (dry and membranous) texture. The phyllaries can be free or fused, and arranged in one to many rows, overlapping like the tiles of a roof (imbricate) or not (this variation is important in identification of tribes and genera).
Each floret may be subtended by a bract, called a "palea" or "receptacular bract". These bracts are often called "chaff". The presence or absence of these bracts, their distribution on the receptacle, and their size and shape are all important diagnostic characteristics for genera and tribes. | [
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956_4 | The florets have five petals fused at the base to form a corolla tube and they may be either actinomorphic or zygomorphic. Disc florets are usually actinomorphic, with five petal lips on the rim of the corolla tube. The petal lips may be either very short, or long, in which case they form deeply lobed petals. The latter is the only kind of floret in the Carduoideae, while the first kind is more widespread. Ray florets are always highly zygomorphic and are characterised by the presence of a ligule, a strap-shaped structure on the edge of the corolla tube consisting of fused petals. In the Asteroideae and other minor subfamilies these are usually borne only on florets at the circumference of the capitulum and have a 3+2 scheme – above the fused corolla tube, three very long fused petals form the ligule, with the other two petals being inconspicuously small. The Cichorioideae has only ray florets, with a 5+0 scheme – all five petals form the ligule. A 4+1 scheme is found in the Barnadesioideae. The tip of the ligule is often divided into teeth, each one representing a petal. Some marginal florets may have no petals at all (filiform floret).
The calyx of the florets may be absent, but when present is always modified into a pappus of two or more teeth, scales or bristles and this is often involved in the dispersion of the seeds. As with the bracts, the nature of the pappus is an important diagnostic feature.
There are usually five stamens. The filaments are fused to the corolla, while the anthers are generally connate (syngenesious anthers), thus forming a sort of tube around the style (theca). They commonly have basal and/or apical appendages. Pollen is released inside the tube and is collected around the growing style, and then, as the style elongates, is pushed out of the tube (nüdelspritze). | [
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956_5 | The pistil consists of two connate carpels. The style has two lobes. Stigmatic tissue may be located in the interior surface or form two lateral lines. The ovary is inferior and has only one ovule, with basal placentation.
Fruits and seeds
In members of the Asteraceae the fruit is achene-like, and is called a cypsela (plural cypselae). Although there are two fused carpels, there is only one locule, and only one seed per fruit is formed. It may sometimes be winged or spiny because the pappus, which is derived from calyx tissue often remains on the fruit (for example in dandelion). In some species, however, the pappus falls off (for example in Helianthus). Cypsela morphology is often used to help determine plant relationships at the genus and species level. The mature seeds usually have little endosperm or none.
Pollen
The pollen of composites is typically echinolophate, a morphological term meaning "with elaborate systems of ridges and spines dispersed around and between the apertures."
Metabolites
In Asteraceae, the energy store is generally in the form of inulin rather than starch. They produce iso/chlorogenic acid, sesquiterpene lactones, pentacyclic triterpene alcohols, various alkaloids, acetylenes (cyclic, aromatic, with vinyl end groups), tannins. They have terpenoid essential oils which never contain iridoids.
Asteraceae produce secondary metabolites, such as flavonoids and terpenoids. Some of these molecules can inhibit protozoan parasites such as Plasmodium, Trypanosoma, Leishmania and parasitic intestinal worms, and thus have potential in medicine.
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] |
956_6 | Taxonomy
History
Compositae, the original name for Asteraceae, were first described in 1740 by Dutch botanist Adriaan van Royen. Traditionally, two subfamilies were recognised: Asteroideae (or Tubuliflorae) and Cichorioideae (or Liguliflorae). The latter has been shown to be extensively paraphyletic, and has now been divided into 12 subfamilies, but the former still stands. The study of this family is known as synantherology.
Phylogeny
The phylogenetic tree presented below is based on Panero & Funk (2002) updated in 2014, and now also includes the monotypic Famatinanthoideae.
The diamond (♦) denotes a very poorly supported node (<50% bootstrap support), the dot (•) a poorly supported node (<80%).
The family includes over 32,000 currently accepted species, in over 1,900 genera (list) in 13 subfamilies. The number of species in the family Asteraceae is rivaled only by Orchidaceae. Which is the larger family is unclear, because of the uncertainty about how many extant species each family includes. The four subfamilies Asteroideae, Cichorioideae, Carduoideae and Mutisioideae contain 99% of the species diversity of the whole family (approximately 70%, 14%, 11% and 3% respectively).
Because of the morphological complexity exhibited by this family, agreeing on generic circumscriptions has often been difficult for taxonomists. As a result, several of these genera have required multiple revisions.
Paleontology and evolutionary processes
The oldest known fossils of members of Asteraceae are pollen grains from the Late Cretaceous of Antarctica, dated to ∼76–66 myr (Campanian to Maastrichtian) and assigned to the extant genus Dasyphyllum. Barreda, et al. (2015) estimated that the crown group of Asteraceae evolved at least 85.9 myr (Late Cretaceous, Santonian) with a stem node age of 88–89 myr (Late Cretaceous, Coniacian). | [
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956_7 | It is not known whether the precise cause of their great success was the development of the highly specialised capitulum, their ability to store energy as fructans (mainly inulin), which is an advantage in relatively dry zones, or some combination of these and possibly other factors. Heterocarpy, or the ability to produce different fruit morphs, has evolved and is common in Asteraceae. It allows seeds to be dispersed over varying distances and each is adapted to different environments, increasing chances of survival.
Etymology and pronunciation
The name Asteraceae () comes to international scientific vocabulary from New Latin, from Aster, the type genus, + -aceae, a standardized suffix for plant family names in modern taxonomy. The genus name comes from the Classical Latin word , "star", which came from Ancient Greek (), "star". It refers to the star-like form of the inflorescence.
The original name Compositae is still valid under the International Code of Nomenclature for algae, fungi, and plants. It refers to the "composite" nature of the capitula, which consist of a few or many individual flowers.
The vernacular name daisy, widely applied to members of this family, is derived from the Old English name of the daisy (Bellis perennis): , meaning "day's eye". This is because the petals open at dawn and close at dusk.
Distribution and habitat
Asteraceae species have a widespread distribution, from subpolar to tropical regions in a wide variety of habitats. Most occur in hot desert and cold or hot semi-desert climates, and they are found on every continent but Antarctica. They are especially numerous in tropical and subtropical regions (notably Central America, eastern Brazil, the Mediterranean, the Levant, southern Africa, central Asia, and southwestern China). The largest proportion of the species occur in the arid and semi-arid regions of subtropical and lower temperate latitudes. The Asteraceae family comprises 10% of all flowering plant species.
Ecology
Asteraceae are especially common in open and dry environments. Many members of Asteraceae are pollinated by insects, which explains their value in attracting beneficial insects, but anemophily is also present (e.g. Ambrosia, Artemisia). There are many apomictic species in the family. | [
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956_8 | Seeds are ordinarily dispersed intact with the fruiting body, the cypsela. Anemochory (wind dispersal) is common, assisted by a hairy pappus. Epizoochory is another common method, in which the dispersal unit, a single cypsela (e.g. Bidens) or entire capitulum (e.g. Arctium) has hooks, spines or some structure to attach to the fur or plumage (or even clothes, as in the photo) of an animal just to fall off later far from its mother plant.
Some members of Asteraceae are economically important as weeds. Notable in the United States are Senecio jacobaea (ragwort), Senecio vulgaris (groundsel), and Taraxacum (dandelion). Some are invasive species in particular regions, often having been introduced by human agency. Examples include various tumbleweeds, Bidens, ragweeds, thistles, and dandelion. Dandelion was introduced into North America by European settlers who used the young leaves as a salad green.
Uses
Asteraceae is an economically important family, providing products such as cooking oils, leaf vegetables like lettuce, sunflower seeds, artichokes, sweetening agents, coffee substitutes and herbal teas. Several genera are of horticultural importance, including pot marigold (Calendula officinalis), Echinacea (coneflowers), various daisies, fleabane, chrysanthemums, dahlias, zinnias, and heleniums. Asteraceae are important in herbal medicine, including Grindelia, yarrow, and many others.
Commercially important plants in Asteraceae include the food crops Lactuca sativa (lettuce), Cichorium (chicory), Cynara scolymus (globe artichoke), Helianthus annuus (sunflower), Smallanthus sonchifolius (yacón), Carthamus tinctorius (safflower) and Helianthus tuberosus (Jerusalem artichoke). | [
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956_9 | Plants are used as herbs and in herbal teas and other beverages. Chamomile, for example, comes from two different species: the annual Matricaria chamomilla (German chamomile) and the perennial Chamaemelum nobile (Roman chamomile). Calendula (known as pot marigold) is grown commercially for herbal teas and potpourri. Echinacea is used as a medicinal tea. The wormwood genus Artemisia includes absinthe (A. absinthium) and tarragon (A. dracunculus). Winter tarragon (Tagetes lucida), is commonly grown and used as a tarragon substitute in climates where tarragon will not survive.
Many members of the family are grown as ornamental plants for their flowers, and some are important ornamental crops for the cut flower industry. Some examples are Chrysanthemum, Gerbera, Calendula, Dendranthema, Argyranthemum, Dahlia, Tagetes, Zinnia, and many others.
Many species of this family possess medicinal properties and are used as traditional antiparasitic medicine.
Members of the family are also commonly featured in medical and phytochemical journals because the sesquiterpene lactone compounds contained within them are an important cause of allergic contact dermatitis. Allergy to these compounds is the leading cause of allergic contact dermatitis in florists in the US. Pollen from ragweed Ambrosia is among the main causes of so-called hay fever in the United States.
Asteraceae are also used for some industrial purposes. French Marigold (Tagetes patula) is common in commercial poultry feeds and its oil is extracted for uses in cola and the cigarette industry. The genera Chrysanthemum, Pulicaria, Tagetes, and Tanacetum contain species with useful insecticidal properties. Parthenium argentatum (guayule) is a source of hypoallergenic latex. | [
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956_10 | Several members of the family are copious nectar producers and are useful for evaluating pollinator populations during their bloom. Centaurea (knapweed), Helianthus annuus (domestic sunflower), and some species of Solidago (goldenrod) are major "honey plants" for beekeepers. Solidago produces relatively high protein pollen, which helps honey bees over winter.
References
Bibliography
External links
Asteraceae at the Angiosperm Phylogeny Website
Compositae.org – Compositae Working Group (CWG) and Global Compositae Database (GCD)
Asterales families
Extant Campanian first appearances | [
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957_0 | Apiaceae or Umbelliferae is a family of mostly aromatic flowering plants named after the type genus Apium and commonly known as the celery, carrot or parsley family, or simply as umbellifers. It is the 16th-largest family of flowering plants, with more than 3,700 species in 434 genera including such well-known and economically important plants such as ajwain, angelica, anise, asafoetida, caraway, carrot, celery, chervil, coriander, cumin, dill, fennel, lovage, cow parsley, parsley, parsnip and sea holly, as well as silphium, a plant whose identity is unclear and which may be extinct.
The family Apiaceae includes a significant number of phototoxic species, such as giant hogweed, and a smaller number of highly poisonous species, such as poison hemlock, water hemlock, spotted cowbane, fool's parsley, and various species of water dropwort.
Description
Most Apiaceae are annual, biennial or perennial herbs (frequently with the leaves aggregated toward the base), though a minority are woody shrubs or small trees such as Bupleurum fruticosum. Their leaves are of variable size and alternately arranged, or with the upper leaves becoming nearly opposite. The leaves may be petiolate or sessile. There are no stipules but the petioles are frequently sheathing and the leaves may be perfoliate. The leaf blade is usually dissected, ternate, or pinnatifid, but simple and entire in some genera, e.g. Bupleurum. Commonly, their leaves emit a marked smell when crushed, aromatic to foetid, but absent in some species. | [
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957_1 | The defining characteristic of this family is the inflorescence, the flowers nearly always aggregated in terminal umbels, that may be simple or more commonly compound, often umbelliform cymes. The flowers are usually perfect (hermaphroditic) and actinomorphic, but there may be zygomorphic flowers at the edge of the umbel, as in carrot (Daucus carota) and coriander, with petals of unequal size, the ones pointing outward from the umbel larger than the ones pointing inward. Some are andromonoecious, polygamomonoecious, or even dioecious (as in Acronema), with a distinct calyx and corolla, but the calyx is often highly reduced, to the point of being undetectable in many species, while the corolla can be white, yellow, pink or purple. The flowers are nearly perfectly pentamerous, with five petals and five stamens. | [
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957_2 | There is often variation in the functionality of the stamens even within a single inflorescence. Some flowers are functionally staminate (where a pistil may be present but has no ovules capable of being fertilized) while others are functionally pistillate (where stamens are present but their anthers do not produce viable pollen). Pollination of one flower by the pollen of a different flower of the same plant (geitonogamy) is common. The gynoecium consists of two carpels fused into a single, bicarpellate pistil with an inferior ovary. Stylopodia support two styles and secrete nectar, attracting pollinators like flies, mosquitoes, gnats, beetles, moths, and bees. The fruit is a schizocarp consisting of two fused carpels that separate at maturity into two mericarps, each containing a single seed. The fruits of many species are dispersed by wind but others such as those of Daucus spp., are covered in bristles, which may be hooked in sanicle Sanicula europaea and thus catch in the fur of animals. The seeds have an oily endosperm and often contain essential oils, containing aromatic compounds that are responsible for the flavour of commercially important umbelliferous seed such as anise, cumin and coriander. The shape and details of the ornamentation of the ripe fruits are important for identification to species level. | [
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957_3 | Taxonomy
Apiaceae was first described by John Lindley in 1836. The name is derived from the type genus Apium, which was originally used by Pliny the Elder circa 50 AD for a celery-like plant. The alternative name for the family, Umbelliferae, derives from the inflorescence being generally in the form of a compound umbel. The family was one of the first to be recognized as a distinct group in Jacques Daleschamps' 1586 Historia generalis plantarum. With Robert Morison's 1672 Plantarum umbelliferarum distribution nova it became the first group of plants for which a systematic study was published.
The family is solidly placed within the Apiales order in the APG III system. It is closely related to Araliaceae and the boundaries between these families remain unclear. Traditionally groups within the family have been delimited largely based on fruit morphology, and the results from this have not been congruent with the more recent molecular phylogenetic analyses. The subfamilial and tribal classification for the family is currently in a state of flux, with many of the groups being found to be grossly paraphyletic or polyphyletic.
General
According to the Angiosperm Phylogeny Website , 434 genera are in the family Apiaceae.
Ecology
The black swallowtail butterfly, Papilio polyxenes, uses the family Apiaceae for food and host plants for oviposition. The 22-spot ladybird is also commonly found eating mildew on these shrubs.
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957_4 | Uses
Many members of this family are cultivated for various purposes. Parsnip (Pastinaca sativa), carrot (Daucus carota) and Hamburg parsley (Petroselinum crispum) produce tap roots that are large enough to be useful as food. Many species produce essential oils in their leaves or fruits and as a result are flavourful aromatic herbs. Examples are parsley (Petroselinum crispum), coriander (Coriandrum sativum), culantro, and dill (Anethum graveolens). The seeds may be used in cuisine, as with coriander (Coriandrum sativum), fennel (Foeniculum vulgare), cumin (Cuminum cyminum), and caraway (Carum carvi).
Other notable cultivated Apiaceae include chervil (Anthriscus cerefolium), angelica (Angelica spp.), celery (Apium graveolens), arracacha (Arracacia xanthorrhiza), sea holly (Eryngium spp.), asafoetida (Ferula asafoetida), galbanum (Ferula gummosa), cicely (Myrrhis odorata), anise (Pimpinella anisum), lovage (Levisticum officinale), and hacquetia (Hacquetia epipactis).
