Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: Case report

Jun Young Kim, Su Keong Hwang, Soonhak Kwon, Jin Sung Park

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Abstract

Background: We report a rare case of an 18-year-old male with unilateral hand tremor who was finally diagnosed with Hirayama disease (HD). Case presentation: An 18-year-old male presented with unilateral polymyoclonus that aggravated with neck flexion. The patient did not complain of muscle weakness or muscle atrophy. The needle electromyography showed giant motor unit potentials in right cervical 7 and 8 innervated muscles. The cervical magnetic resonance imaging on supine and flexion state showed prominent forward effacement of posterior dural sac that was compatible with HD. Conclusions: HD usually presents with unilateral distal hand weakness, muscle atrophy and tremor. Although it is a benign and self-limiting disease, early diagnosis may lead to less clinical deterioration. Moreover, electromyography should be completed in the differentiation of young male patients who present with polymyoclonus without hand weakness or atrophy.

Original languageEnglish
Article number325
JournalBMC Neurology
Volume20
Issue number1
DOIs
StatePublished - 1 Sep 2020

Keywords

  • Aggravation
  • Case report
  • Flexion
  • Hirayama disease
  • Polymyoclonus

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