Hirayama Disease with Rare Presentation in a Young Adult
Abstract
Hirayama disease or Monomelic Amyotrophy (MMA) is a type of rare cervical myelopathy which is an asymmetrical, self-limited with progressive atrophy and weakness of the muscles of hands and forearms. It is mainly found in males of young age. There is occurrence of forward dislocation in the posterior dura of the lower cervical Dural canal at the time of flexion of the neck which causes lower cervical cord atrophy along with asymmetric flattening. Here, we report a rare case of this disease in a 21-year-old male who presented with slowly progressive asymmetrical weakness and atrophy of muscles of both hands and forearms associated with unusual characteristics of autonomic disturbances and lesion of upper motor neuron.
References
Yoo SD, Kim HS, Yun DH et al. Monomelic amyotrophy (Hirayama disease) with upper motor neuron signs: a
case report. Annals of Rehabilitation Medicine 2015; 39(1): 122-127.
Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical
entity. The Japanese Journal of Psychiatry and Neurology 1959; 61: 2190-2197.
Sitt JCM, Fung ELW, Yuen EHY et al. Hirayama disease in a 17-year-old Chinese man. Singapore Medical Journal
; 55(6): e87-9.
Hassan KM, Sahni H. Nosology of juvenile muscular atrophy of distal upper extremity: from monomelic
amyotrophy to Hirayama disease - Indian perspective. BioMed Research International 2013, Article ID 478516,
pages.
Tashiro K, Kikuchi S, Itoyama Y et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity
(Hirayama disease) in Japan. Amyotrophic Lateral Sclerosis 2006; 7(1): 38-45.
Zahid M, Wasim M, Ghaffar YA et al. A case of Hirayama disease in Pakistan. Journal of the Pakistan Medical
Association 2007; 57(1): 41-44.
Hassan KM, Sahni H, Jha A. Clinical and radiological profile of Hirayama disease: a flexion myelopathy
due to tight cervical Dural canal amenable to collar therapy. Annals of Indian Academy of Neurology 2012;
(2): 106-112.
Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Archives of Neurology 1984; 41(4): 388-
Sonwalkar H, Shah R, Khan F et al. Imaging features in Hirayama disease. Neurology India 2008; 56(1): 22-26.
Hirayama K, Tomonaga M, Kitano K et al. Focal cervical poliopathy causing juvenile muscular atrophy of distal
upper extremity: a pathological study. Journal of Neurology, Neurosurgery & Psychiatry 1987; 50(3):
-290.
Kira J, Ochi H. Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy.
Journal of Neurology Neurosurgery and Psychiatry 2001; 70(6): 798-801.
Kikuchi S, Tashiro K, Kitagawa M et al. A mechanism of juvenile muscular atrophy localized in the hand and
forearm (Hirayama’s disease) - flexion myelopathy with tight Dural canal in flexion. Clinical Neurology 1987;
(4): 412-419.
Kato Y, Kataoka H, Ichihara K et al. Biomechanical study of cervical flexion myelopathy using a three-dimensional finite element method. Journal of Neurosurgery: Spine 2008; 8(5): 436-441.