Cultivation
Generally, all members of this family are best cultivated in the cool-season garden; indeed, they may not grow at all if the soils are too warm. Almost every widely cultivated plant of this group is a considered useful as a companion plant. One reason is because the tiny flowers clustered into umbels, are well suited for ladybugs, parasitic wasps, and predatory flies, which actually drink nectar when not reproducing. They then prey upon insect pests on nearby plants. Some of the members of this family considered "herbs" produce scents that are believed to ...mask the odours of nearby plants, thus making them harder for insect pests to find.
Other uses | [
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957_5 | Other uses
The poisonous members of the Apiaceae have been used for a variety of purposes globally. The poisonous Oenanthe crocata has been used to stupefy fish, Cicuta douglasii has been used as an aid in suicides, and arrow poisons have been made from various other family species.
Daucus carota has been used as coloring for butter.
Dorema ammoniacum, Ferula galbaniflua, and Ferula moschata (sumbul) are sources of incense.
The woody Azorella compacta Phil. has been used in South America for fuel.
Toxicity
Many species in the family Apiaceae produce phototoxic substances (called furanocoumarins) that sensitize human skin to sunlight. Contact with plant parts that contain furanocoumarins, followed by exposure to sunlight, may cause phytophotodermatitis, a serious skin inflammation. Phototoxic species include Ammi majus, Notobubon galbanum, the parsnip (Pastinaca sativa) and numerous species of the genus Heracleum, especially the giant hogweed (Heracleum mantegazzianum). Of all the plant species that have been reported to induce phytophotodermatitis, approximately half belong to the family Apiaceae.
The family Apiaceae also includes a smaller number of poisonous species, including poison hemlock, water hemlock, spotted cowbane, fool's parsley, and various species of water dropwort.
Some members of the family Apiaceae, including carrot, celery, fennel, parsley and parsnip, contain polyynes, an unusual class of organic compounds that exhibit cytotoxic effects.
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957_6 | References
Further reading
Constance, L. (1971). "History of the classification of Umbelliferae (Apiaceae)." in Heywood, V. H. [ed.], The biology and chemistry of the Umbelliferae, 1–11. Academic Press, London.
Cronquist, A. (1968). The Evolution and Classification of Flowering Plants. Boston: Houghton Mifflin.
French, D. H. (1971). "Ethnobotany of the Umbelliferae." in Heywood, V. H. [ed.], The biology and chemistry of the Umbelliferae, 385–412. Academic Press, London.
Hegnauer, R. (1971) "Chemical Patterns and Relationships of Umbelliferae." in Heywood, V. H. [ed.], The biology and chemistry of the Umbelliferae, 267–277. Academic Press, London.
Heywood, V. H. (1971). "Systematic survey of Old World Umbelliferae." in Heywood, V. H. [ed.], The biology and chemistry of the Umbelliferae, 31–41. Academic Press, London.
Judd, W. S. et al. (1999). Plant Systematics: A Phylogenetic Approach. Sunderland, MA: Sinauer Associates, Inc.
Nieto Feliner, Gonzalo; Jury, Stephen Leonard & Herrero Nieto, Alberto (eds.) Flora iberica. Plantas vasculares de la Península Ibérica e Islas Baleares. Vol. X. "Araliaceae-Umbelliferae" (2003) Madrid: Real Jardín Botánico, CSIC (in Spanish).
External links
Umbelliferae at The Families of Flowering Plants (DELTA)
Apiaceae at Discover Life
Umbellifer Resource Centre at the Royal Botanic Garden Edinburgh
Umbellifer Information Server at Moscow State University
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958_0 | An axon (from Greek ἄξων áxōn, axis), or nerve fiber (or nerve fibre: see spelling differences), is a long, slender projection of a nerve cell, or neuron, in vertebrates, that typically conducts electrical impulses known as action potentials away from the nerve cell body. The function of the axon is to transmit information to different neurons, muscles, and glands. In certain sensory neurons (pseudounipolar neurons), such as those for touch and warmth, the axons are called afferent nerve fibers and the electrical impulse travels along these from the periphery to the cell body and from the cell body to the spinal cord along another branch of the same axon. Axon dysfunction has caused many inherited and acquired neurological disorders which can affect both the peripheral and central neurons. Nerve fibers are classed into three types – group A nerve fibers, group B nerve fibers, and group C nerve fibers. Groups A and B are myelinated, and group C are unmyelinated. These groups include both sensory fibers and motor fibers. Another classification groups only the sensory fibers as Type I, Type II, Type III, and Type IV.
An axon is one of two types of cytoplasmic protrusions from the cell body of a neuron; the other type is a dendrite. Axons are distinguished from dendrites by several features, including shape (dendrites often taper while axons usually maintain a constant radius), length (dendrites are restricted to a small region around the cell body while axons can be much longer), and function (dendrites receive signals whereas axons transmit them). Some types of neurons have no axon and transmit signals from their dendrites. In some species, axons can emanate from dendrites known as axon-carrying dendrites. No neuron ever has more than one axon; however in invertebrates such as insects or leeches the axon sometimes consists of several regions that function more or less independently of each other. | [
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958_1 | Axons are covered by a membrane known as an axolemma; the cytoplasm of an axon is called axoplasm. Most axons branch, in some cases very profusely. The end branches of an axon are called telodendria. The swollen end of a telodendron is known as the axon terminal which joins the dendron or cell body of another neuron forming a synaptic connection. Axons make contact with other cells—usually other neurons but sometimes muscle or gland cells—at junctions called synapses. In some circumstances, the axon of one neuron may form a synapse with the dendrites of the same neuron, resulting in an autapse. At a synapse, the membrane of the axon closely adjoins the membrane of the target cell, and special molecular structures serve to transmit electrical or electrochemical signals across the gap. Some synaptic junctions appear along the length of an axon as it extends—these are called en passant ("in passing") synapses and can be in the hundreds or even the thousands along one axon. Other synapses appear as terminals at the ends of axonal branches.
A single axon, with all its branches taken together, can innervate multiple parts of the brain and generate thousands of synaptic terminals. A bundle of axons make a nerve tract in the central nervous system, and a fascicle in the peripheral nervous system. In placental mammals the largest white matter tract in the brain is the corpus callosum, formed of some 200 million axons in the human brain.
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958_2 | Anatomy
Axons are the primary transmission lines of the nervous system, and as bundles they form nerves. Some axons can extend up to one meter or more while others extend as little as one millimeter. The longest axons in the human body are those of the sciatic nerve, which run from the base of the spinal cord to the big toe of each foot. The diameter of axons is also variable. Most individual axons are microscopic in diameter (typically about one micrometer (µm) across). The largest mammalian axons can reach a diameter of up to 20 µm. The squid giant axon, which is specialized to conduct signals very rapidly, is close to 1 millimetre in diameter, the size of a small pencil lead. The numbers of axonal telodendria (the branching structures at the end of the axon) can also differ from one nerve fiber to the next. Axons in the central nervous system (CNS) typically show multiple telodendria, with many synaptic end points. In comparison, the cerebellar granule cell axon is characterized by a single T-shaped branch node from which two parallel fibers extend. Elaborate branching allows for the simultaneous transmission of messages to a large number of target neurons within a single region of the brain.
There are two types of axons in the nervous system: myelinated and unmyelinated axons. Myelin is a layer of a fatty insulating substance, which is formed by two types of glial cells: Schwann cells and oligodendrocytes. In the peripheral nervous system Schwann cells form the myelin sheath of a myelinated axon. In the central nervous system oligodendrocytes form the insulating myelin. Along myelinated nerve fibers, gaps in the myelin sheath known as nodes of Ranvier occur at evenly spaced intervals. The myelination enables an especially rapid mode of electrical impulse propagation called saltatory conduction. | [
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958_3 | The myelinated axons from the cortical neurons form the bulk of the neural tissue called white matter in the brain. The myelin gives the white appearance to the tissue in contrast to the grey matter of the cerebral cortex which contains the neuronal cell bodies. A similar arrangement is seen in the cerebellum. Bundles of myelinated axons make up the nerve tracts in the CNS. Where these tracts cross the midline of the brain to connect opposite regions they are called commissures. The largest of these is the corpus callosum that connects the two cerebral hemispheres, and this has around 20 million axons.
The structure of a neuron is seen to consist of two separate functional regions, or compartments – the cell body together with the dendrites as one region, and the axonal region as the other.
Axonal region
The axonal region or compartment, includes the axon hillock, the initial segment, the rest of the axon, and the axon telodendria, and axon terminals. It also includes the myelin sheath. The Nissl bodies that produce the neuronal proteins are absent in the axonal region. Proteins needed for the growth of the axon, and the removal of waste materials, need a framework for transport. This axonal transport is provided for in the axoplasm by arrangements of microtubules and intermediate filaments known as neurofilaments.
Axon hillock
The axon hillock is the area formed from the cell body of the neuron as it extends to become the axon. It precedes the initial segment. The received action potentials that are summed in the neuron are transmitted to the axon hillock for the generation of an action potential from the initial segment.
Axonal initial segment
The axonal initial segment (AIS) is a structurally and functionally separate microdomain of the axon. One function of the initial segment is to separate the main part of an axon from the rest of the neuron; another function is to help initiate action potentials. Both of these functions support neuron cell polarity, in which dendrites (and, in some cases the soma) of a neuron receive input signals at the basal region, and at the apical region the neuron's axon provides output signals. | [
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958_4 | The axon initial segment is unmyelinated and contains a specialized complex of proteins. It is between approximately 20 and 60 µm in length and functions as the site of action potential initiation. Both the position on the axon and the length of the AIS can change showing a degree of plasticity that can fine-tune the neuronal output. A longer AIS is associated with a greater excitability. Plasticity is also seen in the ability of the AIS to change its distribution and to maintain the activity of neural circuitry at a constant level.
The AIS is highly specialized for the fast conduction of nerve impulses. This is achieved by a high concentration of voltage-gated sodium channels in the initial segment where the action potential is initiated. The ion channels are accompanied by a high number of cell adhesion molecules and scaffolding proteins that anchor them to the cytoskeleton. Interactions with ankyrin G are important as it is the major organizer in the AIS.
Axonal transport
The axoplasm is the equivalent of cytoplasm in the cell. Microtubules form in the axoplasm at the axon hillock. They are arranged along the length of the axon, in overlapping sections, and all point in the same direction – towards the axon terminals. This is noted by the positive endings of the microtubules. This overlapping arrangement provides the routes for the transport of different materials from the cell body. Studies on the axoplasm has shown the movement of numerous vesicles of all sizes to be seen along cytoskeletal filaments – the microtubules, and neurofilaments, in both directions between the axon and its terminals and the cell body. | [
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958_5 | Outgoing anterograde transport from the cell body along the axon, carries mitochondria and membrane proteins needed for growth to the axon terminal. Ingoing retrograde transport carries cell waste materials from the axon terminal to the cell body. Outgoing and ingoing tracks use different sets of motor proteins. Outgoing transport is provided by kinesin, and ingoing return traffic is provided by dynein. Dynein is minus-end directed. There are many forms of kinesin and dynein motor proteins, and each is thought to carry a different cargo. The studies on transport in the axon led to the naming of kinesin.
Myelination
In the nervous system, axons may be myelinated, or unmyelinated. This is the provision of an insulating layer, called a myelin sheath. The myelin membrane is unique in its relatively high lipid to protein ratio.
In the peripheral nervous system axons are myelinated by glial cells known as Schwann cells. In the central nervous system the myelin sheath is provided by another type of glial cell, the oligodendrocyte. Schwann cells myelinate a single axon. An oligodendrocyte can myelinate up to 50 axons.
The composition of myelin is different in the two types. In the CNS the major myelin protein is proteolipid protein, and in the PNS it is myelin basic protein.
Nodes of Ranvier
Nodes of Ranvier (also known as myelin sheath gaps) are short unmyelinated segments of a myelinated axon, which are found periodically interspersed between segments of the myelin sheath. Therefore, at the point of the node of Ranvier, the axon is reduced in diameter. These nodes are areas where action potentials can be generated. In saltatory conduction, electrical currents produced at each node of Ranvier are conducted with little attenuation to the next node in line, where they remain strong enough to generate another action potential. Thus in a myelinated axon, action potentials effectively "jump" from node to node, bypassing the myelinated stretches in between, resulting in a propagation speed much faster than even the fastest unmyelinated axon can sustain. | [
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958_6 | Axon terminals
An axon can divide into many branches called telodendria (Greek–end of tree). At the end of each telodendron is an axon terminal (also called a synaptic bouton, or terminal bouton). Axon terminals contain synaptic vesicles that store the neurotransmitter for release at the synapse. This makes multiple synaptic connections with other neurons possible. Sometimes the axon of a neuron may synapse onto dendrites of the same neuron, when it is known as an autapse.
Action potentials
Most axons carry signals in the form of action potentials, which are discrete electrochemical impulses that travel rapidly along an axon, starting at the cell body and terminating at points where the axon makes synaptic contact with target cells. The defining characteristic of an action potential is that it is "all-or-nothing" — every action potential that an axon generates has essentially the same size and shape. This all-or-nothing characteristic allows action potentials to be transmitted from one end of a long axon to the other without any reduction in size. There are, however, some types of neurons with short axons that carry graded electrochemical signals, of variable amplitude. | [
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958_7 | When an action potential reaches a presynaptic terminal, it activates the synaptic transmission process. The first step is rapid opening of calcium ion channels in the membrane of the axon, allowing calcium ions to flow inward across the membrane. The resulting increase in intracellular calcium concentration causes synaptic vesicles (tiny containers enclosed by a lipid membrane) filled with a neurotransmitter chemical to fuse with the axon's membrane and empty their contents into the extracellular space. The neurotransmitter is released from the presynaptic nerve through exocytosis. The neurotransmitter chemical then diffuses across to receptors located on the membrane of the target cell. The neurotransmitter binds to these receptors and activates them. Depending on the type of receptors that are activated, the effect on the target cell can be to excite the target cell, inhibit it, or alter its metabolism in some way. This entire sequence of events often takes place in less than a thousandth of a second. Afterward, inside the presynaptic terminal, a new set of vesicles is moved into position next to the membrane, ready to be released when the next action potential arrives. The action potential is the final electrical step in the integration of synaptic messages at the scale of the neuron.
Extracellular recordings of action potential propagation in axons has been demonstrated in freely moving animals. While extracellular somatic action potentials have been used to study cellular activity in freely moving animals such as place cells, axonal activity in both white and gray matter can also be recorded. Extracellular recordings of axon action potential propagation is distinct from somatic action potentials in three ways: 1. The signal has a shorter peak-trough duration (~150μs) than of pyramidal cells (~500μs) or interneurons (~250μs). 2. The voltage change is triphasic. 3. Activity recorded on a tetrode is seen on only one of the four recording wires. In recordings from freely moving rats, axonal signals have been isolated in white matter tracts including the alveus and the corpus callosum as well hippocampal gray matter. | [
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958_8 | In fact, the generation of action potentials in vivo is sequential in nature, and these sequential spikes constitute the digital codes in the neurons. Although previous studies indicate an axonal origin of a single spike evoked by short-term pulses, physiological signals in vivo trigger the initiation of sequential spikes at the cell bodies of the neurons.
In addition to propagating action potentials to axonal terminals, the axon is able to amplify the action potentials, which makes sure a secure propagation of sequential action potentials toward the axonal terminal. In terms of molecular mechanisms, voltage-gated sodium channels in the axons possess lower threshold and shorter refractory period in response to short-term pulses.
