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Monomelic neurogenic syndromes: a prospective study.

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Monomelic neurogenic amyotrophy in Europeans shows varied progression. Transcranial magnetic stimulation (TMS) can predict if weakness will worsen, aiding prognosis for this rare neurological condition.

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Area of Science:

  • Neurology
  • Neurophysiology

Background:

  • Monomelic neurogenic syndromes are rare, with limited data on classification and prognosis in European populations.
  • Previous studies have not fully elucidated the prognostic indicators for monomelic neurogenic amyotrophy.

Purpose of the Study:

  • To investigate the clinical and neurophysiological features of monomelic neurogenic amyotrophy in a European cohort.
  • To identify prognostic factors for disease progression in adult-onset monomelic neurogenic amyotrophy.

Main Methods:

  • Prospective study of 17 patients with monomelic neurogenic amyotrophy (upper or lower limb onset).
  • Clinical and neurophysiological assessments, including Transcranial Magnetic Stimulation (TMS), were conducted over 3+ years.
  • Comparison with existing data on juvenile-onset Asian cases.

Main Results:

  • Proximal weakness or specific lower leg muscle involvement predicted no further progression.
  • Electromyography (EMG) findings in the contralateral limb did not predict progression.
  • Transcranial magnetic stimulation (TMS) indicating corticospinal dysfunction was a significant predictor of subsequent progression (p=0.01).
  • One case showed late upper limb involvement with conduction block, responding to IVIg therapy.

Conclusions:

  • Adult-onset European monomelic neurogenic amyotrophy presents differently from juvenile Asian forms.
  • Neurophysiological investigations, especially TMS, are crucial for determining prognosis.
  • Multifocal motor neuropathy should be considered in progressive cases, even years after onset.