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European Spine Journal

, Volume 27, Issue 6, pp 1201–1206 | Cite as

Hirayama disease

  • David C. Kieser
  • P. J. Cox
  • S. C. J. Kieser
Grand Rounds

Abstract

Purpose

Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. It is primarily seen in young males of Indian or Asian descent. With increasing dispersion of these populations this condition is increasingly being encountered internationally. This grand round reviews this rare but increasingly recognized condition.

Materials and methods

We present a classic case of a young Indian male with progressive hand and forearm weakness. We discuss the typical clinical presentation, appropriate investigations and management of this condition.

Results

Our patient presented with oblique amyotrophy and underwent a diagnostic flexion MRI scan which revealed anterior translation of the posterior dura with compression of the anterior horns of the lower cervical cord. He has been successfully treated in a cervical collar.

Conclusions

This case illustrates the typical presentation, diagnostic investigations and treatment of Hirayama syndrome. It is hoped that this review will alert clinicians of this condition and optimize the management of affected individuals.

Keywords

Hirayama Oblique amyotrophy Atrophy Juvenile spinal muscular atrophy Juvenile muscular atrophy Monomelic amyotrophy Asymmetric segmental spinal muscular atrophy 

Notes

Acknowledgements

Glynny Kieser for her editorial input.

Compliance with ethical standards

Conflict of interest

None of the authors have any potential conflict of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • David C. Kieser
    • 1
  • P. J. Cox
    • 2
  • S. C. J. Kieser
    • 3
  1. 1.Department of Orthopaedic Surgery and Musculoskeletal Medicine, Canterbury School of Medicine, Canterbury District Health BoardUniversity of OtagoChristchurchNew Zealand
  2. 2.Division of NeuroradiologyUniversity of OxfordOxfordUK
  3. 3.Department of RadiologyCanterbury District Health BoardChristchurchNew Zealand

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