European Spine Journal (2018) 27:1201–1206
David C. Kieser
· P. J. Cox
· S. C. J. Kieser
Received: 18 December 2017 / Revised: 22 January 2018 / Accepted: 4 March 2018 / Published online: 14 March 2018
© Springer-Verlag GmbH Germany, part of Springer Nature 2018
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
Consent was obtained from the patient to use his de-iden-
tiﬁed clinical data and radiological studies as a published
clinical case illustration.
The patient was a 21-year-old male of mixed Euro-
pean–Indian origin who presented to our service with rapid
onset and progressive weakening of his left hand grip 1 year
* David C. Kieser
Extended author information available on the last page of the article