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Hirayama disease

Case contributed by Prashant Gupta
Diagnosis almost certain

Presentation

Bilateral upper limb weakness

Patient Data

Age: 20 years
Gender: Male
mri

Axial and sagittal MRI in neutral position are normal.

Flexion MRI reveals anterior translation of posterior dura with epidural soft tissue (red), dura is represented by a thin hypointense line (blue). T1: note the anterior dural translation in dorsal canal as well with epidural fat prominence (findings easier to appreciate on T1WI). T2: cord is displaced by the epidural soft tissue. Similar findings on a more inferior section, thin dura (blue) well seen to be displaced anteriorly.

Case Discussion

Hirayama disease is a form of muscular dystrophy seen in the 2nd-3rd decades. It is thought to be due to disproportionate growth between the bony spinal canal and canal contents resulting in a lax dura in extension and vice versa.

Contrast-enhanced spinal MRI may be done to demonstrate findings better but is not essential. A dynamic MRI should definitely be done in every suspected case.

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