A severe case of Hirayama disease successfully treated by anterior cervical fusion

Hirayama disease, or juvenile amyotrophy of distal upper extremity, is a benign, self-limiting cervical myelopathy consisting of selective unilateral weakness of the hand and forearm. The weakness slowly progresses until spontaneous arrest occurs within 5 years of onset. The condition predominantly affects Asian males and is thought to be secondary to spinal cord compression during neck flexion, because of a forward displacement of the posterior dural sac. The authors present what is to their knowledge the first reported case of a Caucasian male with a severe form of Hirayama disease, suffering from weakness of the leg as well as the forearm. An abnormal range of cervical flexion was observed at the C5-6 level. The patient was successfully treated by anterior cervical discectomy and fusion.