Iranian Journal of Radiology

Published by: Kowsar

Hirayama Disease: Best Depicted in the Prone Position with Full-Flexion Cervical MRI in A Serial Trial

Yu Hui Won 1 , 2 and Eun Hae Park 2 , 3 , *
Authors Information
1 Department of Physical Medicine and Rehabilitation, Research Institute of Clinical Medicine of Chonbuk National University, Chonbuk National University Medical School, Jeonju, Republic of Korea
2 Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Republic of Korea
3 Department of Radiology, Chonbuk National University Medical School, Research Institute of Clinical Medicine of Chonbuk, Jeonju, Republic of Korea
Article information
  • Iranian Journal of Radiology: April 30, 2019, 16 (2); e80866
  • Published Online: January 21, 2019
  • Article Type: Case Report
  • Received: June 14, 2018
  • Revised: November 22, 2018
  • Accepted: November 26, 2018
  • DOI: 10.5812/iranjradiol.80866

To Cite: Won Y H, Park E H. Hirayama Disease: Best Depicted in the Prone Position with Full-Flexion Cervical MRI in A Serial Trial, Iran J Radiol. 2019 ; 16(2):e80866. doi: 10.5812/iranjradiol.80866.

Copyright © 2019, Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.
1. Introduction
2. Case Presentation
3. Discussion
  • 1. Sobue I, Saito N, Iida M, Ando K. Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol. 1978;3(5):429-32. doi: 10.1002/ana.410030512. [PubMed: 727722].
  • 2. 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(1):38-45. doi: 10.1080/14660820500396877. [PubMed: 16546758].
  • 3. Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease). Handbook of clinical neurology, diseases of the motor system. Amsterdam: Elsevier Science Publishers; 1991. p. 107-20.
  • 4. Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch Neurol. 1984;41(4):388-94. [PubMed: 6703940].
  • 5. 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]. Rinsho Shinkeigaku. 1987;27(4):412-9. Japanese. [PubMed: 3621739].
  • 6. Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee TH. Hirayama disease: MR diagnosis. AJNR Am J Neuroradiol. 1998;19(2):365-8. doi: 10.1016/j.jocn.2014.11.025. [PubMed: 9504496].
  • 7. Foster E, Tsang BK, Kam A, Storey E, Day B, Hill A. Hirayama disease. J Clin Neurosci. 2015;22(6):951-4. doi: 10.1016/j.jocn.2014.11.025. [PubMed: 25766368].
  • 8. Kontzialis M, Yahyavi-Firouz-Abadi N, Zamora CA. Hirayama disease: The importance of adequate flexion MRI for diagnosis. Neurol Sci. 2015;36(9):1695-6. doi: 10.1007/s10072-015-2222-1. [PubMed: 25896623].
  • 9. Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology. 2000;54(10):1922-6. doi: 10.1007/s004150050514. [PubMed: 10822430].
  • 10. Schroder R, Keller E, Flacke S, Schmidt S, Pohl C, Klockgether T, et al. MRI findings in Hirayama's disease: Flexion-induced cervical myelopathy or intrinsic motor neuron disease? J Neurol. 1999;246(11):1069-74. [PubMed: 10631640].
  • 11. 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(10):1102-6. Japanese. [PubMed: 1297554].
  • 12. Tokumaru Y, Hirayama K. [Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases]. Rinsho Shinkeigaku. 2001;41(4-5):173-8. Japanese. [PubMed: 11676157].
  • 13. Paredes I, Esteban J, Ramos A, Gonzalez P, Rivas JJ. A severe case of Hirayama disease successfully treated by anterior cervical fusion. J Neurosurg Spine. 2014;20(2):191-5. doi: 10.3171/2013.10.SPINE13508. [PubMed: 24286527].
  • 14. Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang YC, et al. Hirayama flexion myelopathy: Neutral-position MR imaging findings-importance of loss of attachment. Radiology. 2004;231(1):39-44. doi: 10.1148/radiol.2311030004. [PubMed: 15068939].
  • 15. Lai V, Wong YC, Poon WL, Yuen MK, Fu YP, Wong OW. Forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in Hirayama disease: An initial study on normal subjects compared to patients with Hirayama disease. Eur J Radiol. 2011;80(3):724-8. doi: 10.1016/j.ejrad.2010.07.021. [PubMed: 20727701].
  • 16. Lehman VT, Luetmer PH, Sorenson EJ, Carter RE, Gupta V, Fletcher GP, et al. Cervical spine MR imaging findings of patients with Hirayama disease in North America: A multisite study. AJNR Am J Neuroradiol. 2013;34(2):451-6. doi: 10.3174/ajnr.A3277. [PubMed: 22878010].

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