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Monomelic atrophy.

J Oryema1, P Ashby, S Spiegel

  • 1EMG Department, Toronto Western Hospital, Ontario, Canada.

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
|May 1, 1990
PubMed
Summary
This summary is machine-generated.

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Monomelic amyotrophy, a rare muscle-wasting condition, was observed in five Canadian patients. This study details the clinical presentation and diagnostic findings of this syndrome in a North American population.

Area of Science:

  • Neurology
  • Muscle Diseases
  • Clinical Case Studies

Background:

  • Monomelic amyotrophy (MAM) is a rare neuromuscular disorder characterized by progressive, unilateral muscle wasting.
  • Previous reports of MAM have primarily originated from Japan and India, suggesting potential geographic predispositions.

Observation:

  • This study presents five cases of a syndrome resembling MAM in Canada.
  • The observed muscle wasting predominantly affected forearm muscles in one upper limb, notably sparing the brachioradialis and extensor carpi radialis.
  • Minimal muscle wasting and electromyographic abnormalities were noted in the contralateral upper limb.

Findings:

  • Computed tomography (CT) myelography revealed unilateral wasting of the cervical spinal cord in affected individuals.

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  • The clinical and radiological findings support the diagnosis of MAM in these Canadian patients.
  • The syndrome presented with a characteristic pattern of distal upper limb muscle atrophy.

    • Implications:

    • This report expands the known geographic distribution of monomelic amyotrophy.
    • It highlights the importance of considering MAM in the differential diagnosis of unilateral upper limb weakness, even outside of previously reported regions.
    • Further research is warranted to understand the etiology and potential genetic factors contributing to MAM.