Hirayama’s Disease

Main Article Content

Ekasit Leackong, MD
Kongkiat Kulkantrakorn, MD

Abstract

Hirayama’s disease, also known as juvenile non-progressive cervical amyotrophy or monomelic amyotrophy is a rare focal motor neuron disease that primarily affects young Asian males (15-25 years old). The À rst case was reported by Hirayama in 1959. The patient often has insidious and slowly progressive weakness, followed by a spontaneous arrest within several years. Even though its etiology has not been clearly identiÀ ed, the hypothesis of dynamically chronic compression of cervical spinal cord was proposed. This is demonstrated by magnetic resonance imaging (MRI) of cervical spine which shows forward displacement of the posterior wall of the lower cervical dural canal. It leads to marked, often asymmetric, Á attening of the lower cervical cord. Early diagnosis is key to successful management. Surgical decompression in some cases and physical therapy are the treatment options.1-3 This is a report of a you

Article Details

How to Cite
1.
Leackong E, Kulkantrakorn K. Hirayama’s Disease. BKK Med J [Internet]. 2019 Sep. 28 [cited 2024 Nov. 13];6(1):45. Available from: https://he02.tci-thaijo.org/index.php/bkkmedj/article/view/218619
Section
Case Report

References

1. The Wikimedia Foundation. Monometic Amyotrophy. Accessed June 24, 2013, http://en.wikipedia.org/wiki/Monomelic_amyotrophy
2. Weiss CT. Hirayama’s Disease - Facts and Information. Accessed June 24, 2013, http://www.disabled-world.com/disability/types/hirayamas-disease.php
3. Hirayama K. Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)--half-century progress and establishment since its discovery. Brain Nerve 2008;60:17-29.
4. Ding Y, Wang XB, Li CJ. A clinical research of hirayama disease. Zhonghua Nei Ke Za Zhi 2008;47:991-4.
5. Lim YM, Koh I, Park YM, et al. Exome sequencing identifies KIAA1377 and C5orf42 as susceptibility genes for monomelic amyotrophy. Neuromuscul Disord 2012;22: 394-400.
6. Chen CJ, Chen CM, Wu CL, et al. Hirayama disease: MR diagnosis. AJNR 1998;19:365-8.
7. 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. Ann Indian Acad Neurol 2012;15:106-12.
8. Gandhi D, Goyal M, Bourque PR, et al. Case 68: Hirayama disease. Radiology 2004;230:692-6.
9. Tokumaru Y, Hirayama K. A cervical collar therapy for non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease). Rinsho Shinkeigaku 1992;32:1102-6.
10. Imamura H, Matsumoto S, Hayase M, et al. A case of Hirayama’s disease successfully treated by anterior cervical decompression and fusion. No To Shinkei 2001;53:1033-8.