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Case Reports in Neurological Medicine
Volume 2016 (2016), Article ID 5839761, 4 pages
http://dx.doi.org/10.1155/2016/5839761
Case Report

Hirayama Disease: A Rare Disease with Unusual Features

Department of Medicine, Maulana Azad Medical College and Associated Hospitals, New Delhi 110002, India

Received 28 August 2016; Accepted 4 December 2016

Academic Editor: Dominic B. Fee

Copyright © 2016 S. Anuradha and Vanlalmalsawmdawngliana Fanai. 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, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord atrophy with asymmetric flattening. We report a case of Hirayama disease in a 25-year-old Indian man presenting with gradually progressive asymmetrical weakness and wasting of both hands and forearms along with unusual features of autonomic dysfunction and upper motor neuron lesion.