Hirayama Disease Treated by Anterior Cervical Diskectomy and Fusion: Case Report and Literature Review

World Neurosurg. 2020 Sep:141:171-174. doi: 10.1016/j.wneu.2020.06.037. Epub 2020 Jun 12.

Abstract

Objective: We present the case of a 19-year-old boy who had the classic radiologic and clinical presentations of Hirayama disease treated with anterior cervical diskectomy and fusion (ACDF). We also propose ACDF as promising surgery for the treatment of Hirayama disease. Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord.

Case description: Our patient presented with an insidious, progressive weakness in his right hand, which had been ongoing for 1 year. Physical examination revealed various degrees of right forearm and hand muscle wasting, and decreased right hand extend power with motor grade Ш. Cervical flexed magnetic resonance imaging showed a spinal cord was being compressed-most noticeably at the level of the fifth cervical vertebral body-and that the dorsal epidural space was abnormally expanding. The patient underwent ACDF at the C4-6 level. The pain and paresthesia improved immediately after the surgery. His motor grade improved immediately after the operation, and there were improvements of a modest reversal of muscle wasting at 1 year postoperatively.

Conclusions: ACDF could be considered as an effective treatment option for the treatment of Hirayama disease. Our patient's finger function improved. Therefore we believe that anterior fusion might be the best choice of treatment.

Keywords: ACDF; Hirayama disease.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Cervical Vertebrae / surgery
  • Decompression, Surgical / methods
  • Diskectomy / methods*
  • Humans
  • Male
  • Spinal Cord Compression / etiology
  • Spinal Cord Compression / surgery
  • Spinal Fusion / methods*
  • Spinal Muscular Atrophies of Childhood / complications
  • Spinal Muscular Atrophies of Childhood / surgery*

Supplementary concepts

  • Amyotrophy, monomelic