Presentation
Weakness and atrophy of bilateral upper limbs (left greater than right).
Patient Data
CASE OF THE MONTH & YEAR: This case was selected as the Case of the Month for November 2023 and later as the Case of the year for 2023.
There is anterior displacement of the posterior dura from C3 to D1 vertebral level on flexion with enlargement of posterior epidural space with multiple flow voids within the space. Compression over the spinal cord at these levels on flexion with mildly reduced cord bulk in comparison to the neutral position. Intramedullary STIR & T2W hyperintense cord signal abnormality extending from C3 to C6 vertebral level. Posterior dura becomes normal in position in neutral and extension view.
Case Discussion
The clinical details and MRI findings are in keeping with Hirayama disease. It is also known as non-progressive juvenile spinal muscular atrophy.
Co-author: Dr. Sanaullah Mudassir (DM, Neuro-medicine).
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