Development and growth
Development
The development of the axon to its target, is one of the six major stages in the overall development of the nervous system. Studies done on cultured hippocampal neurons suggest that neurons initially produce multiple neurites that are equivalent, yet only one of these neurites is destined to become the axon. It is unclear whether axon specification precedes axon elongation or vice versa, although recent evidence points to the latter. If an axon that is not fully developed is cut, the polarity can change and other neurites can potentially become the axon. This alteration of polarity only occurs when the axon is cut at least 10 μm shorter than the other neurites. After the incision is made, the longest neurite will become the future axon and all the other neurites, including the original axon, will turn into dendrites. Imposing an external force on a neurite, causing it to elongate, will make it become an axon. Nonetheless, axonal development is achieved through a complex interplay between extracellular signaling, intracellular signaling and cytoskeletal dynamics. | [
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958_9 | Extracellular signaling
The extracellular signals that propagate through the extracellular matrix surrounding neurons play a prominent role in axonal development. These signaling molecules include proteins, neurotrophic factors, and extracellular matrix and adhesion molecules.
Netrin (also known as UNC-6) a secreted protein, functions in axon formation. When the UNC-5 netrin receptor is mutated, several neurites are irregularly projected out of neurons and finally a single axon is extended anteriorly. The neurotrophic factors – nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF) and neurotrophin-3 (NTF3) are also involved in axon development and bind to Trk receptors.
The ganglioside-converting enzyme plasma membrane ganglioside sialidase (PMGS), which is involved in the activation of TrkA at the tip of neutrites, is required for the elongation of axons. PMGS asymmetrically distributes to the tip of the neurite that is destined to become the future axon.
Intracellular signaling
During axonal development, the activity of PI3K is increased at the tip of destined axon. Disrupting the activity of PI3K inhibits axonal development. Activation of PI3K results in the production of phosphatidylinositol (3,4,5)-trisphosphate (PtdIns) which can cause significant elongation of a neurite, converting it into an axon. As such, the overexpression of phosphatases that dephosphorylate PtdIns leads into the failure of polarization.
Cytoskeletal dynamics
The neurite with the lowest actin filament content will become the axon. PGMS concentration and f-actin content are inversely correlated; when PGMS becomes enriched at the tip of a neurite, its f-actin content is substantially decreased. In addition, exposure to actin-depolimerizing drugs and toxin B (which inactivates Rho-signaling) causes the formation of multiple axons. Consequently, the interruption of the actin network in a growth cone will promote its neurite to become the axon.
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958_10 | Growth
Growing axons move through their environment via the growth cone, which is at the tip of the axon. The growth cone has a broad sheet-like extension called a lamellipodium which contain protrusions called filopodia. The filopodia are the mechanism by which the entire process adheres to surfaces and explores the surrounding environment. Actin plays a major role in the mobility of this system. Environments with high levels of cell adhesion molecules (CAMs) create an ideal environment for axonal growth. This seems to provide a "sticky" surface for axons to grow along. Examples of CAM's specific to neural systems include N-CAM, TAG-1—an axonal glycoprotein——and MAG, all of which are part of the immunoglobulin superfamily. Another set of molecules called extracellular matrix-adhesion molecules also provide a sticky substrate for axons to grow along. Examples of these molecules include laminin, fibronectin, tenascin, and perlecan. Some of these are surface bound to cells and thus act as short range attractants or repellents. Others are difusible ligands and thus can have long range effects.
Cells called guidepost cells assist in the guidance of neuronal axon growth. These cells that help axon guidance, are typically other neurons that are sometimes immature. When the axon has completed its growth at its connection to the target, the diameter of the axon can increase by up to five times, depending on the speed of conduction required.
It has also been discovered through research that if the axons of a neuron were damaged, as long as the soma (the cell body of a neuron) is not damaged, the axons would regenerate and remake the synaptic connections with neurons with the help of guidepost cells. This is also referred to as neuroregeneration. | [
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958_11 | Nogo-A is a type of neurite outgrowth inhibitory component that is present in the central nervous system myelin membranes (found in an axon). It has a crucial role in restricting axonal regeneration in adult mammalian central nervous system. In recent studies, if Nogo-A is blocked and neutralized, it is possible to induce long-distance axonal regeneration which leads to enhancement of functional recovery in rats and mouse spinal cord. This has yet to be done on humans. A recent study has also found that macrophages activated through a specific inflammatory pathway activated by the Dectin-1 receptor are capable of promoting axon recovery, also however causing neurotoxicity in the neuron.
Length regulation
Axons vary largely in length from a few micrometers up to meters in some animals. This emphasizes that there must be a cellular length regulation mechanism allowing the neurons both to sense the length of their axons and to control their growth accordingly. It was discovered that motor proteins play an important role in regulating the length of axons. Based on this observation, researchers developed an explicit model for axonal growth describing how motor proteins could affect the axon length on the molecular level. These studies suggest that motor proteins carry signaling molecules from the soma to the growth cone and vice versa whose concentration oscillates in time with a length-dependent frequency.
Classification
The axons of neurons in the human peripheral nervous system can be classified based on their physical features and signal conduction properties. Axons were known to have different thicknesses (from 0.1 to 20 µm) and these differences were thought to relate to the speed at which an action potential could travel along the axon – its conductance velocity. Erlanger and Gasser proved this hypothesis, and identified several types of nerve fiber, establishing a relationship between the diameter of an axon and its nerve conduction velocity. They published their findings in 1941 giving the first classification of axons. | [
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958_12 | Axons are classified in two systems. The first one introduced by Erlanger and Gasser, grouped the fibers into three main groups using the letters A, B, and C. These groups, group A, group B, and group C include both the sensory fibers (afferents) and the motor fibres (efferents). The first group A, was subdivided into alpha, beta, gamma, and delta fibers — Aα, Aβ, Aγ, and Aδ. The motor neurons of the different motor fibers, were the lower motor neurons – alpha motor neuron, beta motor neuron, and gamma motor neuron having the Aα, Aβ, and Aγ nerve fibers respectively.
Later findings by other researchers identified two groups of Aa fibers that were sensory fibers. These were then introduced into a system that only included sensory fibers (though some of these were mixed nerves and were also motor fibers). This system refers to the sensory groups as Types and uses Roman numerals: Type Ia, Type Ib, Type II, Type III, and Type IV.
Lower motor neurons have two kind of fibers:
Different sensory receptors innervate different types of nerve fibers. Proprioceptors are innervated by type Ia, Ib and II sensory fibers, mechanoreceptors by type II and III sensory fibers and nociceptors and thermoreceptors by type III and IV sensory fibers.
Autonomic
The autonomic nervous system has two kinds of peripheral fibers:
Clinical significance
In order of degree of severity, injury to a nerve can be described as neurapraxia, axonotmesis, or neurotmesis.
Concussion is considered a mild form of diffuse axonal injury. Axonal injury can also cause central chromatolysis. The dysfunction of axons in the nervous system is one of the major causes of many inherited neurological disorders that affect both peripheral and central neurons. | [
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958_13 | When an axon is crushed, an active process of axonal degeneration takes place at the part of the axon furthest from the cell body. This degeneration takes place quickly following the injury, with the part of the axon being sealed off at the membranes and broken down by macrophages. This is known as Wallerian degeneration. Dying back of an axon can also take place in many neurodegenerative diseases, particularly when axonal transport is impaired, this is known as Wallerian-like degeneration. Studies suggest that the degeneration happens as
a result of the axonal protein NMNAT2, being prevented from reaching all of the axon.
Demyelination of axons causes the multitude of neurological symptoms found in the disease multiple sclerosis.
Dysmyelination is the abnormal formation of the myelin sheath. This is implicated in several leukodystrophies, and also in schizophrenia.
A severe traumatic brain injury can result in widespread lesions to nerve tracts damaging the axons in a condition known as diffuse axonal injury. This can lead to a persistent vegetative state. It has been shown in studies on the rat that axonal damage from a single mild traumatic brain injury, can leave a susceptibility to further damage, after repeated mild traumatic brain injuries.
A nerve guidance conduit is an artificial means of guiding axon growth to enable neuroregeneration, and is one of the many treatments used for different kinds of nerve injury. | [
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958_14 | History
German anatomist Otto Friedrich Karl Deiters is generally credited with the discovery of the axon by distinguishing it from the dendrites. Swiss Rüdolf Albert von Kölliker and German Robert Remak were the first to identify and characterize the axon initial segment. Kölliker named the axon in 1896. Louis-Antoine Ranvier was the first to describe the gaps or nodes found on axons and for this contribution these axonal features are now commonly referred to as the nodes of Ranvier. Santiago Ramón y Cajal, a Spanish anatomist, proposed that axons were the output components of neurons, describing their functionality. Joseph Erlanger and Herbert Gasser earlier developed the classification system for peripheral nerve fibers, based on axonal conduction velocity, myelination, fiber size etc. Alan Hodgkin and Andrew Huxley also employed the squid giant axon (1939) and by 1952 they had obtained a full quantitative description of the ionic basis of the action potential, leading to the formulation of the Hodgkin–Huxley model. Hodgkin and Huxley were awarded jointly the Nobel Prize for this work in 1963. The formulae detailing axonal conductance were extended to vertebrates in the Frankenhaeuser–Huxley equations. The understanding of the biochemical basis for action potential propagation has advanced further, and includes many details about individual ion channels.
Other animals
The axons in invertebrates have been extensively studied. The longfin inshore squid, often used as a model organism has the longest known axon. The giant squid has the largest axon known. Its size ranges from a half (typically) to one millimetre in diameter and is used in the control of its jet propulsion system. The fastest recorded conduction speed of 210 m/s, is found in the ensheathed axons of some pelagic Penaeid shrimps and the usual range is between 90 and 200 m/s (cf 100–120 m/s for the fastest myelinated vertebrate axon.) | [
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958_15 | In other cases as seen in rat studies an axon originates from a dendrite; such axons are said to have "dendritic origin". Some axons with dendritic origin similarly have a "proximal" initial segment that starts directly at the axon origin, while others have a "distal" initial segment, discernibly separated from the axon origin. In many species some of the neurons have axons that emanate from the dendrite and not from the cell body, and these are known as axon-carrying dendrites. In many cases, an axon originates at an axon hillock on the soma; such axons are said to have "somatic origin". Some axons with somatic origin have a "proximal" initial segment adjacent the axon hillock, while others have a "distal" initial segment, separated from the soma by an extended axon hillock.
See also
Electrophysiology
Ganglionic eminence
Giant axonal neuropathy
Neuronal tracing
Pioneer axon
References
External links
— "Slide 3 Spinal cord"
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960_0 | The ancient Aramaic alphabet was adapted by Arameans from the Phoenician alphabet and became a distinct script by the 8th century BC. It was used to write the Aramaic language and had displaced the Paleo-Hebrew alphabet, itself a derivative of the Phoenician alphabet, for the writing of Hebrew. The letters all represent consonants, some of which are also used as matres lectionis to indicate long vowels.
The Aramaic alphabet is historically significant since virtually all modern Middle Eastern writing systems can be traced back to it as well as numerous non-Chinese writing systems of Central and East Asia. That is primarily from the widespread usage of the Aramaic language as both a lingua franca and the official language of the Neo-Assyrian and Neo-Babylonian Empires, and their successor, the Achaemenid Empire. Among the scripts in modern use, the Hebrew alphabet bears the closest relation to the Imperial Aramaic script of the 5th century BC, with an identical letter inventory and, for the most part, nearly identical letter shapes. The Aramaic alphabet was an ancestor to the Nabataean alphabet and the later Arabic alphabet.
Writing systems (like the Aramaic one) that indicate consonants but do not indicate most vowels other than by means of matres lectionis or added diacritical signs, have been called abjads by Peter T. Daniels to distinguish them from alphabets, such as the Greek alphabet, which represent vowels more systematically. The term was coined to avoid the notion that a writing system that represents sounds must be either a syllabary or an alphabet, which would imply that a system like Aramaic must be either a syllabary (as argued by Ignace Gelb) or an incomplete or deficient alphabet (as most other writers have said). Rather, it is a different type.
Origins
The earliest inscriptions in the Aramaic language use the Phoenician alphabet. Over time, the alphabet developed into the form shown below. Aramaic gradually became the lingua franca throughout the Middle East, with the script at first complementing and then displacing Assyrian cuneiform, as the predominant writing system.
Achaemenid Empire (The First Persian Empire) | [
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960_1 | Achaemenid Empire (The First Persian Empire)
Around 500 BC, following the Achaemenid conquest of Mesopotamia under Darius I, Old Aramaic was adopted by the Persians as the "vehicle for written communication between the different regions of the vast Persian empire with its different peoples and languages. The use of a single official language, which modern scholarship has dubbed as Official Aramaic, Imperial Aramaic or Achaemenid Aramaic, can be assumed to have greatly contributed to the astonishing success of the Achaemenid Persians in holding their far-flung empire together for as long as they did."
Imperial Aramaic was highly standardised; its orthography was based more on historical roots than any spoken dialect and was inevitably influenced by Old Persian. The Aramaic glyph forms of the period are often divided into two main styles, the "lapidary" form, usually inscribed on hard surfaces like stone monuments, and a cursive form whose lapidary form tended to be more conservative by remaining more visually similar to Phoenician and early Aramaic. Both were in use through the Achaemenid Persian period, but the cursive form steadily gained ground over the lapidary, which had largely disappeared by the 3rd century BC.
For centuries after the fall of the Achaemenid Empire in 331 BC, Imperial Aramaic, or something near enough to it to be recognisable, would remain an influence on the various native Iranian languages. The Aramaic script would survive as the essential characteristics of the Iranian Pahlavi writing system.
30 Aramaic documents from Bactria have been recently discovered, an analysis of which was published in November 2006. The texts, which were rendered on leather, reflect the use of Aramaic in the 4th century BC in the Persian Achaemenid administration of Bactria and Sogdiana.
The widespread usage of Achaemenid Aramaic in the Middle East led to the gradual adoption of the Aramaic alphabet for writing Hebrew. Formerly, Hebrew had been written using an alphabet closer in form to that of Phoenician, the Paleo-Hebrew alphabet.
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960_2 | Aramaic-derived scripts
Since the evolution of the Aramaic alphabet out of the Phoenician one was a gradual process, the division of the world's alphabets into the ones derived from the Phoenician one directly and the ones derived from Phoenician via Aramaic is somewhat artificial. In general, the alphabets of the Mediterranean region (Anatolia, Greece, Italy) are classified as Phoenician-derived, adapted from around the 8th century BC, and those of the East (the Levant, Persia, Central Asia and India) are considered Aramaic-derived, adapted from around the 6th century BC from the Imperial Aramaic script of the Achaemenid Empire.
After the fall of the Achaemenid Empire, the unity of the Imperial Aramaic script was lost, diversifying into a number of descendant cursives.
The Hebrew and Nabataean alphabets, as they stood by the Roman era, were little changed in style from the Imperial Aramaic alphabet. Ibn Khaldun (1332–1406) alleges that not only the old Nabataean writing was influenced by the "Syrian script" (i.e. Aramaic), but also the old Chaldean script.
A cursive Hebrew variant developed from the early centuries AD, but it remained restricted to the status of a variant used alongside the noncursive. By contrast, the cursive developed out of the Nabataean alphabet in the same period soon became the standard for writing Arabic, evolving into the Arabic alphabet as it stood by the time of the early spread of Islam.
The development of cursive versions of Aramaic also led to the creation of the Syriac, Palmyrene and Mandaic alphabets, which formed the basis of the historical scripts of Central Asia, such as the Sogdian and Mongolian alphabets.
The Old Turkic script is generally considered to have its ultimate origins in Aramaic, in particular via the Pahlavi or Sogdian alphabets, as suggested by V. Thomsen, or possibly via Kharosthi (cf., Issyk inscription).
