Hirayama disease

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

10 months of right arm weakness.

Patient Data

Age: 20 years
Gender: Male
mri

In neutral position the canal is capacious and no neural exit foraminal stenosis is present. The cord is essentially unremarkable in appearance, although does appear perhaps slightly thinned at the C5/6 level without convincing cord signal abnormality. During flexion there is anterior displacement of the posterior theca such that it compresses the cord between it and the spinal column anteriorly at C5/6. The epidural venous space dorsal to the theca expands to over 6 mm in depth.  

Case Discussion

Features are consistent with and characteristic of Hirayama disease, which is an easily overlooked diagnosis if not suspected. 

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