Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

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.

Hirayama Disease: a Case Report with Late Onset

“…Areas of cervical spinal cord atrophy loss of cervical lordosis are observed in MRI. In Myelography is difficult to retain contrast in the subarachnoid space but is not used anymore [5].…”

“…Differential diagnosis should include Amyotrophic lateral sclerosis, syringomyelia and cervical compression among others. Treatment is conservative with cervical collar but some cases may require surgical treatment, with posterior lateral mass instrumentation [4] or cervical anterior discectomy and fusion [5].…”

Hirayama’s disease associated with cervical deformity and spinal cord compression: a case report from Sweden

“…HD occurs mostly sporadic, although familial occurrences are also reported but are quite rare [ 8 ]. Treatment with a cervical collar is the first line of choice, but surgical intervention may also be considered in patients with rapid neurological deterioration that according to the literature can progress toward spastic paraparesis [ 6 , 9 – 11 ].…”