Brahmi script was also possibly derived or inspired by Aramaic. Brahmic family of scripts includes Devanagari. | [
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960_3 | Languages using the alphabet
Today, Biblical Aramaic, Jewish Neo-Aramaic dialects and the Aramaic language of the Talmud are written in the modern-Hebrew alphabet (distinguished from the Old Hebrew script). In classical Jewish literature, the name given to the modern-Hebrew script was "Ashurit" (the ancient Assyrian script), a script now known widely as the Aramaic script. It is believed that during the period of Assyrian dominion that Aramaic script and language received official status. Syriac and Christian Neo-Aramaic dialects are today written in the Syriac alphabet, which script has superseded the more ancient Assyrian script and now bears its name. Mandaic is written in the Mandaic alphabet. The near-identical nature of the Aramaic and the classical Hebrew alphabets caused Aramaic text to be typeset mostly in the standard Hebrew script in scholarly literature.
Maaloula
In Maaloula, one of few surviving communities in which a Western Aramaic dialect is still spoken, an Aramaic institute was established in 2007 by Damascus University that teaches courses to keep the language alive. The institute's activities were suspended in 2010 amidst fears that the square Aramaic alphabet used in the program too closely resembled the square script of the Hebrew alphabet and all the signs with the square Aramaic script were taken down. The program stated that they would instead use the more distinct Syriac alphabet, although use of the Aramaic alphabet has continued to some degree. Al Jazeera Arabic also broadcast a program about Western Neo-Aramaic and the villages in which it is spoken with the square script still in use.
Letters
Matres lectionis
In Aramaic writing, Waw and Yodh serve a double function. Originally, they represented only the consonants w and y, but they were later adopted to indicate the long vowels ū and ī respectively as well (often also ō and ē respectively). In the latter role, they are known as or "mothers of reading".
Ālap, likewise, has some of the characteristics of a because in initial positions, it indicates a glottal stop (followed by a vowel), but otherwise, it often also stands for the long vowels ā or ē. Among Jews, the influence of Hebrew often led to the use of Hē instead, at the end of a word.
The practice of using certain letters to hold vowel values spread to Aramaic-derived writing systems, such as in Arabic and Hebrew, which still follow the practice. | [
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960_4 | Unicode
The Imperial Aramaic alphabet was added to the Unicode Standard in October 2009, with the release of version 5.2.
The Unicode block for Imperial Aramaic is U+10840–U+1085F:
The Syriac Aramaic alphabet was added to the Unicode Standard in September 1999, with the release of version 3.0.
The Syriac Abbreviation (a type of overline) can be represented with a special control character called the Syriac Abbreviation Mark (U+070F). The Unicode block for Syriac Aramaic is U+0700–U+074F:
See also
Syriac alphabet
References
Sources
Byrne, Ryan. "Middle Aramaic Scripts". Encyclopaedia of Language and Linguistics. Elsevier. (2006)
Daniels, Peter T., et al. eds. The World's Writing Systems. Oxford. (1996)
Coulmas, Florian. The Writing Systems of the World. Blackwell Publishers Ltd, Oxford. (1989)
Rudder, Joshua. Learn to Write Aramaic: A Step-by-Step Approach to the Historical & Modern Scripts. n.p.: CreateSpace Independent Publishing Platform, 2011. 220 pp. . Includes a wide variety of Aramaic scripts.
Ancient Hebrew and Aramaic on Coins, reading and transliterating Proto-Hebrew, online edition (Judaea Coin Archive).
External links
Comparison of Aramaic to related alphabets
Omniglot entry
8th-century BC establishments
Obsolete writing systems
Persian scripts
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966_0 | "American shot" or "cowboy shot" is a translation of a phrase from French film criticism, plan américain, and refers to a medium-long ("knee") film shot of a group of characters, who are arranged so that all are visible to the camera. The usual arrangement is for the actors to stand in an irregular line from one side of the screen to the other, with the actors at the end coming forward a little and standing more in profile than the others. The purpose of the composition is to allow complex dialogue scenes to be played out without changes in camera position. In some literature, this is simply referred to as a 3/4 shot.
One of the other main reasons why French critics called it "American shot" was its frequent use in western genre. This was because a shot that started at knee level would reveal the weapon of a cowboy, usually holstered at his waist. It is actually the closest the camera can get to an actor while keeping both his face and his holstered gun in frame.
The French critics thought it was characteristic of American films of the 1930s or 1940s; however, it was mostly characteristic of cheaper American movies, such as Charlie Chan mysteries where people collected in front of a fireplace or at the foot of the stairs in order to explain what happened a few minutes ago.
Howard Hawks legitimized this style in his films, allowing characters to act, even when not talking, when most of the audience would not be paying attention. It became his trademark style.
References
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967_0 | Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. It is often triggered by a viral infection or (very rarely) vaccinations.
ADEM's symptoms resemble the symptoms of multiple sclerosis (MS), so the disease itself is sorted into the classification of the multiple sclerosis borderline diseases. However, ADEM has several features that distinguish it from MS. Unlike MS, ADEM occurs usually in children and is marked with rapid fever, although adolescents and adults can get the disease too. ADEM consists of a single flare-up whereas MS is marked with several flare-ups (or relapses), over a long period of time. Relapses following ADEM are reported in up to a quarter of patients, but the majority of these 'multiphasic' presentations following ADEM likely represent MS. ADEM is also distinguished by a loss of consciousness, coma and death, which is very rare in MS, except in severe cases.
It affects about 8 per 1,000,000 people per year. Although it occurs in all ages, most reported cases are in children and adolescents, with the average age around 5 to 8 years old. The disease affects males and females almost equally. ADEM shows seasonal variation with higher incidence in winter and spring months which may coincide with higher viral infections during these months. The mortality rate may be as high as 5%; however, full recovery is seen in 50 to 75% of cases with increase in survival rates up to 70 to 90% with figures including minor residual disability as well. The average time to recover from ADEM flare-ups is one to six months. | [
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967_1 | ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. Usually these are found in the subcortical and central white matter and cortical gray-white junction of both cerebral hemispheres, cerebellum, brainstem, and spinal cord, but periventricular white matter and gray matter of the cortex, thalami and basal ganglia may also be involved.
When a person has more than one demyelinating episode of ADEM, the disease is then called recurrent disseminated encephalomyelitis or multiphasic disseminated encephalomyelitis (MDEM). Also, a fulminant course in adults has been described.
Signs and symptoms
ADEM has an abrupt onset and a monophasic course. Symptoms usually begin 1–3 weeks after infection. Major symptoms include fever, headache, nausea and vomiting, confusion, vision impairment, drowsiness, seizures and coma. Although initially the symptoms are usually mild, they worsen rapidly over the course of hours to days, with the average time to maximum severity being about four and a half days. Additional symptoms include hemiparesis, paraparesis, and cranial nerve palsies.
ADEM in COVID-19
Neurological symptoms were the main presentation of COVID-19, which did not correlate with the severity of respiratory symptoms. The high incidence of ADEM with hemorrhage is striking. Brain inflammation is likely caused by an immune response to the disease rather than neurotropism. CSF analysis was not indicative of an infectious process, neurological impairment was not present in the acute phase of the infection, and neuroimaging findings were not typical of classical toxic and metabolic disorders. The finding of bilateral periventricular relatively asymmetrical lesions allied with deep white matter involvement, that may also be present in cortical gray-white matter junction, thalami, basal ganglia, cerebellum, and brainstem suggests an acute demyelination process. Additionally, hemorrhagic white matter lesions, clusters of macrophages related to axonal injury and ADEM-like appearance were also found in subcortical white matter. | [
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] |
967_2 | Causes
Since the discovery of the anti-MOG specificity against multiple sclerosis diagnosis it is considered that ADEM is one of the possible clinical causes of anti-MOG associated encephalomyelitis
About how the anti-MOG antibodies appear in the patients serum there are several theories:
A preceding antigenic challenge can be identified in approximately two-thirds of people. Some viral infections thought to induce ADEM include influenza virus, dengue, enterovirus, measles, mumps, rubella, varicella zoster, Epstein–Barr virus, cytomegalovirus, herpes simplex virus, hepatitis A, coxsackievirus and COVID-19. Bacterial infections include Mycoplasma pneumoniae, Borrelia burgdorferi, Leptospira, and beta-hemolytic Streptococci.
Exposure to vaccines: The only vaccine proven related to ADEM is the Semple form of the rabies vaccine, but hepatitis B, pertussis, diphtheria, measles, mumps, rubella, pneumococcus, varicella, influenza, Japanese encephalitis, and polio vaccines have all been implicated. The majority of the studies that correlate vaccination with ADEM onset use small samples or case studies. Large scale epidemiological studies (e.g., of MMR vaccine or smallpox vaccine) do not show increased risk of ADEM following vaccination. An upper bound for the risk of ADEM from measles vaccination, if it exists, can be estimated to be 10 per million, which is far lower than the risk of developing ADEM from an actual measles infection, which is about 1 per 1,000 cases. For a rubella infection, the risk is 1 per 5,000 cases. Some early vaccines, later shown to have been contaminated with host animal CNS tissue, had ADEM incident rates as high as 1 in 600.
In rare cases, ADEM seems to follow from organ transplantation.
Diagnosis
ADEM term has been inconsistently used at different times. Currently, the commonly accepted international standard for the clinical case definition is the one published by the International Pediatric MS Study Group, revision 2007. | [
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967_3 | Given that the definition is clinical, it is currently unknown if all the cases with ADEM are positive for anti-MOG autoantibody, but in any case, it seems strongly related to ADEM diagnosis.
Differential diagnosis
Multiple sclerosis
While ADEM and MS both involve autoimmune demyelination, they differ in many clinical, genetic, imaging, and histopathological aspects. Some authors consider MS and its borderline forms to constitute a spectrum, differing only in chronicity, severity, and clinical course, while others consider them discretely different diseases.
Typically, ADEM appears in children following an antigenic challenge and remains monophasic. Nevertheless, ADEM does occur in adults, and can also be clinically multiphasic.
Problems for differential diagnosis increase due to the lack of agreement for a definition of multiple sclerosis. If MS were defined just by the separation in time and space of the demyelinating lesions as McDonald did, it would not be enough to make a difference, as some cases of ADEM satisfy these conditions. Therefore, some authors propose to establish the separation line in the shape of the lesions around the veins, being therefore "perivenous vs. confluent demyelination".
The pathology of ADEM is very similar to that of MS with some differences. The pathological hallmark of ADEM is perivenular inflammation with limited "sleeves of demyelination". Nevertheless, MS-like plaques (confluent demyelination) can appear
Plaques in the white matter in MS are sharply delineated, while the glial scar in ADEM is smooth. Axons are better preserved in ADEM lesions. Inflammation in ADEM is widely disseminated and ill-defined, and finally, lesions are strictly perivenous, while in MS they are disposed around veins, but not so sharply.
Nevertheless, the co-occurrence of perivenous and confluent demyelination in some individuals suggests pathogenic overlap between acute disseminated encephalomyelitis and multiple sclerosis and misclassification even with biopsy or even postmortem ADEM in adults can progress to MS
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967_4 | Multiphasic disseminated encephalomyelitis
When the person has more than one demyelinating episode of ADEM, the disease is then called recurrent disseminated encephalomyelitis or multiphasic disseminated encephalomyelitis (MDEM).
It has been found that anti-MOG auto-antibodies are related to this kind of ADEM
Another variant of ADEM in adults has been described, also related to anti-MOG auto-antibodies, has been named fulminant disseminated encephalomyelitis, and it has been reported to be clinically ADEM, but showing MS-like lesions on autopsy. It has been classified inside the anti-MOG associated inflammatory demyelinating diseases.
Acute hemorrhagic leukoencephalitis
Acute hemorrhagic leukoencephalitis (AHL, or AHLE), acute hemorrhagic encephalomyelitis (AHEM), acute necrotizing hemorrhagic leukoencephalitis (ANHLE), Weston-Hurst syndrome, or Hurst's disease, is a hyperacute and frequently fatal form of ADEM. AHL is relatively rare (less than 100 cases have been reported in the medical literature ), it is seen in about 2% of ADEM cases, and is characterized by necrotizing vasculitis of venules and hemorrhage, and edema. Death is common in the first week and overall mortality is about 70%, but increasing evidence points to favorable outcomes after aggressive treatment with corticosteroids, immunoglobulins, cyclophosphamide, and plasma exchange. About 70% of survivors show residual neurological deficits, but some survivors have shown surprisingly little deficit considering the magnitude of the white matter affected.
This disease has been occasionally associated with ulcerative colitis and Crohn's disease, malaria, sepsis associated with immune complex deposition, methanol poisoning, and other underlying conditions. Also anecdotal association with MS has been reported | [
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967_5 | Laboratory studies that support diagnosis of AHL are: peripheral leukocytosis, cerebrospinal fluid (CSF) pleocytosis associated with normal glucose and increased protein. On magnetic resonance imaging (MRI), lesions of AHL typically show extensive T2-weighted and fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities with areas of hemorrhages, significant edema, and mass effect.
Treatment
No controlled clinical trials have been conducted on ADEM treatment, but aggressive treatment aimed at rapidly reducing inflammation of the CNS is standard. The widely accepted first-line treatment is high doses of intravenous corticosteroids, such as methylprednisolone or dexamethasone, followed by 3–6 weeks of gradually lower oral doses of prednisolone. Patients treated with methylprednisolone have shown better outcomes than those treated with dexamethasone. Oral tapers of less than three weeks duration show a higher chance of relapsing, and tend to show poorer outcomes. Other anti-inflammatory and immunosuppressive therapies have been reported to show beneficial effect, such as plasmapheresis, high doses of intravenous immunoglobulin (IVIg), mitoxantrone and cyclophosphamide. These are considered alternative therapies, used when corticosteroids cannot be used or fail to show an effect. | [
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967_6 | There is some evidence to suggest that patients may respond to a combination of methylprednisolone and immunoglobulins if they fail to respond to either separately
In a study of 16 children with ADEM, 10 recovered completely after high-dose methylprednisolone, one severe case that failed to respond to steroids recovered completely after IV Ig; the five most severe cases – with ADAM and severe peripheral neuropathy – were treated with combined high-dose methylprednisolone and immunoglobulin, two remained paraplegic, one had motor and cognitive handicaps, and two recovered. A recent review of IVIg treatment of ADEM (of which the previous study formed the bulk of the cases) found that 70% of children showed complete recovery after treatment with IVIg, or IVIg plus corticosteroids. A study of IVIg treatment in adults with ADEM showed that IVIg seems more effective in treating sensory and motor disturbances, while steroids seem more effective in treating impairments of cognition, consciousness and rigor. This same study found one subject, a 71-year-old man who had not responded to steroids, that responded to an IVIg treatment 58 days after disease onset.
Prognosis
Full recovery is seen in 50 to 70% of cases, ranging to 70 to 90% recovery with some minor residual disability (typically assessed using measures such as mRS or EDSS), average time to recover is one to six months. The mortality rate may be as high as 5–10%. Poorer outcomes are associated with unresponsiveness to steroid therapy, unusually severe neurological symptoms, or sudden onset. Children tend to have more favorable outcomes than adults, and cases presenting without fevers tend to have poorer outcomes. The latter effect may be due to either protective effects of fever, or that diagnosis and treatment is sought more rapidly when fever is present.
