Abstract
Hirayama disease, a juvenile muscular atrophy of the distal upper extremity, is a rare form of cervical flexion myelopathy characterized by insidiously progressive weakness of the hands and forearm muscles (i.e., painless amyotrophy). The pathognomonic finding is a markedly forward-shifted spinal cord during neck flexion, demonstrated by dynamic magnetic resonance imaging (MRI), as in a young man with muscle atrophy in the bilateral distal upper extremities. In this report, the authors describe a 31-year-old man who had the classic radiological and clinical presentations of Hirayama disease. Since prior medical treatment had been ineffective for years, he underwent multilevel instrumented anterior cervical discectomy and fusion (ACDF) to keep his subaxial cervical spine slightly-lordotic (nonflexion). His motor evoked potential amplitude improved immediately during the operation, and there were improvements of myelopathy and a modest reversal of muscle wasting at 1 year postoperatively. Postoperative dynamic cervical spine MRI also demonstrated minimal cord compression and elimination of the venous plexus engorgement dorsal to the thecal sac. Although Hirayama disease is benign in nature and frequently self-limiting, multilevel instrumented ACDF could be a reasonable management option.