Susac syndrome

Case contributed by Craig Hacking
Diagnosis almost certain

Presentation

3 months of mild fluctuating confusion, altered behavior and ataxia. New right-sided hearing loss (SNHL).

Patient Data

Age: 35 years
Gender: Female
mri

Multiple FLAIR hyperintense and diffusion restricted lesions in the corpus callosum, the largest in the splenium on the left. Numerous bilateral white matter lesions in a semiovale distribution of similar signal intensity. No brainstem lesions.

Case Discussion

Given the history and MRI features (particularly the callosal lesions), a provisional diagnosis of Susac syndrome was made with the differential of multiple sclerosis. The patient was treated with steroids and the symptoms and lesions resolved although there was some permanent mild hearing loss.

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