ADEM can progress to MS. It will be considered MS if some lesions appear in different times and brain areas
Motor deficits
Residual motor deficits are estimated to remain in about 8 to 30% of cases, the range in severity from mild clumsiness to ataxia and hemiparesis. | [
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967_7 | Neurocognitive
Patients with demyelinating illnesses, such as MS, have shown cognitive deficits even when there is minimal physical disability. Research suggests that similar effects are seen after ADEM, but that the deficits are less severe than those seen in MS. A study of six children with ADEM (mean age at presentation 7.7 years) were tested for a range of neurocognitive tests after an average of 3.5 years of recovery. All six children performed in the normal range on most tests, including verbal IQ and performance IQ, but performed at least one standard deviation below age norms in at least one cognitive domain, such as complex attention (one child), short-term memory (one child) and internalizing behaviour/affect (two children). Group means for each cognitive domain were all within one standard deviation of age norms, demonstrating that, as a group, they were normal. These deficits were less severe than those seen in similar aged children with a diagnosis of MS.
Another study compared nineteen children with a history of ADEM, of which 10 were five years of age or younger at the time (average age 3.8 years old, tested an average of 3.9 years later) and nine were older (mean age 7.7y at time of ADEM, tested an average of 2.2 years later) to nineteen matched controls. Scores on IQ tests and educational achievement were lower for the young onset ADEM group (average IQ 90) compared to the late onset (average IQ 100) and control groups (average IQ 106), while the late onset ADEM children scored lower on verbal processing speed. Again, all groups means were within one standard deviation of the controls, meaning that while effects were statistically reliable, the children were as a whole, still within the normal range. There were also more behavioural problems in the early onset group, although there is some suggestion that this may be due, at least in part, to the stress of hospitalization at a young age.
Research
The relationship between ADEM and anti-MOG associated encephalomyelitis is currently under research. A new entity called MOGDEM has been proposed. | [
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] |
967_8 | About animal models, the main animal model for MS, experimental autoimmune encephalomyelitis (EAE) is also an animal model for ADEM. Being an acute monophasic illness, EAE is far more similar to ADEM than MS.
See also
Optic neuritis
Transverse myelitis
Victoria Arlen
References
External links
Information for parents about Acute disseminated encephalomyelitis
Multiple sclerosis
Autoimmune diseases
Central nervous system disorders
Enterovirus-associated diseases
Measles | [
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969_0 | Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements. Ataxia is a clinical manifestation indicating dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum. Ataxia can be limited to one side of the body, which is referred to as hemiataxia. Several possible causes exist for these patterns of neurological dysfunction. Dystaxia is a mild degree of ataxia. Friedreich's ataxia has gait abnormality as the most commonly presented symptom. The word is from Greek α- [a negative prefix] + -τάξις [order] = "lack of order".
Types
Cerebellar
The term cerebellar ataxia is used to indicate ataxia due to dysfunction of the cerebellum. The cerebellum is responsible for integrating a significant amount of neural information that is used to coordinate smoothly ongoing movements and to participate in motor planning. Although ataxia is not present with all cerebellar lesions, many conditions affecting the cerebellum do produce ataxia. People with cerebellar ataxia may have trouble regulating the force, range, direction, velocity, and rhythm of muscle contractions. This results in a characteristic type of irregular, uncoordinated movement that can manifest itself in many possible ways, such as asthenia, asynergy, delayed reaction time, and dyschronometria. Individuals with cerebellar ataxia could also display instability of gait, difficulty with eye movements, dysarthria, dysphagia, hypotonia, dysmetria, and dysdiadochokinesia. These deficits can vary depending on which cerebellar structures have been damaged, and whether the lesion is bi- or unilateral. | [
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969_1 | People with cerebellar ataxia may initially present with poor balance, which could be demonstrated as an inability to stand on one leg or perform tandem gait. As the condition progresses, walking is characterized by a widened base and high stepping, as well as staggering and lurching from side to side. Turning is also problematic and could result in falls. As cerebellar ataxia becomes severe, great assistance and effort are needed to stand and walk. Dysarthria, an impairment with articulation, may also be present and is characterized by "scanning" speech that consists of slower rate, irregular rhythm, and variable volume. Also, slurring of speech, tremor of the voice, and ataxic respiration may occur. Cerebellar ataxia could result with incoordination of movement, particularly in the extremities. Overshooting (or hypermetria) occurs with finger-to-nose testing and heel to shin testing; thus, dysmetria is evident. Impairments with alternating movements (dysdiadochokinesia), as well as dysrhythmia, may also be displayed. Tremor of the head and trunk (titubation) may be seen in individuals with cerebellar ataxia. | [
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969_2 | Dysmetria is thought to be caused by a deficit in the control of interaction torques in multijoint motion. Interaction torques are created at an associated joint when the primary joint is moved. For example, if a movement required reaching to touch a target in front of the body, flexion at the shoulder would create a torque at the elbow, while extension of the elbow would create a torque at the wrist. These torques increase as the speed of movement increases and must be compensated and adjusted for to create coordinated movement. This may, therefore, explain decreased coordination at higher movement velocities and accelerations.
Dysfunction of the vestibulocerebellum (flocculonodular lobe) impairs balance and the control of eye movements. This presents itself with postural instability, in which the person tends to separate his/her feet upon standing, to gain a wider base and to avoid titubation (bodily oscillations tending to be forward-backward ones). The instability is, therefore, worsened when standing with the feet together, regardless of whether the eyes are open or closed. This is a negative Romberg's test, or more accurately, it denotes the individual's inability to carry out the test, because the individual feels unstable even with open eyes.
Dysfunction of the spinocerebellum (vermis and associated areas near the midline) presents itself with a wide-based "drunken sailor" gait (called truncal ataxia), characterised by uncertain starts and stops, lateral deviations, and unequal steps. As a result of this gait impairment, falling is a concern in patients with ataxia. Studies examining falls in this population show that 74–93% of patients have fallen at least once in the past year and up to 60% admit to fear of falling.
'Dysfunction of the cerebrocerebellum' (lateral hemispheres) presents as disturbances in carrying out voluntary, planned movements by the extremities (called appendicular ataxia). These include:
Intention tremor (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes, as well as the limbs and torso)
Peculiar writing abnormalities (large, unequal letters, irregular underlining) | [
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969_3 | Peculiar writing abnormalities (large, unequal letters, irregular underlining)
A peculiar pattern of dysarthria (slurred speech, sometimes characterised by explosive variations in voice intensity despite a regular rhythm)
Inability to perform rapidly alternating movements, known as dysdiadochokinesia, occurs, and could involve rapidly switching from pronation to supination of the forearm. Movements become more irregular with increases of speed.
Inability to judge distances or ranges of movement happens. This dysmetria is often seen as undershooting, hypometria, or overshooting, hypermetria, the required distance or range to reach a target. This is sometimes seen when a patient is asked to reach out and touch someone's finger or touch his or her own nose.
The rebound phenomenon, also known as the loss of the check reflex, is also sometimes seen in patients with cerebellar ataxia, for example, when patients are flexing their elbows isometrically against a resistance. When the resistance is suddenly removed without warning, the patients' arms may swing up and even strike themselves. With an intact check reflex, the patients check and activate the opposing triceps to slow and stop the movement.
Patients may exhibit a constellation of subtle to overt cognitive symptoms, which are gathered under the terminology of Schmahmann's syndrome. | [
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969_4 | Sensory
The term sensory ataxia is used to indicate ataxia due to loss of proprioception, the loss of sensitivity to the positions of joint and body parts. This is generally caused by dysfunction of the dorsal columns of the spinal cord, because they carry proprioceptive information up to the brain. In some cases, the cause of sensory ataxia may instead be dysfunction of the various parts of the brain that receive positional information, including the cerebellum, thalamus, and parietal lobes.
Sensory ataxia presents itself with an unsteady "stomping" gait with heavy heel strikes, as well as a postural instability that is usually worsened when the lack of proprioceptive input cannot be compensated for by visual input, such as in poorly lit environments.
Physicians can find evidence of sensory ataxia during physical examination by having patients stand with their feet together and eyes shut. In affected patients, this will cause the instability to worsen markedly, producing wide oscillations and possibly a fall; this is called a positive Romberg's test. Worsening of the finger-pointing test with the eyes closed is another feature of sensory ataxia. Also, when patients are standing with arms and hands extended toward the physician, if the eyes are closed, the patients' fingers tend to "fall down" and then be restored to the horizontal extended position by sudden muscular contractions (the "ataxic hand").
Vestibular
The term vestibular ataxia is used to indicate ataxia due to dysfunction of the vestibular system, which in acute and unilateral cases is associated with prominent vertigo, nausea, and vomiting. In slow-onset, chronic bilateral cases of vestibular dysfunction, these characteristic manifestations may be absent, and dysequilibrium may be the sole presentation.
Causes
The three types of ataxia have overlapping causes, so can either coexist or occur in isolation. Cerebellar ataxia can have many causes despite normal neuroimaging. | [
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] |
969_5 | Focal lesions
Any type of focal lesion of the central nervous system (such as stroke, brain tumor, multiple sclerosis, inflammatory [such as sarcoidosis], and "chronic lymphocytyc inflammation with pontine perivascular enhancement responsive to steroids syndrome" [CLIPPERS]) will cause the type of ataxia corresponding to the site of the lesion: cerebellar if in the cerebellum; sensory if in the dorsal spinal cord...to include cord compression by thickened ligamentum flavum or stenosis of the boney spinal canal...(and rarely in the thalamus or parietal lobe); or vestibular if in the vestibular system (including the vestibular areas of the cerebral cortex).
Exogenous substances (metabolic ataxia)
Exogenous substances that cause ataxia mainly do so because they have a depressant effect on central nervous system function. The most common example is ethanol (alcohol), which is capable of causing reversible cerebellar and vestibular ataxia. Chronic intake of ethanol causes atrophy of the cerebellum by oxidative and endoplasmic reticulum stresses induced by thiamine deficiency. Other examples include various prescription drugs (e.g. most antiepileptic drugs have cerebellar ataxia as a possible adverse effect), Lithium level over 1.5mEq/L, synthetic cannabinoid HU-211 ingestion and various other medical and recreational drugs (e.g. ketamine, PCP or dextromethorphan, all of which are NMDA receptor antagonists that produce a dissociative state at high doses). A further class of pharmaceuticals which can cause short term ataxia, especially in high doses, are benzodiazepines. Exposure to high levels of methylmercury, through consumption of fish with high mercury concentrations, is also a known cause of ataxia and other neurological disorders.
Radiation poisoning
Ataxia can be induced as a result of severe acute radiation poisoning with an absorbed dose of more than 30 grays.
Vitamin B12 deficiency
Vitamin B12 deficiency may cause, among several neurological abnormalities, overlapping cerebellar and sensory ataxia. | [
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] |
969_6 | Hypothyroidism
Symptoms of neurological dysfunction may be the presenting feature in some patients with hypothyroidism. These include reversible cerebellar ataxia, dementia, peripheral neuropathy, psychosis and coma. Most of the neurological complications improve completely after thyroid hormone replacement therapy.
Causes of isolated sensory ataxia
Peripheral neuropathies may cause generalised or localised sensory ataxia (e.g. a limb only) depending on the extent of the neuropathic involvement. Spinal disorders of various types may cause sensory ataxia from the lesioned level below, when they involve the dorsal columns.
Non-hereditary cerebellar degeneration
Non-hereditary causes of cerebellar degeneration include chronic alcohol use disorder, head injury, paraneoplastic and non-paraneoplastic autoimmune ataxia, high altitude cerebral oedema, coeliac disease, normal pressure hydrocephalus and infectious or post-infectious cerebellitis.
Hereditary ataxias
Ataxia may depend on hereditary disorders consisting of degeneration of the cerebellum or of the spine; most cases feature both to some extent, and therefore present with overlapping cerebellar and sensory ataxia, even though one is often more evident than the other. Hereditary disorders causing ataxia include autosomal dominant ones such as spinocerebellar ataxia, episodic ataxia, and dentatorubropallidoluysian atrophy, as well as autosomal recessive disorders such as Friedreich's ataxia (sensory and cerebellar, with the former predominating) and Niemann Pick disease, ataxia-telangiectasia (sensory and cerebellar, with the latter predominating), and abetalipoproteinaemia. An example of X-linked ataxic condition is the rare fragile X-associated tremor/ataxia syndrome or FXTAS.
Arnold–Chiari malformation (congenital ataxia)
Arnold–Chiari malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of cerebrospinal fluid outflow. | [
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969_7 | Succinic semialdehyde dehydrogenase deficiency
Succinic semialdehyde dehydrogenase deficiency is an autosomal-recessive gene disorder where mutations in the ALDH5A1 gene results in the accumulation of gamma-Hydroxybutyric acid (GHB) in the body. GHB accumulates in the nervous system and can cause ataxia as well as other neurological dysfunction.
Wilson's disease
Wilson's disease is an autosomal-recessive gene disorder whereby an alteration of the ATP7B gene results in an inability to properly excrete copper from the body. Copper accumulates in the nervous system and liver and can cause ataxia as well as other neurological and organ impairments.
Gluten ataxia
Gluten ataxia is an autoimmune disease triggered by the ingestion of gluten. Early diagnosis and treatment with a gluten-free diet can improve ataxia and prevent its progression. The effectiveness of the treatment depends on the elapsed time from the onset of the ataxia until diagnosis, because the death of neurons in the cerebellum as a result of gluten exposure is irreversible. It accounts for 40% of ataxias of unknown origin and 15% of all ataxias. Less than 10% of people with gluten ataxia present any gastrointestinal symptom and only about 40% have intestinal damage. This entity is classified into primary auto-immune cerebellar ataxias (PACA).
Potassium pump
Malfunction of the sodium-potassium pump may be a factor in some ataxias. The - pump has been shown to control and set the intrinsic activity mode of cerebellar Purkinje neurons. This suggests that the pump might not simply be a homeostatic, "housekeeping" molecule for ionic gradients; but could be a computational element in the cerebellum and the brain. Indeed, an ouabain block of - pumps in the cerebellum of a live mouse results in it displaying ataxia and dystonia. Ataxia is observed for lower ouabain concentrations, dystonia is observed at higher ouabain concentrations. | [
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969_8 | Cerebellar ataxia associated with anti-GAD antibodies
Antibodies against the enzyme glutamic acid decarboxylase (GAD: enzyme changing glutamate into GABA) cause cerebellar deficits. The antibodies impair motor learning and cause behavioral deficits.
GAD antibodies related ataxia is part of the group called immune-mediated cerebellar ataxias. The antibodies induce a synaptopathy. The cerebellum is particularly vulnerable to autoimmune disorders. Cerebellar circuitry has capacities to compensate and restore function thanks to cerebellar reserve, gathering multiple forms of plasticity. LTDpathies gather immune disorders targeting long-term depression (LTD), a form of plasticity.
Diagnosis
Imaging studies - A CT scan or MRI of the brain might help determine potential causes. An MRI can sometimes show shrinkage of the cerebellum and other brain structures in people with ataxia. It may also show other treatable findings, such as a blood clot or benign tumour, that could be pressing on the cerebellum.
Lumbar puncture (spinal tap) - A needle is inserted into the lower back (lumbar region) between two lumbar vertebrae to obtain a sample of cerebrospinal fluid for testing.
Genetic testing - Determines whether the mutation that causes one of the hereditary ataxic conditions is present. Tests are available for many but not all of the hereditary ataxias. | [
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969_9 | Treatment
The treatment of ataxia and its effectiveness depend on the underlying cause. Treatment may limit or reduce the effects of ataxia, but it is unlikely to eliminate them entirely. Recovery tends to be better in individuals with a single focal injury (such as stroke or a benign tumour), compared to those who have a neurological degenerative condition. A review of the management of degenerative ataxia was published in 2009. A small number of rare conditions presenting with prominent cerebellar ataxia are amenable to specific treatment and recognition of these disorders is critical. Diseases include vitamin E deficiency, abetalipoproteinemia, cerebrotendinous xanthomatosis, Niemann–Pick type C disease, Refsum's disease, glucose transporter type 1 deficiency, episodic ataxia type 2, gluten ataxia, glutamic acid decarboxylase ataxia. Novel therapies target the RNA defects associated with cerebellar disorders, using in particular anti-sense oligonucleotides.
