Case Report – Mri Findings In Hirayama Disease Hirayama Disease----A Case Report
Journal Title: IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) - Year 2017, Vol 16, Issue 12
Abstract
We report the MR findings in a case of Hirayama disease, a kind of cervical myelopathy related to flexion movements of the neck. In flexion MR studies, we can see the striking and pathognomonic picture of anterior shifting of posterior dura at the lower cervical spinal canal. In nonflexion studies, we find that asymmetric cord atrophy, especially at the lower cervical cord, though subtle, is highly suggestive of Hirayama disease. When it is seen, a flexion MR study is warranted to prove this diagnosis. Hirayama disease, also termed nonprogressive juvenile spinal muscular atrophy of the distal upper limbs, is a kind of cervical myelopathy related to flexion movements of the neck (1–6). The pathogenetic mechanism of this disease is attributed to forward displacement of the posterior wall of the lower cervical dural canal when the neck is in flexion, which causes marked, often asymmetric, flattening of the lower cervical cord (1, 6–9). We report a case of Hirayama disease and describe the pathognomonic findings at flexion magnetic resonance (MR) imaging. We also discuss the mechanism behind this characteristic appearance and describe findings suggestive of Hirayama disease on routine nonflexion MR studies.
Authors and Affiliations
Dr. Suman Chaudhary, Dr. Ravindra Kumar, Dr. A. P. Verma, Dr. Hemant
Keywords
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- EP ID EP250431
- DOI 10.9790/0853-1010104
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