Download PDF
CC BY-NC-ND 4.0 · Asian J Neurosurg 2020; 15(02): 405-408
DOI: 10.4103/ajns.AJNS_291_19
Case Report

Surgical management of hirayama disease: A rare entity with unusual clinical features

Sudhir Srivastava
Department of Orthopedics, Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra
,
Nandan Marathe
Department of Orthopedics, Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra
,
Aditya Raj
Department of Orthopedics, Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra
,
Sunil Bhosale
Department of Orthopedics, Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra
,
Kiran Dhole
1   Department of Orthopedics, LTMMC and General Hospital, Mumbai, Maharashtra
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Hirayama disease (HD) is a rare type of cervical myelopathy in young males due to neck flexion causing cervical cord atrophy and asymmetric flattening with preferential involvement of anterior horn cells of the spinal cord. This is due to forward displacement of the cord during neck flexion getting compressed between the posterior part of the vertebral body and the posterior dura. The spinal cord involvement occurs due to repeated flexion and extension motion of the neck leading to selective spinal cells injury and atrophy. Most cases report an asymmetric lower motor neuron type of weakness predominantly involving the forearm and hand muscles. We report here a case of HD in an 18-year-old male who presented to us with weakness and wasting in the right hand. The patient was progressively symptomatic over a period of 1 year before presentation. The etiology and the exact cause of HD largely remain debatable and rely on the understanding of few theories which have been put forward. The natural history of this disease reaches a plateau in terms of neurological involvement after 2–5 years and is considered a self-remitting disorder. The patient was initially managed with a cervical collar immobilization but symptoms were largely not improving which was attributed to poor brace compliance. The patient was then managed surgically with a posterior lateral mass instrumentation without fusion in a lordotic alignment at the levels of maximal dural shift anteriorly. The patient improved neurologically following the surgery and maintained the intact status at the last follow-up.

Key-words:

Cervical flexion myelopathy - Hirayama disease - instrumentation without fusion

Financial support and sponsorship

Nil.




Publication History

Received: 18 September 2019

Accepted: 18 February 2020

Article published online:
16 August 2022

© 2020. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity; A new clinical entity. Psychiatr Neurol Jpn. 1959;61:2190-7.
  • 2 Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol 1998;19:365-8.
  • 3 Sonwalkar HA, Shah RS, Khan FK, Gupta AK, Bodhey NK, Vottath S, et al. Imaging features in Hirayama disease. Neurol India 2008;56:22-6.
  • 4 Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E, et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 2006;7:38-45.
  • 5 Huang YC, Ro LS, Chang HS, Chen CM, Wu YR, Lee JD, et al. A clinical study of Hirayama disease in Taiwan. Muscle Nerve 2008;37:576-82.
  • 6 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. Ann Rehabil Med 2015;39:122-7.
  • 7 Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 2000;54:1922-6.
  • 8 Kikuchi S, Shinpo K, Niino M, Higashi T, Tashiro K. Cervical myelopathy due to a “tight dural canal in flexion” with a posterior epidural cavity. Intern Med 2002;41:746-8.
  • 9 Anuradha S, Fanai V. Hirayama disease: A rare disease with unusual features. Case Rep Neurol Med 2016;2016:5839761.
  • 10 Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease): Focal cervical ischemic poliomyelopathy. Neuropathology 2000;20 Suppl:S91-4.
  • 11 Konno S, Goto S, Murakami M, Mochizuki M, Motegi H, Moriya H. Juvenile amyotrophy of the distal upper extremity: Pathologic findings of the dura mater and surgical management. Spine (Phila Pa 1976) 1997;22:486-92.
  • 12 Lin MS, Kung WM, Chiu WT, Lyu RK, Chen CJ, Chen TY. Hirayama disease. J Neurosurg Spine 2010;12:629-34.
  • 13 Xu Q, Gu R, Zhu Q, Suya D. A severe case of Hirayama disease successfully treated by posterior cervical fixation without decompression and fusion. World Neurosurg 2019;122:326-30.
  • 14 Ito H, Takai K, Taniguchi M. Cervical duraplasty with tenting sutures via laminoplasty for cervical flexion myelopathy in patients with Hirayama disease: Successful decompression of a “tight dural canal in flexion” without spinal fusion. J Neurosurg Spine 2014;21:743-52.