Hirayama disease

Case contributed by Andrew Dixon
Diagnosis certain

Presentation

Bilateral hand muscle wasting.

Patient Data

Age: 30 years
Gender: Male

MRI Cx Spine neutral position

mri

Cervical spine MRI images in the neutral position show marked lower cervical cord atrophy with some linear high T2 cord signal.

MRI Cx Spine flexed position

mri

MRI cervical spine images in the flexed position demonstrate anterior displacement of the dura from C4 to T1 with expanded enhancing posterior epidural space. Cord atrophy due to this phenomenon is known as Hirayama disease. 

Case Discussion

Marked lower cervical cord atrophy which is confirmed on post contrast flexion imaging to be due to Hirayama disease. This disease classically presents with bilateral forearm and hand muscle wasting.

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