Hirayama diseaseis a relatively rare myelopathy, a self limiting juvenile spinal muscular atrophy of the distal upper limbs. The exact etiology is unknown however a limited form of ischemic cervical myelopathy has been proposed as the cause. It affects mostly males in second to third decade. Cervical MRI is the preferred investigation. We report a case of Hirayama disease in a 24 year Indian male presenting with gradually progressive weakness and wasting of both the hands.

1. Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Internal medicine. 2000;39(4):283-90.
2. Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E, Akiguchi I, Nakashima K, Kira JI, Hirayama K. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotrophic Lateral Sclerosis. 2006 Jan 1;7(1):38-45.
3. Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases. Rinshoshinkeigaku= Clinical neurology. 2001;41(4-5):173-8.
4. Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy. Neuropathology. 2000 Sep;20:91-4.
5. Tokumaru Y, Hirayama K. A cervical collar therapy for non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease). Rinshoshin-keigaku= Clinical neurology. 1992 Oct;32(10):1102-6.
6. Robert B Daroff, Joseoh Jankovic , John C Mazziotta , Scott L Pomeroy . Neurology in clinical practice.7^th ed. Elsevier; 2016, 1498 p.

Dr Meghana Murthy

Junior Resident, Department of Medicine

Adichunchanagiri Institute of Medical Sciences