Flexion Cervical Myelopathy (Hirayama Disease) –  Reality or Myth? Two Case Reports

Authors: Z. Kadaňka Jr;  B. Adamová
Authors‘ workplace: Neurologická klinika LF MU a FN Brno
Published in: Cesk Slov Neurol N 2014; 77/110(3): 362-367
Category: Case Report


Cervical flexion myelopathy (Hirayama disease) is a rare disease of the cervical spine. It is characterized by progressive muscular weakness and atrophy of the distal upper limb (brachioradialis muscle is spared), predominantly affecting male adolescents between 15 and 25 years of age. There is no sensory or deep tendon reflexes involvement. Cervical MRI images show local cord atrophy, T1-weighted images show widened lateral epidural space on flexion on that is hyperintense on T2-weighted, especially contrast-enhanced, images. The existence of the Hirayama disease is disputed by many authors. We present two patients with clinical symptoms of this disease and summarize facts about the diagnosis and treatment of Hirayama disease.

Key words:
Hirayama disease – cervical myelopathy – segmental spinal muscular atrophy – amyotrophy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manu­script met the ICMJE “uniform requirements” for biomedical papers.


1. Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. Psychiatr Neurol Jpn 1959; 61: 2190–2197.

2. Schröder R, Keller E, Flacke S, Schmidt S, Pohl C,Klockgether T et al. MRI findings in Hirayama disease: flexion‑induced cervical myelopathy or intrinsic motor neurone disease? J Neurol 1999; 246(11): 1069– 1074.

3. Kang JS, Jochem‑ Gawehn S, Laufs H, Ferbert A, Vieregge P, Ziemann U. Hirayama disease in Germany: case reports and review of the literature. Nervenarzt 2011; 82(10): 1264– 1272. doi: 10.1007/ s00115- 011- 3320- 9.

4. Ghosh PS, Moodley M, Friedman NR, Rothner AD, Ghosh D. Hirayama disease in children from North America. J Child Neurol 2011; 26(12): 1542– 1547. doi: 10.1177/ 0883073811409226.

5. Finsterer J, Löscher W, Wanschitz J, Baumann M, Quasthoff S, Grisold W. Hirayama disease in Austria. Joint Bone Spine 2013; 80(5): 503– 507. doi: 10.1016/ j.jbspin.2012.10.013.

6. Dejobert M, Geffray A, Delpierre C, Chassande B, Larrieu E, Magni C. Hirayama disease: three cases. Dia­gn Interv Imaging 2013; 94(3): 319– 323. doi: 10.1016/ j.diii.2012.10.008.

7. Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E et al. Nationwide survey of juvenile muscular atrophy of distal upper extremities (Hirayama disease) in Japan. Amyotroph Later Scler 2006; 7(1): 38– 45.

8. Hirayama K. Non‑ progressive juvenile spinal atrophy od the distal upper limb (Hirayama disease). In: De Jong JM (ed). Handbook of clinical neurology. Amsterdam: The Netherlands: Elsevier 1991; 15: 107– 120.

9. Chen CJ, Chen CM, Wu CL, Ro LS, Chen ST, Lee Th.Hiarayama disease: MR dia­gnosis. AJNR Am J Neuroradiol 1998; 19(2): 365– 368.

10. Konno S, Goto S, Murakami M, Mochizuki M, Motegi H, Moriya H. Juvenile amyotrophy of the upper extremity: pathologic fidings of the dura mater and surgical management. Spine 1997; 22(5): 486– 492.

11. Hirayama K, Tomonaga M, 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–  290.

12. Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile of juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases. Rinsho Shinkeigaku 2001; 41(4– 5): 173– 178.

13. Vargas MC, Castillo M. Magnetic resonance imaging in Hirayama Disease. J Radiol Case Rep 2011; 5(3): 17– 23. doi: 10.3941/ jrcr.v5i3.602.

14. 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(5): 576– 582. doi: 10.1002/ mus.20980.

15. Shinomiya K, Dawson J, Spengler DM, Konrad P, Blumenkopf B. An analysis of the posterior epidural ligament role on the cervical spinal cord. Spine 1996; 21(18): 2081– 2088.

16. Willeit J, Kiechl S, Kiechl‑ Kohlendorfer U, Golaszewski S, Peer S, Poewe W. Juvenile asymmetric segmental spinal muscular atrophy (Hirayama’s disease): three cases without evidence of „flexion myelopathy“. Acta Neurol Scand 2001; 104(5): 320– 322.

17. Pradhan S, Gupta RK. Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. J Neurol Sci 1997; 146(2): 133– 138.

18. Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 2000; 54(10): 1922– 1926.

19. Bartlett RJ, Rowland Hill CA, Rigby AS, Chandrasekaran S, Narayanamurthy H. MRI of the cervical spine with neck extension: is it useful? Br J Radiol 2012; 85(1016): 1044– 1051. doi: 10.1259/ bjr/ 94315429.

20. Hirayama K. Juvenile muscular atrophy of unilateral upper extremity (Hiarayama disease) –  half century progress and establishment its discovery. Brain Nerve 2008; 60(1): 17– 29.

21. Harding AE, Bradbury PG, Murray NM. Chronic asymmetric spinal muscular atrophy. J Neurol Sci 1983; 59(1): 69– 83.

22. Hassan KM, Sahni H, Jah 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(2): 106– 112. doi: 10.4103/ 0972– 2327.94993.

23. Agundez M, Rouco I, Barcena J, Mateos B, Barredo J, Zarranz JJ. Hirayama disease: Is surgery an option? Neurologia 2013. doi: 10.1016/ j.nrl.2013.05.005.

24. Watanabe K, Hasegawa K, Hirano T, Endo N, Yamazaki K, Homma T. Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients. J Neurosurg Spine 2005; 3(2): 86– 91.

25. Arrese I, Rivas JJ, Esteban J, Ramos A, Lobato RD. A case of Hirayama disease treated with laminectomy and duraplasty without spinal fusion. Neurochirurgia 2009; 20(6): 555– 558.

Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 3

2014 Issue 3

Most read in this issue
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account