Hirayama disease: a rare neurological condition of cervical cord pathology
DOI:
https://doi.org/10.18203/issn.2455-4510.IntJResOrthop20172891Keywords:
Hirayama, Muscular atrophy, Cervical cordAbstract
The disease is characterized by a progressive muscle weakness and wasting of distal upper limb muscles sparing brachioradialis. The pathology is in the lower cervical cord due to forward displacement of dural sac and spinal cord by neck flexion. Repeated neck flexion may result in ischemia of the lower cervical anterior horns. The diagnosis can be confirmed by MRI, EMG. It requires a high degree of suspicion in a young patient presenting with unilateral upper limb distal muscle weakness with no sensory involvement, as early diagnosis will help in preventing the progression of the disease by employing simple measures like usage of a cervical collar. We report a case of a 25 year old man presenting with progressive distal upper limb muscle wasting, on investigating further diagnosed to have Hirayama disease.
References
Hirayama K, Tsubaki T, Toyokura Y, Okinaka S. Juvenile muscular atrophy of unilateral upper extremity. Neurol. 1963;13(5):373.
Sitt JC, Fung EL, Yuen EH, Ahuja AT. Hirayama disease in a 17-year-old Chinese man. Singapore Med J. 2014;55(6):87.
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 Indian Acad Neurol. 2012;15(2):106.
Nascimento OJ, Freitas MR. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease): a clinical variant of the benign monomelic amyotrophy. Arquivos de neuro-psiquiatria. 2000;58(3):814-9.
Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. American J Neuroradiol. 1998;19(2):365-8.
Kikuchi S, Tashiro K, Kitagawa M, Iwasaki Y, Abe H. A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama's disease)--flexion myelopathy with tight dural canal in flexion. Clin Neurol. 1987;27(4):412-9.
Gandhi D, Goyal M, Bourque PR, Jain R. Case 68: Hirayama Disease 1. Radiology. 2004;230(3):692-6.
Hirayama KE, Tomonaga MA, Kitano K, Yamada T, Kojima S, Arai K. Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol, Neurosurg Psychiatry. 1987;50(3):285-90.
Lin MS, Kung WM, Chiu WT, Lyu RK, Chen CJ, Chen TY. Hirayama disease: clinical article. Journal of Neurosurgery: Spine. 2010;12(6):629-34.
Yoo SD, Kim HS, Yun DH, Kim DH, Chon J, Lee SA, et al. Monomelic amyotrophy (Hirayama disease) with upper motor neuron signs: a case report. Annals of rehabilitation medicine. 2015;39(1):122-7.