Abstract
BACKGROUND Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America that was successfully treated via surgical intervention. OBSERVATIONS The patient was a 15-year boy with insidious onset upper limb weakness and atrophy. His findings were a classic presentation of HD although his complex history and relative rarity of the disease caused him to remain undiagnosed for months. After conservative management via cervical collar failed, the patient was successfully treated via C5-C7 anterior cervical discectomy and fusion. The patient’s symptoms stabilized by the 3-month follow-up. LESSONS The diagnosis of HD is easy to miss because of the lack of reporting and widespread knowledge of this condition in North America. Thus, when presented with a case of insidious onset limb weakness in a juvenile patient, HD should be placed on the differential list and verified with cervical flexion magnetic resonance imaging. Additionally, surgical intervention should be considered a safe and effective option for HD when conservative methods have failed.
Original language | English (US) |
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Article number | CASE21697 |
Journal | Journal of Neurosurgery: Case Lessons |
Volume | 3 |
Issue number | 10 |
DOIs | |
State | Published - Mar 2022 |
Keywords
- ACDF
- Hirayama disease
- anterior cervical discectomy and fusion
- spinal muscular atrophy
- surgical outcomes
ASJC Scopus subject areas
- Clinical Neurology
- Surgery