Hirayama disease - A case report and literature review

Background: Hirayama disease (HD) is a rare, benign neurological disorder predominantly affecting young males. It is characterized by an insidious onset of unilateral or asymmetrical distal upper-limb weakness and muscle wasting, primarily due to dynamic spinal cord compression during neck flexion. Case Description: We report on a 19-year-old right-hand-dominant schoolboy who presented with progressive right-hand weakness, numbness, and neck pain. Examination revealed right upper-limb muscle wasting, especially in the intrinsic hand muscles. Dynamic cervical magnetic resonance imaging demonstrated anterior displacement of the posterior dura and maximal cord compression at the C6–C7 level during neck flexion. The patient underwent cervical laminectomy to decompress the spinal cord. Postoperatively, he was referred for physiotherapy but defaulted on further treatment. Management of HD includes conservative cervical collar immobilization to prevent neck flexion and progression of neurological deficits. Surgical intervention is reserved for progressive or severe cases with significant cord compression. Our decision for a laminectomy was based on the patient’s radiological findings and clinical progression. Awareness and early diagnosis remain critical to preserving neurological function. Conclusion: This case underlines the importance of dynamic imaging in diagnosing HD and highlights surgical decompression as an effective treatment in progressive cases.