The movement disorders associated with ataxia can be managed by pharmacological treatments and through physical therapy and occupational therapy to reduce disability. Some drug treatments that have been used to control ataxia include: 5-hydroxytryptophan (5-HTP), idebenone, amantadine, physostigmine, L-carnitine or derivatives, trimethoprim/sulfamethoxazole, vigabatrin, phosphatidylcholine, acetazolamide, 4-aminopyridine, buspirone, and a combination of coenzyme Q10 and vitamin E.
Physical therapy requires a focus on adapting activity and facilitating motor learning for retraining specific functional motor patterns. A recent systematic review suggested that physical therapy is effective, but there is only moderate evidence to support this conclusion. The most commonly used physical therapy interventions for cerebellar ataxia are vestibular habituation, Frenkel exercises, proprioceptive neuromuscular facilitation (PNF), and balance training; however, therapy is often highly individualized and gait and coordination training are large components of therapy. | [
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969_10 | Current research suggests that, if a person is able to walk with or without a mobility aid, physical therapy should include an exercise program addressing five components: static balance, dynamic balance, trunk-limb coordination, stairs, and contracture prevention. Once the physical therapist determines that the individual is able to safely perform parts of the program independently, it is important that the individual be prescribed and regularly engage in a supplementary home exercise program that incorporates these components to further improve long term outcomes. These outcomes include balance tasks, gait, and individual activities of daily living. While the improvements are attributed primarily to changes in the brain and not just the hip or ankle joints, it is still unknown whether the improvements are due to adaptations in the cerebellum or compensation by other areas of the brain.
Decomposition, simplification, or slowing of multijoint movement may also be an effective strategy that therapists may use to improve function in patients with ataxia. Training likely needs to be intense and focused—as indicated by one study performed with stroke patients experiencing limb ataxia who underwent intensive upper limb retraining. Their therapy consisted of constraint-induced movement therapy which resulted in improvements of their arm function. Treatment should likely include strategies to manage difficulties with everyday activities such as walking. Gait aids (such as a cane or walker) can be provided to decrease the risk of falls associated with impairment of balance or poor coordination. Severe ataxia may eventually lead to the need for a wheelchair. To obtain better results, possible coexisting motor deficits need to be addressed in addition to those induced by ataxia. For example, muscle weakness and decreased endurance could lead to increasing fatigue and poorer movement patterns. | [
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969_11 | There are several assessment tools available to therapists and health care professionals working with patients with ataxia. The International Cooperative Ataxia Rating Scale (ICARS) is one of the most widely used and has been proven to have very high reliability and validity. Other tools that assess motor function, balance and coordination are also highly valuable to help the therapist track the progress of their patient, as well as to quantify the patient's functionality. These tests include, but are not limited to:
The Berg Balance Scale
Tandem Walking (to test for Tandem gaitability)
Scale for the Assessment and Rating of Ataxia (SARA)
tapping tests – The person must quickly and repeatedly tap their arm or leg while the therapist monitors the amount of dysdiadochokinesia.
finger-nose testing – This test has several variations including finger-to-therapist's finger, finger-to-finger, and alternate nose-to-finger.
Industry Insights
According to the report published by the Facts and Factors, global demand for the ataxia market was estimated at approximately USD 29,401.1 Million in 2020 and is expected to generate revenue of around USD 46,000.8 Million by the end of 2026, growing at a CAGR of around 10.2% between 2021 and 2026.
Other uses
The term "ataxia" is sometimes used in a broader sense to indicate lack of coordination in some physiological process. Examples include optic ataxia (lack of coordination between visual inputs and hand movements, resulting in inability to reach and grab objects) and ataxic respiration (lack of coordination in respiratory movements, usually due to dysfunction of the respiratory centres in the medulla oblongata). Optic ataxia may be caused by lesions to the posterior parietal cortex, which is responsible for combining and expressing positional information and relating it to movement. Outputs of the posterior parietal cortex include the spinal cord, brain stem motor pathways, pre-motor and pre-frontal cortex, basal ganglia and the cerebellum. Some neurons in the posterior parietal cortex are modulated by intention. Optic ataxia is usually part of Balint's syndrome, but can be seen in isolation with injuries to the superior parietal lobule, as it represents a disconnection between visual-association cortex and the frontal premotor and motor cortex. | [
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969_12 | See also
Ataxic cerebral palsy
Spinocerebellar ataxia
Bruns apraxia
References
Further reading
External links
Symptoms and signs: Nervous system
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974_0 | Augusta Ada King, Countess of Lovelace (née Byron; 10 December 1815 – 27 November 1852) was an English mathematician and writer, chiefly known for her work on Charles Babbage's proposed mechanical general-purpose computer, the Analytical Engine. She was the first to recognise that the machine had applications beyond pure calculation, and to have published the first algorithm intended to be carried out by such a machine. As a result, she is often regarded as the first computer programmer.
Ada Byron was the only child of poet Lord Byron and mathematician Lady Byron. All of Byron's other children were born out of wedlock to other women. Byron separated from his wife a month after Ada was born and left England forever. Four months later, he commemorated the parting in a poem that begins, "Is thy face like thy mother's my fair child! ADA! sole daughter of my house and heart?". He died in Greece when Ada was eight years old. Her mother remained bitter and promoted Ada's interest in mathematics and logic in an effort to prevent her from developing her father's perceived insanity. Despite this, Ada remained interested in him, naming her two sons Byron and Gordon. Upon her death, she was buried next to him at her request. Although often ill in her childhood, Ada pursued her studies assiduously. She married William King in 1835. King was made Earl of Lovelace in 1838, Ada thereby becoming Countess of Lovelace.
Her educational and social exploits brought her into contact with scientists such as Andrew Crosse, Charles Babbage, Sir David Brewster, Charles Wheatstone, Michael Faraday and the author Charles Dickens, contacts which she used to further her education. Ada described her approach as "poetical science" and herself as an "Analyst (& Metaphysician)".
When she was a teenager (18), her mathematical talents led her to a long working relationship and friendship with fellow British mathematician Charles Babbage, who is known as "the father of computers". She was in particular interested in Babbage's work on the Analytical Engine. Lovelace first met him in June 1833, through their mutual friend, and her private tutor, Mary Somerville. | [
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974_1 | Between 1842 and 1843, Ada translated an article by Italian military engineer Luigi Menabrea about the Analytical Engine, supplementing it with an elaborate set of notes, simply called "Notes". Lovelace's notes are important in the early history of computers, containing what many consider to be the first computer program—that is, an algorithm designed to be carried out by a machine. Other historians reject this perspective and point out that Babbage's personal notes from the years 1836/1837 contain the first programs for the engine. She also developed a vision of the capability of computers to go beyond mere calculating or number-crunching, while many others, including Babbage himself, focused only on those capabilities. Her mindset of "poetical science" led her to ask questions about the Analytical Engine (as shown in her notes) examining how individuals and society relate to technology as a collaborative tool.
She died of uterine cancer in 1852 at the age of 36, the same age at which her father died.
Biography
Childhood
Lord Byron expected his child to be a "glorious boy" and was disappointed when Lady Byron gave birth to a girl. The child was named after Byron's half-sister, Augusta Leigh, and was called "Ada" by Byron himself. On 16 January 1816, at Lord Byron's command, Lady Byron left for her parents' home at Kirkby Mallory, taking their five-week-old daughter with her. Although English law at the time granted full custody of children to the father in cases of separation, Lord Byron made no attempt to claim his parental rights, but did request that his sister keep him informed of Ada's welfare.
On 21 April, Lord Byron signed the deed of separation, although very reluctantly, and left England for good a few days later. Aside from an acrimonious separation, Lady Byron continued throughout her life to make allegations about her husband's immoral behaviour. This set of events made Lovelace infamous in Victorian society. Ada did not have a relationship with her father. He died in 1824 when she was eight years old. Her mother was the only significant parental figure in her life. Lovelace was not shown the family portrait of her father until her 20th birthday. | [
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974_2 | Lovelace did not have a close relationship with her mother. She was often left in the care of her maternal grandmother Judith, Hon. Lady Milbanke, who doted on her. However, because of societal attitudes of the time—which favoured the husband in any separation, with the welfare of any child acting as mitigation—Lady Byron had to present herself as a loving mother to the rest of society. This included writing anxious letters to Lady Milbanke about her daughter's welfare, with a cover note saying to retain the letters in case she had to use them to show maternal concern. In one letter to Lady Milbanke, she referred to her daughter as "it": "I talk to it for your satisfaction, not my own, and shall be very glad when you have it under your own." Lady Byron had her teenage daughter watched by close friends for any sign of moral deviation. Lovelace dubbed these observers the "Furies" and later complained they exaggerated and invented stories about her.
Lovelace was often ill, beginning in early childhood. At the age of eight, she experienced headaches that obscured her vision. In June 1829, she was paralyzed after a bout of measles. She was subjected to continuous bed rest for nearly a year, something which may have extended her period of disability. By 1831, she was able to walk with crutches. Despite the illnesses, she developed her mathematical and technological skills.
Ada Byron had an affair with a tutor in early 1833. She tried to elope with him after she was caught, but the tutor's relatives recognised her and contacted her mother. Lady Byron and her friends covered the incident up to prevent a public scandal. Lovelace never met her younger half-sister, Allegra, the daughter of Lord Byron and Claire Clairmont. Allegra died in 1822 at the age of five. Lovelace did have some contact with Elizabeth Medora Leigh, the daughter of Byron's half-sister Augusta Leigh, who purposely avoided Lovelace as much as possible when introduced at court.
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974_3 | Adult years
Lovelace became close friends with her tutor Mary Somerville, who introduced her to Charles Babbage in 1833. She had a strong respect and affection for Somerville, and they corresponded for many years. Other acquaintances included the scientists Andrew Crosse, Sir David Brewster, Charles Wheatstone, Michael Faraday and the author Charles Dickens. She was presented at Court at the age of seventeen "and became a popular belle of the season" in part because of her "brilliant mind." By 1834 Ada was a regular at Court and started attending various events. She danced often and was able to charm many people, and was described by most people as being dainty, although John Hobhouse, Byron's friend, described her as "a large, coarse-skinned young woman but with something of my friend's features, particularly the mouth". This description followed their meeting on 24 February 1834 in which Ada made it clear to Hobhouse that she did not like him, probably due to her mother's influence, which led her to dislike all of her father's friends. This first impression was not to last, and they later became friends.
On 8 July 1835, she married William, 8th Baron King, becoming Lady King. They had three homes: Ockham Park, Surrey; a Scottish estate on Loch Torridon in Ross-shire; and a house in London. They spent their honeymoon at Worthy Manor in Ashley Combe near Porlock Weir, Somerset. The Manor had been built as a hunting lodge in 1799 and was improved by King in preparation for their honeymoon. It later became their summer retreat and was further improved during this time. From 1845, the family's main house was Horsley Towers, built in the Tudorbethan fashion by the architect of the Houses of Parliament, Charles Barry, and later greatly enlarged to Lovelace's own designs. | [
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974_4 | They had three children: Byron (born 1836); Anne Isabella (called Annabella, born 1837); and Ralph Gordon (born 1839). Immediately after the birth of Annabella, Lady King experienced "a tedious and suffering illness, which took months to cure." Ada was a descendant of the extinct Barons Lovelace and in 1838, her husband was made Earl of Lovelace and Viscount Ockham, meaning Ada became the Countess of Lovelace. In 1843–44, Ada's mother assigned William Benjamin Carpenter to teach Ada's children and to act as a "moral" instructor for Ada. He quickly fell for her and encouraged her to express any frustrated affections, claiming that his marriage meant he would never act in an "unbecoming" manner. When it became clear that Carpenter was trying to start an affair, Ada cut it off.
In 1841, Lovelace and Medora Leigh (the daughter of Lord Byron's half-sister Augusta Leigh) were told by Ada's mother that Ada's father was also Medora's father. On 27 February 1841, Ada wrote to her mother: "I am not in the least astonished. In fact, you merely confirm what I have for years and years felt scarcely a doubt about, but should have considered it most improper in me to hint to you that I in any way suspected." She did not blame the incestuous relationship on Byron, but instead blamed Augusta Leigh: "I fear she is more inherently wicked than he ever was." In the 1840s, Ada flirted with scandals: firstly, from a relaxed approach to extra-marital relationships with men, leading to rumours of affairs; and secondly, from her love of gambling. She apparently lost more than £3,000 on the horses during the later 1840s. The gambling led to her forming a syndicate with male friends, and an ambitious attempt in 1851 to create a mathematical model for successful large bets. This went disastrously wrong, leaving her thousands of pounds in debt to the syndicate, forcing her to admit it all to her husband. She had a shadowy relationship with Andrew Crosse's son John from 1844 onwards. John Crosse destroyed most of their correspondence after her death as part of a legal agreement. She bequeathed him the only heirlooms her father had personally left to her. During her final illness, she would panic at the idea of the younger Crosse being kept from visiting her. | [
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974_5 | Education
From 1832, when she was seventeen, her mathematical abilities began to emerge, and her interest in mathematics dominated the majority of her adult life. Her mother's obsession with rooting out any of the insanity of which she accused Byron was one of the reasons that Ada was taught mathematics from an early age. She was privately educated in mathematics and science by William Frend, William King, and Mary Somerville, the noted 19th-century researcher and scientific author. In the 1840s, the mathematician Augustus De Morgan extended her "much help in her mathematical studies" including study of advanced calculus topics including the "numbers of Bernoulli" (that formed her celebrated algorithm for Babbage's Analytical Engine). In a letter to Lady Byron, De Morgan suggested that Ada's skill in mathematics might lead her to become "an original mathematical investigator, perhaps of first-rate eminence."
Lovelace often questioned basic assumptions through integrating poetry and science. Whilst studying differential calculus, she wrote to De Morgan:
I may remark that the curious transformations many formulae can undergo, the unsuspected and to a beginner apparently impossible identity of forms exceedingly dissimilar at first sight, is I think one of the chief difficulties in the early part of mathematical studies. I am often reminded of certain sprites and fairies one reads of, who are at one's elbows in one shape now, and the next minute in a form most dissimilar.
Lovelace believed that intuition and imagination were critical to effectively applying mathematical and scientific concepts. She valued metaphysics as much as mathematics, viewing both as tools for exploring "the unseen worlds around us."
Death | [
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974_6 | Death
Lovelace died at the age of 36 on 27 November 1852, from uterine cancer. The illness lasted several months, in which time Annabella took command over whom Ada saw, and excluded all of her friends and confidants. Under her mother's influence, Ada had a religious transformation and was coaxed into repenting of her previous conduct and making Annabella her executor. She lost contact with her husband after confessing something to him on 30 August which caused him to abandon her bedside. It is not known what she told him. She was buried, at her request, next to her father at the Church of St. Mary Magdalene in Hucknall, Nottinghamshire. A memorial plaque, written in Latin, to her and her father is in the chapel attached to Horsley Towers.
Work
Throughout her life, Lovelace was strongly interested in scientific developments and fads of the day, including phrenology and mesmerism. After her work with Babbage, Lovelace continued to work on other projects. In 1844, she commented to a friend Woronzow Greig about her desire to create a mathematical model for how the brain gives rise to thoughts and nerves to feelings ("a calculus of the nervous system"). She never achieved this, however. In part, her interest in the brain came from a long-running pre-occupation, inherited from her mother, about her "potential" madness. As part of her research into this project, she visited the electrical engineer Andrew Crosse in 1844 to learn how to carry out electrical experiments. In the same year, she wrote a review of a paper by Baron Karl von Reichenbach, Researches on Magnetism, but this was not published and does not appear to have progressed past the first draft. In 1851, the year before her cancer struck, she wrote to her mother mentioning "certain productions" she was working on regarding the relation of maths and music.
