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.


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