Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report
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CASE REPORT
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Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease: case report Jun-Young Kim1†, Su-Keong Hwang2†, Soonhak Kwon2 and Jin-Sung Park3*
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. Keywords: Polymyoclonus, Hirayama disease, Flexion, Aggravation, Case report
Background Benign focal amyotrophy, or Hirayama disease (HD), is a rare self-limiting cervical myelopathy that predominantly affects adolescent males. It is clinically characterized by asymmetric weakness of the upper extremity muscles that are innervated by C7-T1 roots. Recent literature describes the high prevalence of cold paresis, fasciculation, paresthesia, numbness, and tremor in association with the disease [1, 2]. Only recent studies have recognized the presence of tremor in HD; it has been clinically described in terms of jerky, irregular, tremulous movements; myoclonic tremor; and polymyoclonus [3–5]. We * Correspondence: [email protected]; [email protected] † Jun-Young Kim and Su-Keong Hwang contributed equally to this work. 3 Department of Neurology, School of Medicine, Kyungpook National University, Kyungpook National University Chilgok Hospital, 807 Hoguk-ro, Daegu, South Korea Full list of author information is available at the end of the article
report a unique HD patient, who presented with polymyoclonus as the initial symptom.
Case presentation An 18-year-old boy presented with a 1-year history of hand tremor of the right hand, which worsened with neck flexion. He had no previous history of autoimmune diseases, including thyroid disease or family history of genetic disease. The initial neurological examination revealed no significant muscle weakness or atrophy, and except for a mildly decreased right triceps tendon reflex, deep tendon reflexes were normal. He complained of hand tremor of the right hand that worsened during neck flexion (Video 1). The hand tremor was irregular and jerky myoclonic tremors that were confined to his right hand, which was compatible with polymyoclonus. The routine laboratory findings, including complete blood count, electrolytes, liver function
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