Lovelace first met Charles Babbage in June 1833, through their mutual friend Mary Somerville. Later that month, Babbage invited Lovelace to see the prototype for his difference engine. She became fascinated with the machine and used her relationship with Somerville to visit Babbage as often as she could. Babbage was impressed by Lovelace's intellect and analytic skills. He called her "The Enchantress of Number." In 1843, he wrote to her: | [
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974_7 | During a nine-month period in 1842–43, Lovelace translated the Italian mathematician Luigi Menabrea's article on Babbage's newest proposed machine, the Analytical Engine. With the article, she appended a set of notes. Explaining the Analytical Engine's function was a difficult task, as many other scientists did not really grasp the concept and the British establishment had shown little interest in it. Lovelace's notes even had to explain how the Analytical Engine differed from the original Difference Engine. Her work was well received at the time; the scientist Michael Faraday described himself as a supporter of her writing.
The notes are around three times longer than the article itself and include (in Note G), in complete detail, a method for calculating a sequence of Bernoulli numbers using the Analytical Engine, which might have run correctly had it ever been built (only Babbage's Difference Engine has been built, completed in London in 2002). Based on this work, Lovelace is now considered by many to be the first computer programmer and her method has been called the world's first computer program. Others dispute this because some of Charles Babbage's earlier writings could be considered computer programs.
Note G also contains Lovelace's dismissal of artificial intelligence. She wrote that "The Analytical Engine has no pretensions whatever to originate anything. It can do whatever we know how to order it to perform. It can follow analysis; but it has no power of anticipating any analytical relations or truths." This objection has been the subject of much debate and rebuttal, for example by Alan Turing in his paper "Computing Machinery and Intelligence". | [
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974_8 | Lovelace and Babbage had a minor falling out when the papers were published, when he tried to leave his own statement (criticising the government's treatment of his Engine) as an unsigned preface, which could have been mistakenly interpreted as a joint declaration. When Taylor's Scientific Memoirs ruled that the statement should be signed, Babbage wrote to Lovelace asking her to withdraw the paper. This was the first that she knew he was leaving it unsigned, and she wrote back refusing to withdraw the paper. The historian Benjamin Woolley theorised that "His actions suggested he had so enthusiastically sought Ada's involvement, and so happily indulged her ... because of her 'celebrated name'." Their friendship recovered, and they continued to correspond. On 12 August 1851, when she was dying of cancer, Lovelace wrote to him asking him to be her executor, though this letter did not give him the necessary legal authority. Part of the terrace at Worthy Manor was known as Philosopher's Walk, as it was there that Lovelace and Babbage were reputed to have walked while discussing mathematical principles.
First computer program
In 1840, Babbage was invited to give a seminar at the University of Turin about his Analytical Engine. Luigi Menabrea, a young Italian engineer and the future Prime Minister of Italy, transcribed Babbage's lecture into French, and this transcript was subsequently published in the Bibliothèque universelle de Genève in October 1842.
Babbage's friend Charles Wheatstone commissioned Ada Lovelace to translate Menabrea's paper into English. She then augmented the paper with notes, which were added to the translation. Ada Lovelace spent the better part of a year doing this, assisted with input from Babbage. These notes, which are more extensive than Menabrea's paper, were then published in the September 1843 edition of Taylor's Scientific Memoirs under the initialism AAL.
Ada Lovelace's notes were labelled alphabetically from A to G. In note G, she describes an algorithm for the Analytical Engine to compute Bernoulli numbers. It is considered to be the first published algorithm ever specifically tailored for implementation on a computer, and Ada Lovelace has often been cited as the first computer programmer for this reason. The engine was never completed so her program was never tested. | [
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974_9 | In 1953, more than a century after her death, Ada Lovelace's notes on Babbage's Analytical Engine were republished as an appendix to B. V. Bowden's Faster than Thought: A Symposium on Digital Computing Machines. The engine has now been recognised as an early model for a computer and her notes as a description of a computer and software.
Insight into potential of computing devices
In her notes, Ada Lovelace emphasised the difference between the Analytical Engine and previous calculating machines, particularly its ability to be programmed to solve problems of any complexity. She realised the potential of the device extended far beyond mere number crunching. In her notes, she wrote:
This analysis was an important development from previous ideas about the capabilities of computing devices and anticipated the implications of modern computing one hundred years before they were realised. Walter Isaacson ascribes Ada's insight regarding the application of computing to any process based on logical symbols to an observation about textiles: "When she saw some mechanical looms that used punchcards to direct the weaving of beautiful patterns, it reminded her of how Babbage's engine used punched cards to make calculations." This insight is seen as significant by writers such as Betty Toole and Benjamin Woolley, as well as the programmer John Graham-Cumming, whose project Plan 28 has the aim of constructing the first complete Analytical Engine.
According to the historian of computing and Babbage specialist Doron Swade: Ada saw something that Babbage in some sense failed to see. In Babbage's world his engines were bound by number...What Lovelace saw...was that number could represent entities other than quantity. So once you had a machine for manipulating numbers, if those numbers represented other things, letters, musical notes, then the machine could manipulate symbols of which number was one instance, according to rules. It is this fundamental transition from a machine which is a number cruncher to a machine for manipulating symbols according to rules that is the fundamental transition from calculation to computation—to general-purpose computation—and looking back from the present high ground of modern computing, if we are looking and sifting history for that transition, then that transition was made explicitly by Ada in that 1843 paper.
Controversy over contribution
Though Lovelace is often referred to as the first computer programmer, some biographers, computer scientists and historians of computing claim otherwise. | [
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974_10 | Allan G. Bromley, in the 1990 article Difference and Analytical Engines:
Bruce Collier, who later wrote a biography of Babbage, wrote in his 1970 Harvard University PhD thesis that Lovelace "made a considerable contribution to publicizing the Analytical Engine, but there is no evidence that she advanced the design or theory of it in any way".
Eugene Eric Kim and Betty Alexandra Toole consider it "incorrect" to regard Lovelace as the first computer programmer, as Babbage wrote the initial programs for his Analytical Engine, although the majority were never published. Bromley notes several dozen sample programs prepared by Babbage between 1837 and 1840, all substantially predating Lovelace's notes. Dorothy K. Stein regards Lovelace's notes as "more a reflection of the mathematical uncertainty of the author, the political purposes of the inventor, and, above all, of the social and cultural context in which it was written, than a blueprint for a scientific development."
Doron Swade, a specialist on history of computing known for his work on Babbage, discussed Lovelace during a lecture on Babbage's analytical engine. He explained that Ada was only a "promising beginner" instead of genius in mathematics, that she began studying basic concepts of mathematics five years after Babbage conceived the analytical engine so she could not have made important contributions to it, and that she only published the first computer program instead of actually writing it. But he agrees that Ada was the only person to see the potential of the analytical engine as a machine capable of expressing entities other than quantities.
In his self-published book, Idea Makers, Stephen Wolfram defends Lovelace's contributions. While acknowledging that Babbage wrote several unpublished algorithms for the Analytical Engine prior to Lovelace's notes, Wolfram argues that "there's nothing as sophisticated—or as clean—as Ada's computation of the Bernoulli numbers. Babbage certainly helped and commented on Ada's work, but she was definitely the driver of it." Wolfram then suggests that Lovelace's main achievement was to distill from Babbage's correspondence "a clear exposition of the abstract operation of the machine—something which Babbage never did."
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974_11 | In popular culture
1810s
Lord Byron wrote the poem "Fare Thee Well" to his wife Lady Byron in 1816, following their separation after the birth of Ada Lovelace. In the poem he writes:
And when thou would'st solace gather—
When our child's first accents flow—
Wilt thou teach her to say "Father!"
Though his care she must forego?
When her little hands shall press thee—
When her lip to thine is pressed—
Think of him whose prayer shall bless thee—
Think of him thy love had blessed!
Should her lineaments resemble
Those thou never more may'st see,
Then thy heart will softly tremble
With a pulse yet true to me.
1970s
Lovelace is portrayed in Romulus Linney's 1977 play Childe Byron.
1990s
In the 1990 steampunk novel The Difference Engine by William Gibson and Bruce Sterling, Lovelace delivers a lecture on the "punched cards" programme which proves Gödel's incompleteness theorems decades before their actual discovery.
In the 1997 film Conceiving Ada, a computer scientist obsessed with Ada finds a way of communicating with her in the past by means of "undying information waves".
In Tom Stoppard's 1993 play Arcadia, the precocious teenage genius Thomasina Coverly—a character "apparently based" on Ada Lovelace (the play also involves Lord Byron)—comes to understand chaos theory, and theorises the second law of thermodynamics, before either is officially recognised.
2000s
Lovelace features in John Crowley's 2005 novel, Lord Byron's Novel: The Evening Land, as an unseen character whose personality is forcefully depicted in her annotations and anti-heroic efforts to archive her father's lost novel.
2010s
The 2015 play Ada and the Engine by Lauren Gunderson portrays Lovelace and Charles Babbage in unrequited love, and it imagines a post-death meeting between Lovelace and her father.
Lovelace and Babbage are the main characters in Sydney Padua's webcomic and graphic novel The Thrilling Adventures of Lovelace and Babbage. The comic features extensive footnotes on the history of Ada Lovelace, and many lines of dialogue are drawn from actual correspondence. | [
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974_12 | Lovelace and Mary Shelley as teenagers are the central characters in Jordan Stratford's steampunk series, The Wollstonecraft Detective Agency.
Lovelace, identified as Ada Augusta Byron, is portrayed by Lily Lesser in the second season of The Frankenstein Chronicles. She is employed as an "analyst" to provide the workings of a life-sized humanoid automaton. The brass workings of the machine are reminiscent of Babbage's analytical engine. Her employment is described as keeping her occupied until she returns to her studies in advanced mathematics.
Lovelace and Babbage appear as characters in the second season of the ITV series Victoria (2017). Emerald Fennell portrays Lovelace in the episode, "The Green-Eyed Monster."
The Cardano cryptocurrency platform, which was launched in 2017, uses Ada as the name for their cryptocurrency and Lovelace as the smallest sub-unit of an Ada.
"Lovelace" is the name given to the operating system designed by the character Cameron Howe in Halt and Catch Fire.
Lovelace is a primary character in the 2019 Big Finish Doctor Who audio play The Enchantress of Numbers, starring Tom Baker as the Fourth Doctor and Jane Slavin as his current companion, WPC Ann Kelso. Lovelace is played by Finty Williams.
In 2019, Lovelace is a featured character in the play STEM FEMMES by Philadelphia theater company Applied Mechanics.
2020s
Lovelace features as a character in "Spyfall, Part 2", the second episode of Doctor Who, series 12, which first aired on BBC One on 5 January 2020. The character was portrayed by Sylvie Briggs, alongside characterisations of Charles Babbage and Noor Inayat Khan. In 2021, Nvidia named their upcoming GPU architecture (to be released in 2022), "Ada Lovelace", after her.
Commemoration
The computer language Ada, created on behalf of the United States Department of Defense, was named after Lovelace. The reference manual for the language was approved on 10 December 1980 and the Department of Defense Military Standard for the language, MIL-STD-1815, was given the number of the year of her birth. | [
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974_13 | In 1981, the Association for Women in Computing inaugurated its Ada Lovelace Award. Since 1998, the British Computer Society (BCS) has awarded the Lovelace Medal, and in 2008 initiated an annual competition for women students. BCSWomen sponsors the Lovelace Colloquium, an annual conference for women undergraduates. Ada College is a further-education college in Tottenham Hale, London, focused on digital skills.
Ada Lovelace Day is an annual event celebrated on the second Tuesday of October, which began in 2009. Its goal is to "... raise the profile of women in science, technology, engineering, and maths," and to "create new role models for girls and women" in these fields. Events have included Wikipedia edit-a-thons with the aim of improving the representation of women on Wikipedia in terms of articles and editors to reduce unintended gender bias on Wikipedia. The Ada Initiative was a non-profit organisation dedicated to increasing the involvement of women in the free culture and open source movements.
The Engineering in Computer Science and Telecommunications College building in Zaragoza University is called the Ada Byron Building. The computer centre in the village of Porlock, near where Lovelace lived, is named after her. Ada Lovelace House is a council-owned building in Kirkby-in-Ashfield, Nottinghamshire, near where Lovelace spent her infancy.
In 2012, a Google Doodle and blog post honoured her on her birthday.
In 2013, Ada Developers Academy was founded and named after her. The mission of Ada Developers Academy is to diversify tech by providing women and gender diverse people the skills, experience, and community support to become professional software developers to change the face of tech.
On 17 September 2013, an episode of Great Lives about Ada Lovelace aired.
As of November 2015, all new British passports have included an illustration of Lovelace and Babbage.
In 2017, a Google Doodle honoured her with other women on International Women's Day.
On 2 February 2018, Satellogic, a high-resolution Earth observation imaging and analytics company, launched a ÑuSat type micro-satellite named in honour of Ada Lovelace.
In March 2018, The New York Times published a belated obituary for Ada Lovelace. | [
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] |
974_14 | On 27 July 2018, Senator Ron Wyden submitted, in the United States Senate, the designation of 9 October 2018 as National Ada Lovelace Day: "To honor the life and contributions of Ada Lovelace as a leading woman in science and mathematics". The resolution (S.Res.592) was considered, and agreed to without amendment and with a preamble by unanimous consent.
In November 2020 it was announced that Trinity College Dublin whose library had previously held forty busts, all of them of men, was commissioning four new busts of women, one of whom was to be Lovelace.
Bicentenary
The bicentenary of Ada Lovelace's birth was celebrated with a number of events, including:
The Ada Lovelace Bicentenary Lectures on Computability, Israel Institute for Advanced Studies, 20 December 2015 – 31 January 2016.
Ada Lovelace Symposium, University of Oxford, 13–14 October 2015.
Ada.Ada.Ada, a one-woman show about the life and work of Ada Lovelace (using an LED dress), premiered at Edinburgh International Science Festival on 11 April 2015, and continues to touring internationally to promote diversity on STEM at technology conferences, businesses, government and educational organisations.
Special exhibitions were displayed by the Science Museum in London, England and the Weston Library (part of the Bodleian Library) in Oxford, England.
Publications
Lovelace, Ada King. Ada, the Enchantress of Numbers: A Selection from the Letters of Lord Byron's Daughter and her Description of the First Computer. Mill Valley, CA: Strawberry Press, 1992. .
Publication history
Six copies of the 1843 first edition of Sketch of the Analytical Engine with Ada Lovelace's "Notes" have been located. Three are held at Harvard University, one at the University of Oklahoma, and one at the United States Air Force Academy. On 20 July 2018, the sixth copy was sold at auction to an anonymous buyer for £95,000. A digital facsimile of one of the copies in the Harvard University Library is available online.
In December 2016, a letter written by Ada Lovelace was forfeited by Martin Shkreli to the New York State Department of Taxation and Finance for unpaid taxes owed by Shkreli.
See also | [
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] |
974_15 | See also
Ai-Da (robot)
Code: Debugging the Gender Gap
List of pioneers in computer science
Timeline of women in science
Women in computing
Women in STEM fields
Explanatory notes
References
General sources
.
.
.
.
.
.
.
With notes upon the memoir by the translator.
Miller, Clair Cain. "Ada Lovelace, 1815–1852," New York Times, 8 March 2018.
.
.
.
.
.
.
.
Further reading
Miranda Seymour, In Byron's Wake: The Turbulent Lives of Byron's Wife and Daughter: Annabella Milbanke and Ada Lovelace, Pegasus, 2018, 547 pp.
