Susac syndrome

Case contributed by Dr Luke Danaher

Presentation

Vision impairment and deafness.

Patient Data

Age: 45 years
Gender: Female
MRI

Scattered T2 FLAIR white matter hyperintensities within the supratentorial brain. These are most pronounced in the periventricular white matter and corpus callosum.

Age advanced callosal atrophy.

No abnormal diffusion restriction is present. There are no retained intracranial blood products.  

Case Discussion

The imaging findings were initially attributed to a primary demyelinating process such as multiple sclerosis.

The patient had a longstanding history of deafness and vision impairment. The diagnosis Susac syndrome is made on the clinical triad of:

  • vision impairment
  • deafness
  • encephalopathy
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Case information

rID: 50606
Case created: 13th Jan 2017
Last edited: 14th Jan 2017
Inclusion in quiz mode: Included

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