Susac Syndrome (Retinocochleocerebral Vasculopathy)

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BACKGROUND AND CLINICAL INFORMATION: Head  

Age and sex: Usualy seen in young patients and has a definitive female preponderance (M:F=4:1).

Clinical features: Organic brain syndrome of rapid onset is present as the first attack in three-fourth of patients. Encephalopathy is frequently associated with psychiatric features. Retina shows multiple bilateral branch retinal artery occlusion. Hearing impairment is usually an acute onset, bilateral, sensorineuronal hearing loss, especially of lower frequencies. Blindness, deafness, and severe dementia are rare. No death was reported.

NEUROIMAGING: Head  

Cerebral angiography is usually unremarkable. MRI may reveal multifocal hyperintensities on T2-weighed images in the white and gray matter of the cerebrum and infractentorium structures, with gadolinium ehancement during attacks. The MRI picture may suggests multiple sclerosis.

HISTOPATHOLOGY AND IMMUNOHISTOCHEMISTRY: Head  

Multifocal necrosis: Foci of small necrosis measuring up to about 500 micron in diameter, in both the cerebral cortex and white matter with loss of neurons, axons, and myelin.

Vasculopathy: small arterioles about 40-60 micron in diameter may be found in within the infarcts. Adventitia and media of pial and cortical arterioles were thickened.

EM: thick basal lamina in cortical and white matter capillaries.

REFERENCES: Head

Pape, T et al., Medicine 1998 77:3-11.

Petty GW et al., Medicine 1998 77:12-40.

Heiskala H et al., J Neurol Sci 1988 86:239.

Kaminska EA et al., Can J Neurol Sci 1990 17:241.

Monteiro MLR et al., Neurology 1985 35:1113.

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Background    Neuroimaging    Histopathology & Immunohistochemistry   Reference