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DOI: https://doi.org/10.14245/ns.1836178.089    [Accepted]
Published online January 4, 2019.
Anterior Cervical Discectomy and Fusion for Hirayama Disease: A Case Report and Literature Review
Yi-Hsuan Kuo1,2, Chao-Hung Kuo1,2,3  , Wen-Cheng Huang1,2, Jau-Ching Wu1,2 
1Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
2School of Medicine, National Yang-Ming University, Taipei, Taiwan
3Department of Biomedical Engineering, School of Biomedical Science and Engineering, National Yang-Ming University, Taipei, Taiwan
Corresponding Author:  Jau-Ching Wu
Tel: +886-2-28757718   Fax: +886-2-28757702   Email: jauching@gmail.com
Received: August 5, 2018   Revised: December 24, 2018   Accepted: December 26, 2018
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 images (MRIs), as can be seen in a young male with muscle atrophy in the bilateral distal upper extremities. In this report, the authors describe a 31-year-old man who had classic radiological and clinical presentations of Hirayama disease. Since prior medical treatment had been ineffective for years, 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 and modest reversal of the muscle wasting 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.
Keywords: Hirayama disease; juvenile muscular atrophy of distal upper extremi; cervical flexion myelopathy


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