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| title | chunk | source | category | tags | date_saved | instance |
|---|---|---|---|---|---|---|
| ALS | 11/11 | https://en.wikipedia.org/wiki/ALS | reference | science, encyclopedia | 2026-05-05T11:04:10.701513+00:00 | kb-cron |
Descriptions of the disease date back to at least 1824 by Charles Bell. In 1850, François-Amilcar Aran was the first to describe a disorder he named "progressive muscular atrophy", a form of ALS in which only the lower motor neurons are affected. In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot, who initially introduced the term amyotrophic lateral sclerosis in his 1874 paper. Flail arm syndrome, a regional variant of ALS, was first described by Alfred Vulpian in 1886. Flail leg syndrome, another regional variant of ALS, was first described by Pierre Marie and his student Patrikios in 1918.
=== Diagnostic criteria === In the 1950s, electrodiagnostic testing (EMG) and nerve conduction velocity (NCV) testing began to be used to evaluate clinically suspected ALS. In 1969, Edward H. Lambert published the first EMG/NCS diagnostic criteria for ALS, consisting of four findings he considered to strongly support the diagnosis. Since then, several diagnostic criteria have been developed, which are mostly in use for research purposes for inclusion/exclusion criteria, and to stratify patients for analysis in trials. Research diagnostic criteria for ALS include the "El Escorial" in 1994, revised in 1998. In 2006, the "Awaji" criteria proposed using EMG and NCV tests to help diagnose ALS earlier, and most recently, the "Gold Coast" criteria in 2019 simplified diagnosis, included cognitive change, and no longer required multiple regions to be affected.
=== Name ===
Amyotrophic is derived from Greek: a- means "no", myo- (from mûs) refers to "muscle", and trophḗ means "nourishment". Therefore, amyotrophy means "muscle malnourishment" or the wasting of muscle tissue. Lateral identifies the locations in the spinal cord of the affected motor neurons. Sclerosis means "scarring" or "hardening" and refers to the death of the motor neurons in the spinal cord. ALS is sometimes referred to as Charcot's disease (not to be confused with Charcot–Marie–Tooth disease or Charcot joint disease), because Jean-Martin Charcot was the first to connect the clinical symptoms with the pathology seen at autopsy. The British neurologist Russell Brain coined the term motor neuron disease in 1933 to reflect his belief that ALS, progressive bulbar palsy, and progressive muscular atrophy were all different forms of the same disease. In some countries, especially the United States, ALS is called Lou Gehrig's disease after the American baseball player Lou Gehrig, who was diagnosed with ALS in 1939. In the United States and continental Europe, the term ALS (as well as Lou Gehrig's disease in the U.S.) refers to all forms of the disease, including "classical" ALS, progressive bulbar palsy, progressive muscular atrophy, and primary lateral sclerosis. In the United Kingdom and Australia, the term motor neuron disease refers to all forms of the disease, while ALS only refers to "classical" ALS, meaning the form with both upper and lower motor neuron involvement.
== Society and culture ==
In addition to the baseball player Lou Gehrig, several notable individuals have or have had ALS. Theoretical physicist Stephen Hawking, who notably lived longer than any other known person with the condition, was the subject of the critically acclaimed biopic The Theory of Everything. American sociology professor and ALS patient Morrie Schwartz was the subject of the memoir Tuesdays with Morrie and the film of the same name. In August 2014, the "Ice Bucket Challenge" to raise money for ALS research went viral online. Participants filmed themselves filling a bucket full of ice water and pouring it onto themselves; they then nominated other individuals to do the same. Many participants donated to ALS research at the ALS Association, the ALS Therapy Development Institute, ALS Society of Canada, or Motor Neurone Disease Association in the UK.
== References ==
== External links ==
ALS Association Official Website ALS Therapy Development Institute International Alliance of ALS/MND Associations International Symposium on ALS/MND