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Susac Syndrome

Description

Susac Syndrome (named for the first author of the first paper on the syndrome) is a rare autoimmune endotheliopathy with microangiopathic occlusive arteriolar disease. It may be misdiagnosed as multiple sclerosis or acute disseminated encephalomyelitis (ADEM).


 

Classic triad (may be absent at presentation):

(1) encephalopathy with neuropsychological deficits

(2) branch retinal artery occlusions with visual loss

(3) sensorineural hearing loss

 

Additional findings may include:

(1) migraine

(2) tinnitus

 

MRI of the brain shows multifocal lesions in the white matter and corpus callosum. These are hyperintense in FLAIR images. Lesions in the corpus callosum are centrally located and have a punched-out appearance. Leptomeningeal enhancement may be present.

 

Fluorescein angiography of the retina shows multifocal fluorescence with a distinctive pattern.

 

Laboratory testing may show the presence of anti-endothelial cell antibody (AECA) which can be demonstrated by indirect immunofluorescence.

 

Patients often respond to intravenous corticosteroids and immunosuppressive therapy. Some patients may have a spontaneous recovery.

 


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Specialty

ICD-10