Hirayama Disease - A Not So Foreign Entity: Case Report and Literature Review

Yuh SJ1*, Guillon LL2 , Lalonde K1 , Boubez G3 , Wang Z3 and Shedid D1

Department of Neurosurgery, Centre Hospitalier de l?Universit? de Montr?al (CHUM), Canada 2 Department of Radiology, Centre Hospitalier de l?Universit? de Montr?al (CHUM), Canada 3 Department of Orthopedics, Centre Hospitalier de l?Universit? de Montr?al (CHUM), Canada

^*Correspondance to: Sung-Joo Yuh 

 PDF  Full Text DOI: 10.25107/2474-1647.3137

Abstract

Introduction: Hirayama disease, also known as juvenile muscular atrophy of the distal upper limb, is a rare disease that is characterized by an insidious-asymmetric onset of muscular atrophy and weakness in the distal upper extremities. Case Report: Here we present a case in a young male of Canadian decent, who presented with severe atrophy of both forearms, sparring however the brachioradialis. Motor strength revealed weakness in all muscle groups in the upper extremities, more prominent in the distal muscle groups bilaterally. There was marked difficulty with prehension. He also found his walking to be more difficult. Cervical flexion MRI demonstrate forward migration of the dura mater wall with an enlarged posterior epidural space, with disappearance of these large flow voids with the neck in neutral position. Conclusion: Thought typically a benign self-limiting type of cervical myelopathy, early diagnosis of Hirayama disease is essential. It is important to appreciate the typical clinical and imaging features to accurately differentiate this rare disease from other neurological misdiagnoses. Progressive neurological and presence of myelomalacia should warrant aggressive surgical treatment as in our case

Keywords

Hirayama disease; Cervical myelopathy; Surgery

Cite the article

Yuh SJ, Guillon LL, Lalonde K, Boubez G, Wang Z, Shedid D. Hirayama Disease - A Not So Foreign Entity: Case Report and Literature Review. Clin Surg. 2021; 6: 3137..