Christopher Hollings, Ursula Martin, and Adrian Rice, Ada Lovelace: The Making of a Computer Scientist, Bodleian Library, 2018, 114 pp.
Jenny Uglow, "Stepping Out of Byron's Shadow", The New York Review of Books, vol. LXV, no. 18 (22 November 2018), pp. 30–32.
Jennifer Chiaverini, Enchantress of Numbers, Dutton, 2017, 426 pp.
External links
"Ada's Army gets set to rewrite history at Inspirefest 2018" by Luke Maxwell, 4 August 2018
"Untangling the Tale of Ada Lovelace" by Stephen Wolfram, December 2015
1815 births
1852 deaths
19th-century British women scientists
19th-century British writers
19th-century English mathematicians
19th-century English women writers
19th-century British inventors
19th-century English nobility
Ada (programming language)
British countesses
British women computer scientists
British women mathematicians
Burials in Nottinghamshire
Ada
Women computer scientists
Computer designers
Daughters of barons
Deaths from cancer in England
Deaths from uterine cancer
English computer programmers
English people of Scottish descent
English women poets
Lord Byron
Mathematicians from London
Women of the Victorian era
Burials at the Church of St Mary Magdalene, Hucknall | [
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980_0 | August William Derleth (February 24, 1909 – July 4, 1971) was an American writer and anthologist. Though best remembered as the first book publisher of the writings of H. P. Lovecraft, and for his own contributions to the Cthulhu Mythos and the cosmic horror genre, as well as his founding of the publisher Arkham House (which did much to bring supernatural fiction into print in hardcover in the US that had only been readily available in the UK), Derleth was a leading American regional writer of his day, as well as prolific in several other genres, including historical fiction, poetry, detective fiction, science fiction, and biography.
A 1938 Guggenheim Fellow, Derleth considered his most serious work to be the ambitious Sac Prairie Saga, a series of fiction, historical fiction, poetry, and non-fiction naturalist works designed to memorialize life in the Wisconsin he knew. Derleth can also be considered a pioneering naturalist and conservationist in his writing.
Life
The son of William Julius Derleth and Rose Louise Volk, Derleth grew up in Sauk City, Wisconsin. He was educated in local parochial and public high school. Derleth wrote his first fiction at age 13. He was interested most in reading, and he made three trips to the library a week. He would save his money to buy books (his personal library exceeded 12,000 later on in life). Some of his biggest influences were Ralph Waldo Emerson's essays, Walt Whitman, H. L. Mencken's The American Mercury, Samuel Johnson's The History of Rasselas, Prince of Abissinia, Alexandre Dumas, Edgar Allan Poe, Walter Scott, and Henry David Thoreau's Walden.
Forty rejected stories and three years later, according to anthologist Jim Stephens, he sold his first story, "Bat's Belfry", to Weird Tales magazine. Derleth wrote throughout his four years at the University of Wisconsin, where he received a B.A. in 1930. During this time he also served briefly as associate editor of Minneapolis-based Fawcett Publications Mystic Magazine. | [
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980_1 | Returning to Sauk City in the summer of 1931, Derleth worked in a local canning factory and collaborated with childhood friend Mark Schorer (later Chairman of the University of California, Berkeley English Department). They rented a cabin, writing Gothic and other horror stories and selling them
to Weird Tales magazine. Derleth won a place on the O'Brien Roll of Honor for Five Alone, published in Place of Hawks, but was first found in Pagany magazine.
As a result of his early work on the Sac Prairie Saga, Derleth was awarded the prestigious Guggenheim Fellowship; his sponsors were Helen C. White, Nobel Prize-winning novelist Sinclair Lewis and poet Edgar Lee Masters of Spoon River Anthology fame.
In the mid-1930s, Derleth organized a Ranger's Club for young people, served as clerk and president of the local school board, served as a parole officer, organized a local men's club and a parent-teacher association. He also lectured in American regional literature at the University of Wisconsin and was a contributing editor of Outdoors Magazine.
With longtime friend Donald Wandrei, Derleth in 1939 founded Arkham House. Its initial objective was to publish the works of H. P. Lovecraft, with whom Derleth had corresponded since his teenage years. At the same time, he began teaching a course in American Regional Literature at the University of Wisconsin.
In 1941, he became literary editor of The Capital Times newspaper in Madison, a post he held until his resignation in 1960. His hobbies included fencing, swimming, chess, philately and comic-strips (Derleth reportedly used the funding from his Guggenheim Fellowship to bind his comic book collection, most recently valued in the millions of dollars, rather than to travel abroad as the award intended.). Derleth's true avocation, however, was hiking the terrain of his native Wisconsin lands, and observing and recording nature with an expert eye.
Derleth once wrote of his writing methods, "I write very swiftly, from 750,000 to a million words yearly, very little of it pulp material."
In 1948, he was elected president of the Associated Fantasy Publishers at the 6th World Science Fiction Convention in Toronto. | [
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980_2 | He was married April 6, 1953, to Sandra Evelyn Winters. They divorced six years later. Derleth retained custody of the couple's two children, April Rose and Walden William. April earned a Bachelor of Arts degree in English from the University of Wisconsin-Madison in 1977. She became majority stockholder, President, and CEO of Arkham House in 1994. She remained in that capacity until her death. She was known in the community as a naturalist and humanitarian. April died on March 21, 2011.
In 1960, Derleth began editing and publishing a magazine called Hawk and Whippoorwill, dedicated to poems of man and nature.
Derleth died of a heart attack on July 4, 1971, and is buried in St. Aloysius Cemetery in Sauk City. The U.S. 12 bridge over the Wisconsin River is named in his honor. Derleth was Roman Catholic.
Career
Derleth wrote more than 150 short stories and more than 100 books during his lifetime.
The Sac Prairie Saga
Derleth wrote an expansive series of novels, short stories, journals, poems, and other works about Sac Prairie (whose prototype is Sauk City). Derleth intended this series to comprise up to 50 novels telling the projected life-story of the region from the 19th century onwards, with analogies to Balzac's Human Comedy and Proust's Remembrance of Things Past.
This, and other early work by Derleth, made him a well-known figure among the regional literary figures of his time: early Pulitzer Prize winners Hamlin Garland and Zona Gale, as well as Sinclair Lewis, the last both an admirer and critic of Derleth. | [
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980_3 | As Edward Wagenknecht wrote in Cavalcade of the American Novel, "What Mr. Derleth has that is lacking...in modern novelists generally, is a country. He belongs. He writes of a land and a people that are bone of his bone and flesh of his flesh. In his fictional world, there is a unity much deeper and more fundamental than anything that can be conferred by an ideology. It is clear, too, that he did not get the best, and most fictionally useful, part of his background material from research in the library; like Scott, in his Border novels, he gives, rather, the impression of having drunk it in with his mother's milk."
Jim Stephens, editor of An August Derleth Reader, (1992), argues: "what Derleth accomplished....was to gather a Wisconsin mythos which gave respect to the ancient fundament of our contemporary life."
The author inaugurated the Sac Prairie Saga with four novellas comprising Place of Hawks, published by Loring & Mussey in 1935. At publication, The Detroit News wrote: "Certainly with this book Mr. Derleth may be added to the American writers of distinction."
Derleth's first novel, Still is the Summer Night, was published two years later by the famous Charles Scribners' editor Maxwell Perkins, and was the second in his Sac Prairie Saga.
Village Year, the first in a series of journals – meditations on nature, Midwestern village American life, and more – was published in 1941 to praise from The New York Times Book Review: "A book of instant sensitive responsiveness...recreates its scene with acuteness and beauty, and makes an unusual contribution to the Americana of the present day." The New York Herald Tribune observed that "Derleth...deepens the value of his village setting by presenting in full the enduring natural background; with the people projected against this, the writing comes to have the quality of an old Flemish picture, humanity lively and amusing and loveable in the foreground and nature magnificent beyond." James Grey, writing in the St. Louis Dispatch concluded, "Derleth has achieved a kind of prose equivalent of the Spoon River Anthology." | [
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980_4 | In the same year, Evening in Spring was published by Charles Scribners & Sons. This work Derleth considered among his finest. What The Milwaukee Journal called "this beautiful little love story", is an autobiographical novel of first love beset by small-town religious bigotry. The work received critical praise: The New Yorker considered it a story told "with tenderness and charm", while the Chicago Tribune concluded: "It's as though he turned back the pages of an old diary and told, with rekindled emotion, of the pangs of pain and the sharp, clear sweetness of a boy's first love." Helen Constance White, wrote in The Capital Times that it was "...the best articulated, the most fully disciplined of his stories."
These were followed in 1943 with Shadow of Night, a Scribners' novel of which The Chicago Sun wrote: "Structurally it has the perfection of a carved jewel...A psychological novel of the first order, and an adventure tale that is unique and inspiriting."
In November 1945, however, Derleth's work was attacked by his one-time admirer and mentor, Sinclair Lewis. Writing in Esquire, Lewis observed, "It is a proof of Mr. Derleth's merit that he makes one want to make the journey and see his particular Avalon: The Wisconsin River shining among its islands, and the castles of Baron Pierneau and Hercules Dousman. He is a champion and a justification of regionalism. Yet he is also a burly, bounding, bustling, self-confident, opinionated, and highly-sweatered young man with faults so grievous that a melancholy perusal of them may be of more value to apprentices than a study of his serious virtues. If he could ever be persuaded that he isn't half as good as he thinks he is, if he would learn the art of sitting still and using a blue pencil, he might become twice as good as he thinks he is – which would about rank him with Homer." Derleth good-humoredly reprinted the criticism along with a photograph of himself sans sweater, on the back cover of his 1948 country journal: Village Daybook. | [
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980_5 | A lighter side to the Sac Prairie Saga is a series of quasi-autobiographical short stories known as the "Gus Elker Stories", amusing tales of country life that Peter Ruber, Derleth's last editor, said were "...models of construction and...fused with some of the most memorable characters in American literature." Most were written between 1934 and the late 1940s, though the last, "Tail of the Dog", was published in 1959 and won the Scholastic Magazine short story award for the year. The series was collected and republished in Country Matters in 1996.
Walden West, published in 1961, is considered by many Derleth's finest work. This prose meditation is built out of the same fundamental material as the series of Sac Prairie journals, but is organized around three themes: "the persistence of memory...the sounds and odors of the country...and Thoreau's observation that the 'mass of men lead lives of quiet desperation.'" A blend of nature writing, philosophic musings, and careful observation of the people and place of "Sac Prairie." Of this work, George Vukelich, author of "North Country Notebook", writes: "Derleth's Walden West is...the equal of Sherwood Anderson's Winesburg,Ohio, Thornton Wilder's Our Town, and Edgar Lee Masters' Spoon River Anthology." This was followed eight years later by Return to Walden West, a work of similar quality, but with a more noticeable environmentalist edge to the writing, notes critic Norbert Blei.
A close literary relative of the Sac Prairie Saga was Derleth's Wisconsin Saga, which comprises several historical novels.
Detective and mystery fiction
Detective fiction represented another substantial body of Derleth's work. Most notable among this work was a series of 70 stories in affectionate pastiche of Sherlock Holmes, whose creator, Sir Arthur Conan Doyle, he admired greatly. These included one published novel as well (Mr. Fairlie's Final Journey). The series features a (Sherlock Holmes-styled) British detective named Solar Pons, of 7B Praed Street in London. The series was greatly admired by such notable writers and critics of mystery and detective fiction as Ellery Queen (Frederic Dannay), Anthony Boucher, Vincent Starrett and Howard Haycraft. | [
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980_6 | In his 1944 volume The Misadventures of Sherlock Holmes, Ellery Queen wrote of Derleth's The Norcross Riddle, an early Pons story: "How many budding authors, not even old enough to vote, could have captured the spirit and atmosphere with as much fidelity?" Queen adds, "...and his choice of the euphonic Solar Pons is an appealing addition to the fascinating lore of Sherlockian nomenclature." Vincent Starrett, in his foreword to the 1964 edition of The Casebook of Solar Pons, wrote that the series is "...as sparkling a galaxy of Sherlockian pastiches as we have had since the canonical entertainments came to an end."
Despite close similarities to Doyle's creation, Pons lived in the post-World War I era, in the decades of the 1920s and 1930s. Though Derleth never wrote a Pons novel to equal The Hound of the Baskervilles, editor Peter Ruber wrote: "...Derleth produced more than a few Solar Pons stories almost as good as Sir Arthur's, and many that had better plot construction."
Although these stories were a form of diversion for Derleth, Ruber, who edited The Original Text Solar Pons Omnibus Edition (2000), argued: "Because the stories were generally of such high quality, they ought to be assessed on their own merits as a unique contribution in the annals of mystery fiction, rather than suffering comparison as one of the endless imitators of Sherlock Holmes."
Some of the stories were self-published, through a new imprint called "Mycroft & Moran", an appellation of humorous significance to Holmesian scholars. For approximately a decade, an active supporting group was the Praed Street Irregulars, patterned after the Baker Street Irregulars.
In 1946, Conan Doyle's two sons made some attempts to force Derleth to cease publishing the Solar Pons series, but the efforts were unsuccessful and eventually withdrawn.
Derleth's mystery and detective fiction also included a series of works set in Sac Prairie and featuring Judge Peck as the central character. | [
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980_7 | Youth and children's fiction
Derleth wrote many and varied children's works, including biographies meant to introduce younger readers to explorer Jacques Marquette, as well as Ralph Waldo Emerson and Henry David Thoreau. Arguably most important among his works for younger readers, however, is the Steve and Sim Mystery Series, also known as the Mill Creek Irregulars series. The ten-volume series, published between 1958 and 1970, is set in Sac Prairie of the 1920s and can thus be considered in its own right a part of the Sac Prairie Saga, as well as an extension of Derleth's body of mystery fiction. Robert Hood, writing in the New York Times said: "Steve and Sim, the major characters, are twentieth-century cousins of Huck Finn and Tom Sawyer; Derleth's minor characters, little gems of comic drawing." The first novel in the series, The Moon Tenders, does, in fact, involve a rafting adventure down the Wisconsin River, which led regional writer Jesse Stuart to suggest the novel was one that "older people might read to recapture the spirit and dream of youth." The connection to the Sac Prairie Saga was noted by the Chicago Tribune: "Once again a small midwest community in 1920s is depicted with perception, skill, and dry humor."
Arkham House and the "Cthulhu Mythos"
Derleth was a correspondent and friend of H. P. Lovecraft – when Lovecraft wrote about "le Comte d'Erlette" in his fiction, it was in homage to Derleth. Derleth invented the term "Cthulhu Mythos" to describe the fictional universe depicted in the series of stories shared by Lovecraft and other writers in his circle. | [
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980_8 | When Lovecraft died in 1937, Derleth and Donald Wandrei assembled a collection of Lovecraft's stories and tried to get them published. Existing publishers showed little interest, so Derleth and Wandrei founded Arkham House in 1939 for that purpose. The name of the company derived from Lovecraft's fictional town of Arkham, Massachusetts, which features in many of his stories. In 1939, Arkham House published The Outsider and Others, a huge collection that contained most of Lovecraft's known short stories. Derleth and Wandrei soon expanded Arkham House and began a regular publishing schedule after its second book, Someone in the Dark, a collection of some of Derleth's own horror stories, was published in 1941.
Following Lovecraft's death, Derleth wrote a number of stories based on fragments and notes left by Lovecraft. These were published in Weird Tales and later in book form, under the byline "H. P. Lovecraft and August Derleth", with Derleth calling himself a "posthumous collaborator." This practice has raised objections in some quarters that Derleth simply used Lovecraft's name to market what was essentially his own fiction; S. T. Joshi refers to the "posthumous collaborations" as marking the beginning of "perhaps the most disreputable phase of Derleth's activities". | [
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