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{{Amyotrophic lateral sclerosis}}
{{Amyotrophic lateral sclerosis}}
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{{CMG}}; {{AE}} {{MMJ}}


==Overview==
==Overview==
The word ''amyotrophic'' is present Greek in origin. ''A'' means no or negative, ''myo'' refers to [[muscle]], and ''trophic'' means nourishment. When put together it means "no-muscle-nourishment." ''[[Anatomical terms of location|Lateral]]'' identifies the areas of the [[spinal cord]] where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening  ([[sclerosis]]) in the region. <ref>[http://www.alsa.org/als/what.cfm What is ALS - The ALS Association] Retrieved October 24, 2006</ref>
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==
==Historical Perspective==
* 1850 - English scientist [[Augustus Waller]] describes the appearance of shriveled nerve fibers
* 1869 - French doctor [[Jean-Martin Charcot]] first describes ALS in scientific literature
* 1881 - "On Amyotrophic Lateral Sclerosis" gets translated into English and published in a three-volume edition of Lectures on the Diseases of the Nervous System
* 1939 - ALS becomes a ''cause célèbre'' in the United States when baseball legend Lou Gehrig's career—and, two years later, his life—is ended by the disease.
* 1950s - ALS epidemic occurs among the Chamorro people on Guam
* 1991 - Researchers link chromosome 21 to FALS (Familial ALS)
* 1993 - SOD1 gene on chromosome 21 found to play a role in some cases of FALS
* 1996 - Rilutek® becomes the first FDA-approved drug for ALS
* 1998 - El Escorial is developed as the standard for confirming ALS
* 2001 - Alsin gene on chromosome 2 found to cause ALS2


*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]]).<ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref><ref name="pmid20739583">{{cite journal| author=Kumar DR, Aslinia F, Yale SH, Mazza JJ| title=Jean-Martin Charcot: the father of neurology. | journal=Clin Med Res | year= 2011 | volume= 9 | issue= 1 | pages= 46-9 | pmid=20739583 | doi=10.3121/cmr.2009.883 | pmc=3064755 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20739583  }} </ref>
**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.<ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref><ref name="pmid20739583">{{cite journal| author=Kumar DR, Aslinia F, Yale SH, Mazza JJ| title=Jean-Martin Charcot: the father of neurology. | journal=Clin Med Res | year= 2011 | volume= 9 | issue= 1 | pages= 46-9 | pmid=20739583 | doi=10.3121/cmr.2009.883 | pmc=3064755 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20739583  }} </ref>
**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. <ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref><ref name="pmid20739583">{{cite journal| author=Kumar DR, Aslinia F, Yale SH, Mazza JJ| title=Jean-Martin Charcot: the father of neurology. | journal=Clin Med Res | year= 2011 | volume= 9 | issue= 1 | pages= 46-9 | pmid=20739583 | doi=10.3121/cmr.2009.883 | pmc=3064755 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20739583  }} </ref>
**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.<ref name="pmid20739583">{{cite journal| author=Kumar DR, Aslinia F, Yale SH, Mazza JJ| title=Jean-Martin Charcot: the father of neurology. | journal=Clin Med Res | year= 2011 | volume= 9 | issue= 1 | pages= 46-9 | pmid=20739583 | doi=10.3121/cmr.2009.883 | pmc=3064755 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20739583  }} </ref><ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref>
==Famous Cases==
Lou Gehrig was a famous baseball player for the New York Yankees.<ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref>
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.<ref name="pmid10679709">{{cite journal| author=Goetz CG| title=Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot. | journal=Muscle Nerve | year= 2000 | volume= 23 | issue= 3 | pages= 336-43 | pmid=10679709 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10679709  }} </ref>
==References==
==References==
{{reflist|2}}
{{Reflist|2}}


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Latest revision as of 17:08, 24 September 2018

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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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