Amyotrophic lateral sclerosis

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

Amyotrophic lateral sclerosis
Stephen Hawking, a physicist who has ALS.
ICD-10 G12.2
ICD-9 335.20
OMIM 105400
DiseasesDB 29148
MedlinePlus 000688
MeSH D000690

Overview

Amyotrophic Lateral Sclerosis is a chronic progressive neurological disease that affects both upper and lower motor neurons. It presents with muscle spasms, fasciculation, weakness and which could ultimately lead to muscles paralysis. ALS is diagnosed clinically, however additional testing with electromyography to confirm muscle denervation. Laboratory tests might be required to rule out other reversible causes of weakness. ALS has a poor prognosis with death usually due to respiratory complications with a median survival of 3-5 years. ALS is managed by a multidisciplinary care setting with fewer disease modifying agents to date.

Historical Perspective

Amyotrophic Lateral Sclerosis was first described by French neurologist Jean-Martin Charcot in his Tuesday lectures in late 1860 and then a publication in 1874. He is also known as the Father of Amyotrophic Lateral Sclerosis because of his devotion to the disease. He complied 50 years of previous work on muscle weakness. He was the first scientist to link the disease to its specific pathology. [1]

Classification

Pathophysiology

Causes

Differentiating Amyotrophic lateral sclerosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Evaluation | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

References

  1. Katz JS, Dimachkie MM, Barohn RJ (2015). "Amyotrophic Lateral Sclerosis: A Historical Perspective". Neurol Clin. 33 (4): 727–34. doi:10.1016/j.ncl.2015.07.013. PMID 26515617.

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