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

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.

SUBMITTER: Kim JY

PROVIDER: S-EPMC7460763 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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

Kim Jun-Young JY Hwang Su-Keong SK Kwon Soonhak S Park Jin-Sung JS

BMC neurology 20200901 1

<h4>Background</h4>We report a rare case of an 18-year-old male with unilateral hand tremor who was finally diagnosed with Hirayama disease (HD).<h4>Case presentation</h4>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 flex ...[more]

PMID: 32873232

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

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

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