Bilateral Hirayama Disease: A Case Report

Hirayama disease is a rare restricted form of motor neuron disease. It commonly affects young males. Patients typically presentwith the insidious onset of unilateral weakness and atrophy of the hand muscles that often progresses to the forearm. In somecases, the syndrome is bilateral but often asymmetrical. Of note, the brachioradialis muscle is usually spared. The syndromeaffects C7–C8–T1 muscles with sparing of the C5–6 muscles. We report a case of a 25-year-old male who presented with 2years of history of progressive wasting and weakness of muscles of bilateral hands and forearms. Based on clinical features,electrodiagnostic studies and dynamic magnetic resonance imaging cervical spine diagnosis of Hirayama disease were made.The patient was treated conservatively with a cervical collar. Over a period of 8 months follow-up, no progression was seen.

Severe Spinal Injury in Hirayama Disease

Hirayama disease is a rare neurological disorder characterized by an insidious progressive subacute unilateral or bilateral weakness of the hands and forearm muscles leading to a painless amyotrophy. The disease primarily affects young men in the second to third decades of life. It has always been described as a second motor neuron disease, thus sparing the pyramidal and sensitive pathways. It usually has a slow progression course of 3 to 5 years followed by stabilization. Since its initial description by Keyzo Hirayama in 1959, most cases have been reported in Asia, particularly Japan and India, although the disease reportedly has worldwide distribution.