1480. Anterior Cervical Discectomy and Fusion for Hirayama Disease: A Case Report and Literature Review

Authors: Yi-Hsuan Kuo, MD; Chao-Hung Kuo; Jau-Ching Wu; Wen-Cheng Huang (Taipei, Taiwan)


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. The pathognomonic finding is a markedly forward-shifted spinal cord during neck flexion, demonstrated by dynamic magnetic resonance images (MRIs).


In this report, we describe a 31-year-old man who had classic radiological and clinical presentations of Hirayama disease. He underwent multi-level instrumented anterior cervical discectomy and fusion (ACDF) to keep his sub-axial cervical spine slightly-lordotic (non-flexion).


His motor evoked potential (MEP) amplitude improved immediately intra-operation, and there were improvements of myelopathy at 1-year post-operation. The postoperative dynamic cervical spine MRIs 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, multi-level instrumented ACDF could be a reasonable option of management.