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AAEM case report #28: monomelic amyotrophy

P D Donofrio1

  • 1Department of Neurology, Bowman Gray School of Medicine, Winston-Salem, North Carolina.

Muscle & Nerve
|October 1, 1994
PubMed
Summary
This summary is machine-generated.

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Monomelic amyotrophy is a rare motor neuron disease causing painless arm weakness in young men, primarily of Japanese/Indian descent. Symptoms progress over years before stabilizing, with specific electrophysiologic findings aiding diagnosis.

Area of Science:

  • Neurology
  • Rare Diseases
  • Motor Neuron Diseases

Background:

  • Monomelic amyotrophy (MMA) is a rare, sporadic motor neuron disease.
  • Typically affects young adult males, predominantly of Japanese and Indian heritage.

Observation:

  • Presents as painless, asymmetric weakness and atrophy in distal upper extremities.
  • Leg involvement and pyramidal signs are uncommon.
  • Often confused with other conditions affecting the cervical cord, brachial plexus, or nerves.

Findings:

  • Electrophysiologic studies show reduced muscle action potential amplitudes, fasciculations, and denervation.
  • Necropsy revealed anterior horn cell changes (shrinkage, necrosis, gliosis).
  • Disease progression usually spans several years before spontaneous arrest.

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Implications:

  • Highlights the need for accurate differential diagnosis in upper extremity weakness.
  • Understanding MMA's unique presentation aids early identification.
  • Further research into pathogenesis and treatment is warranted.