Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis)

Amyotrophic lateral sclerosis (ALS), a rapidly progressive, invariably fatal disease, involves mixed upper and lower motor neurons in different spinal cord regions. Patients with bulbar onset progress more rapidly than patients with limb onset or with a lower motor neuron presentation. Recent descri...

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Bibliographic Details
Published inNeurologic clinics Vol. 33; no. 4; p. 775
Main Authors Jawdat, Omar, Statland, Jeffrey M, Barohn, Richard J, Katz, Jonathan S, Dimachkie, Mazen M
Format Journal Article
LanguageEnglish
Published United States 01.11.2015
Subjects
Aged
Amyotrophic Lateral Sclerosis - classification
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - physiopathology
Disease Progression
Humans
Male
Neuroimaging
Prognosis
Brachial amyotrophic diplegia
Leg amyotrophic diplegia
Motor neuron disease
Amyotrophic lateral sclerosis
Isolated bulbar ALS
Flail arm
Flail leg
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Summary:Amyotrophic lateral sclerosis (ALS), a rapidly progressive, invariably fatal disease, involves mixed upper and lower motor neurons in different spinal cord regions. Patients with bulbar onset progress more rapidly than patients with limb onset or with a lower motor neuron presentation. Recent descriptions of regional variants suggest some patients have ALS isolated to a single spinal region for many years, including brachial amyotrophic diplegia, leg amyotrophic diplegia, and isolated bulbar palsy. Clearer definitions of regional variants will have implications for prognosis, understanding the pathophysiology of ALS, identifying genetic factors related to slower disease progression, and future planning of clinical trials.
ISSN:1557-9875
DOI:10.1016/j.ncl.2015.07.003