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Monomelic amyotrophy with late progression.

J Rowin1, M N Meriggioli, E J Cochran

  • 1Section of Neuromuscular Disease, Department of Neurological Sciences, Rush University, Chicago, IL, USA. jrowin@rush.edu

Neuromuscular Disorders : NMD
|April 12, 2001
PubMed
Summary
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Monomelic amyotrophy, a juvenile-onset muscle wasting disease, typically stabilizes. This case shows it can rarely progress to lower limbs years later.

Area of Science:

  • Neurology
  • Clinical Medicine
  • Rare Diseases

Background:

  • Monomelic amyotrophy (MA) is a rare, sporadic, juvenile-onset motor neuron disease.
  • Characterized by unilateral hand muscle weakness and atrophy.
  • Generally considered benign and non-progressive, stabilizing within five years.

Observation:

  • This report details a rare case of monomelic amyotrophy.
  • The patient experienced a prolonged period of disease stability.
  • Late clinical progression to the lower extremities was observed.

Findings:

  • Monomelic amyotrophy can exhibit late-onset progression beyond the typical five-year stabilization period.
  • Progression may extend to affect lower limb muscles.
  • This challenges the established understanding of MA as strictly non-progressive.

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Implications:

  • Highlights the need for long-term monitoring in monomelic amyotrophy patients.
  • Suggests a potential for delayed or atypical disease trajectories.
  • Informs differential diagnosis and management strategies for motor neuron diseases.