Which syndromes associated with amyotrophic lateral sclerosis (ALS) cause focal muscular atrophy (FMA)?

Updated: Feb 20, 2018
  • Author: Sridharan Ramaratnam, MD, MBBS; Chief Editor: Helmi L Lutsep, MD  more...
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Answer

Amyotrophic lateral sclerosis and syndromes that mimic ALS

  • ALS: A small percentage of patients with adult-onset motor neuron disease have one of the restricted subtypes, which traditionally are included within the clinical spectrum of ALS versus progressive muscular atrophy (the pure LMN form) versus progressive bulbar palsy (the pure bulbar form).

  • In the El Escorial terminology, none of these subtypes would be considered "definite" or "probable" but instead, "suspected" or "possible" ALS.

  • Most patients who are diagnosed initially with a restricted subtype evolve clinically to classical ALS.


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