Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review

• Published in: Case reports in neurology Vol. 12; no. 3; pp. 291 - 298
• Main Authors: Al-Hashel, Jasem Y., Abdelnabi, Ehab A., Ibrahim Ismail, Ismail
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 17-09-2020; Karger Publishers
• Subjects: Atrophy; Case reports; Disease; Epidural; Family medical history; hirayama’s disease; juvenile non-progressive amyotrophy; Literature reviews; Magnetic resonance imaging; Males; monomelic amyotrophy; Patients; Single Case – General Neurology; Single Case − General Neurology; Spinal cord; Juvenile non-progressive amyotrophy; Hirayama’s disease; Monomelic amyotrophy
• ISSN: 1662-680X; 1662-680X
• DOI: 10.1159/000508994

Hirayama disease is a rare neurological entity that is characterized by initial progressive muscular wasting and weakness of the distal upper limb in young men, followed by a spontaneous arrest within several years. The disease is believed to be a result of forward displacement of the cervical dural sac and spinal cord induced by neck flexion. It is commonly seen in Asia and rarely encountered in the Middle East countries. We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.