A 34-Year-Old Female Patient with Hirayama Disease Complicated by Severe Spinal Cord Injury.

Abstract

Hirayama disease (HD), characterized by insidiously progressive muscular atrophy of forearms and hands, is a self-limited cervical myelopathy that predominantly affects male adolescents, with female patients, especially middle-aged women, rarely affected. We present a rare case of HD with severe spinal cord injury in a 34-year-old woman. A 34-year-old woman presented with progressive weakness of both hands and forearms for a duration of 6 years and spastic gait for 1 year. The patient worked as a teacher and experienced long-term and repetitive neck flexion while writing at a desk, potentially causing cervical loading. Examination showed weakness and muscular atrophy of the bilateral upper limbs and pyramidal signs, including Hoffmann sign and hyperreflexia of the lower limbs. Radiologic studies revealed midcervical kyphosis and spinal cord compression by forward displacement of the dura mater with neck flexion. The clinical presentation and radiologic characteristics confirmed the diagnosis of HD. Anterior cervical corpectomy and fusion was performed owing to severe spinal cord injury, and the patient experienced satisfactory improvement. The present case clarified the potential involvement of cervical kyphosis and cervical loading-related exercise in the onset and progression of HD. Anterior cervical corpectomy and fusion could serve as a promising treatment of HD with severe spinal cord injury.