Progressive Muscular Atrophy

Neurol Clin. 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005.

Abstract

Progressive muscular atrophy (PMA) is a rare, sporadic, adult-onset motor neuron disease, clinically characterized by isolated lower motor neuron features; however, clinically evident upper motor neuron signs may emerge in some patients. Subclinical upper motor neuron involvement is identified pathologically, radiologically, and neurophysiologically in a substantial number of patients with PMA. Patients with subclinical upper motor neuron involvement do not fulfill the revised El Escorial criteria to participate in amyotrophic lateral sclerosis clinical trials. Intravenous immunoglobulin therapy is only marginally beneficial in a small subgroup of patients with lower motor neuron syndrome without conduction block.

Keywords: Lower motor neuron syndrome; Lower motor neuron-onset ALS; PMA; Progressive muscular atrophy.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Disease Management
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Motor Neuron Disease / classification
  • Motor Neuron Disease / diagnosis
  • Motor Neuron Disease / therapy
  • Muscular Atrophy, Spinal / diagnosis*
  • Muscular Atrophy, Spinal / epidemiology
  • Muscular Atrophy, Spinal / physiopathology
  • Muscular Atrophy, Spinal / therapy*
  • Neuroimaging
  • Severity of Illness Index

Substances

  • Immunoglobulins, Intravenous