Hirayama disease

Emma Foster; Benjamin K-T Tsang; Anthony Kam; Elsdon Storey; Bruce Day; Aron Hill

doi:10.1016/j.jocn.2014.11.025

Hirayama disease

J Clin Neurosci. 2015 Jun;22(6):951-4. doi: 10.1016/j.jocn.2014.11.025. Epub 2015 Mar 9.

Authors

Emma Foster¹, Benjamin K-T Tsang², Anthony Kam³, Elsdon Storey², Bruce Day⁴, Aron Hill²

Affiliations

¹ Department of Neurosciences, The Alfred Hospital, 55 Commercial Road, Melbourne, VIC 3004, Australia. Electronic address: dr.ecfoster@gmail.com.
² Department of Neurosciences, The Alfred Hospital, 55 Commercial Road, Melbourne, VIC 3004, Australia.
³ Department of Radiology, The Alfred Hospital, Melbourne, VIC, Australia.
⁴ Department of Neurosciences, The Alfred Hospital, 55 Commercial Road, Melbourne, VIC 3004, Australia; Monash University, Melbourne, VIC, Australia.

PMID: 25766368
DOI: 10.1016/j.jocn.2014.11.025

Abstract

This article discusses three patients with likely Hirayama disease. They have no other significant past medical history and no personal or family history of other neurological disorders. Hirayama disease is a form of cervical myelopathy attributed to forward displacement of the posterior cervical dural sac on neck flexion with resultant cord compression and/or venous congestion. It is characterized by a pure motor focal amyotrophy in the distribution of C7, C8 and T1 spinal segmental-innervated muscles and differs from other motor neuron diseases by virtue of its ultimately non-progressive course.

Keywords: Cervical myelopathy; Hirayama disease; Motor focal amyotrophy; Motor neuron disease.

Publication types

Case Reports
Review

MeSH terms

Adult
Cervical Vertebrae / pathology
Humans
Magnetic Resonance Imaging / methods
Male
Spinal Muscular Atrophies of Childhood / diagnosis*
Spinal Muscular Atrophies of Childhood / therapy*
Thoracic Vertebrae / pathology
Young Adult

Supplementary concepts

Amyotrophy, monomelic