Markedly improved function in severe Hirayama disease by anterior cervical fusion: A case report

Abstract

Background: Hirayama disease (HD) is a juvenile muscular atrophy of the distal upper extremities and is considered to be associated with cervical flexion. HD is managed by conservative treatment using cervical collar therapy or surgery, which may shorten the period of the disease or improve the functional outcome. However, surgical treatment for advanced and severe HD has rarely been reported, and it is unknown whether the surgical treatment effectively improves it. We present a rare case of rapidly progressing and advanced HD that showed remarkable recovery after early surgery. Case Description: An 18-year-old man presented with a 4-month history of HD, which rapidly progressed. The patient had severe weakness accompanied by atrophy of the right hand and forearm and mild weakness in the left-side counterparts. Five months after the onset, the patient underwent anterior cervical fixation at C5–7 and showed excellent recovery from severe HD; at postoperative month 8, he had no difficulty in daily life functioning, although a slight muscle weakness remained in the right forearm and hand. Conclusions: Even in cases of advanced HD, early surgical treatment may be effective in improving the function of upper extremities and could be considered for the treatment of patients.

Keywords

• Anterior cervical fixation
• Cervical flexion myelopathy
• Hirayama disease