Department of Orthopedic Surgery, School of Medicine, Catholic University of Daegu, Deagu, South Korea.
Department of Pediatric Neurology, School of Medicine, Kyungpook National University Children's Hospital, Daegu, South Korea.
BMC Neurol. 2020 Sep 1;20(1):325. doi: 10.1186/s12883-020-01904-z.
We report a rare case of an 18-year-old male with unilateral hand tremor who was finally diagnosed with Hirayama disease (HD).
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.
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.
我们报告了一例 18 岁男性单侧手震颤病例，最终诊断为平山病（HD）。
一名 18 岁男性出现单侧肌阵挛，屈颈时加重。患者无肌肉无力或肌肉萎缩主诉。针极肌电图显示右侧颈 7、8 神经根支配的肌肉出现巨大运动单位电位。仰卧位和屈颈位颈椎磁共振成像显示硬脊膜后缘明显向前移位，符合 HD 表现。
HD 通常表现为单侧手部远端无力、肌肉萎缩和震颤。虽然它是一种良性和自限性疾病，但早期诊断可能会减少临床恶化。此外，对于出现无手部无力或萎缩的肌阵挛的年轻男性患者，应进行肌电图检查以明确诊断。
