Hirayama Disease: A Rare Case Report and Literature Review

Volume 3 | Issue 2 | October 2022-March 2023 | page: 109-112 | Sharvin K Sheth, Amit C Jhala, Jay V Shah


Authors: Sharvin K Sheth [1], Amit C Jhala [1], Jay V Shah [2]

[1] Department of Spine Surgery, Chirayu Orthopaedic and Spine Hospital, Ahmedabad, Gujarat, India.
[2] Department of Radiology, Medimax Advance Radio Imaging, Ahmedabad, Gujarat, India.

Address of Correspondence

Dr. Amit C Jhala,
Consultant Spine Surgeon and Chief of Department of Spine Surgery, Chirayu Orthopaedic and Spine Hospital, Ahmedabad, Gujarat, India.


Hirayama disease is a rare neurological condition and is characterized by a sporadic juvenile muscular atrophy of distal upper extremity in young males. The disease is more prevalent in Japan and other Asian countries, though a few cases have been reported in Western countries as well. It manifests as a self-limiting, gradually progressive atrophic weakness of forearm and hand. The anterior displacement of posterior dura during neck flexion leading to cervical cord atrophy has been hypothesized. We discuss a case of a 21-year-old male patient with progressive distal upper extremity weakness, diagnosed with Hirayama disease, and literature review for the same.
Keywords: Hirayama Disease, Juvenile muscular atrophy, Monomelic amyotrophy


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How to Cite this Article: Sheth SK, Jhala AC , Shah JV |  Hirayama Disease: A Rare Case Report and Literature Review | Back Bone: The Spine Journal | October 2022-March 2023; 3(2): 109-112.

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