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Case Reports in Medicine
Volume 2013, Article ID 606894, 4 pages
http://dx.doi.org/10.1155/2013/606894
Case Report

Bimelic Hirayama Disease: Clinical Dilemma Solved by Imaging

1Department of Radiodiagnosis, VMMC and Safdarjung Hospital, New Delhi 110029, India
2Department of General Surgery, VMMC and Safdarjung Hospital, New Delhi 110029, India
3Department of Otorhinolaryngology, VMMC and Safdarjung Hospital, New Delhi 110029, India

Received 5 December 2012; Accepted 10 March 2013

Academic Editor: Jahn M. Nesland

Copyright © 2013 Shalabh Jain et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hirayama disease (juvenile muscular atrophy of distal upper extremity) is a cervical myelopathy predominantly affecting adolescent males. It is characterized by progressive muscular weakness and atrophy of unilateral or asymmetrically bilateral distal upper limbs. We report a case of an 18-year-male painter, who presented with gradually progressive, symmetrical bilateral weakness of hands and forearm for the last two years. On the basis of clinical examination, a provisional diagnosis of lower motor neuron type of symmetrical distal weakness due to heavy metal intoxication was kept. However, imaging studies helped in making a definitive diagnosis of Hirayama disease. The patient was advised cervical collar, and there was no progression in symptoms after six months of followup. Due to the rarity of bilateral symmetrical involvement in Hirayama disease, it remains obscured or unsuspected clinically, and MRI plays a pivotal role in diagnosis.