Amyotrophic lateral sclerosis historical perspective

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

For the first time ALS was identified as a specific disease by Jean Martin Charcot, a French neurologist working in Paris in 1869s, and thus is still sometimes called Charcot’s disease in some countries such as France. He described and diagnosed the first cases of ALS as a specific neurological disease associated with a distinct pathology. Studies conducted between 1865 to 1869 by Charcot and his colleague Joffroy found that lesions within the lateral column in the spinal cord resulted in chronic progressive paralysis and contractures (no atrophy of muscles), while lesions of the anterior horn of the spinal cord resulted in paralysis without contractures (with atrophy of muscles). These findings supported his hypothesis at the time that the motor component of the spinal cord consisted of a two-part system, and that the location of the lesion results in a varying clinical presentation. While the term amyotrophic lateral sclerosis was not offered by Charcot until 1874 when his lectures were compiled into a collection of his work titled “Oeuvres Completes,” ALS is still referred to as Charcot’s disease in many parts of the world. While numerous molecular and genetic discoveries have allowed for a greater understanding of this disease, his original descriptions of the associated clinical and pathological findings of ALS have remained virtually unaltered. Lou Gehrig was a famous baseball player for the New York Yankees. He was one of the most talented baseball players of all time; yet he is also remembered for ALS, the disease that took his life (June 2, 1941) and still bears his name as its eponym.

Historical Perspective

  • For the first time ALS was identified as a specific disease by Jean Martin Charcot, a French neurologist working in Paris in 1869s, and thus is still sometimes called Charcot’s disease in some countries such as France.
    • He described and diagnosed the first cases of ALS as a specific neurological disease associated with a distinct pathology.
    • Studies conducted between 1865 to 1869 by Charcot and his colleague Joffroy found that lesions within the lateral column in the spinal cord resulted in chronic progressive paralysis and contractures (no atrophy of muscles), while lesions of the anterior horn of the spinal cord resulted in paralysis without contractures (with atrophy of muscles).[1][2]
    • These findings supported his hypothesis at the time that the motor component of the spinal cord consisted of a two-part system, and that the location of the lesion results in a varying clinical presentation.[1][2]
    • While the term amyotrophic lateral sclerosis was not offered by Charcot until 1874 when his lectures were compiled into a collection of his work titled “Oeuvres Completes,” ALS is still referred to as Charcot’s disease in many parts of the world. [1][2]
    • While numerous molecular and genetic discoveries have allowed for a greater understanding of this disease, his original descriptions of the associated clinical and pathological findings of ALS have remained virtually unaltered.[2][1]

Famous Cases

Lou Gehrig was a famous baseball player for the New York Yankees.[1] He was one of the most talented baseball players of all time; yet he is also remembered for ALS, the disease that took his life (June 2, 1941) and still bears his name as its eponym.[1]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 Goetz CG (2000). "Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot". Muscle Nerve. 23 (3): 336–43. PMID 10679709.
  2. 2.0 2.1 2.2 2.3 Kumar DR, Aslinia F, Yale SH, Mazza JJ (2011). "Jean-Martin Charcot: the father of neurology". Clin Med Res. 9 (1): 46–9. doi:10.3121/cmr.2009.883. PMC 3064755. PMID 20739583.

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