A case of Hirayama disease treated with laminectomy and duraplasty without spinal fusion.

INTRODUCTION

Juvenile amyotrophy of the distal upper extremity (JADUE) is a rare disease afecting young males. Since neck flexion has been thought to be the cause of the spinal cord damage, cervical orthosis, spinal fusion by an anterior or posterior approach and duraplasty in combination with posterior spinal fusion have been proposed as treatment of JADUE. We are presenting the case of a patient with JADUE who was surgically treated with duraplasty without spinal fusion, thus avoiding the compression of the spinal cord without limitation of cervical movement.

CASE

A previously healthy 19-year-old man presented with insidious onset of weakness in the left forearm and hand for the past year. On MRI, during neck flexion, the posterior dura showed anterior displacement that compressed the cervical spinal cord. The dura was opened linearly from C3 to C6, observing the herniation of the spinal cord through the opening. Duraplasty was performed in order to increase the room of the spinal cord. No spinal fusion was performed.

DISCUSSION

The postoperative course was uneventful. Clinical deterioration stopped following operation and two years later unchanged as compared to the preoperative one.