Fact-sheet: Susac's syndrome - Retinocochleocerebral vasculopathy


Updated on 08/25/2020 at 4:56 PM

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Definition

Probable autoimmune microangiopathy affecting the cerebral, cochlear, and retinal precapillary arteries.
It preferentially affects young women.
The classic findings are:

  • central nervous system dysfunction
  • branch retinal artery occlusions
  • sensorineural hearing loss

Clinical features

  • Encephalopathy
  • Decreased hearing acuity
  • Visual disturbances.

Laboratory findings

  • Lumbar puncture: Variable elevation of CSF protein along with moderate pleocytosis.

CT

Cerebral atrophy in severe cases.

MRI

Multifocal involvement of white matter, gray matter, and the leptomeninges.

  • T2 and T2-FLAIR: Multiple hyperintensities approximately 5 mm in greatest dimension located in the supratentorial white matter.
    The central snowball-like foci sparing the periphery of the corpus callosum are typical.
    The hyperintensities also involve the periventricular subcortical regions and the centrum semiovale.
    The basal ganglia are frequently involved.
  • T1 after gadolinium injection: Enhancement may be present.
  • Diffusion: correlates with microinfarcts. String-of-pearls appearance
  • Diffusion tensor imaging: decreased fractional anisotropy in the prefrontal white matter with involvement of the fibers of the genu of the corpus callosum.

Management

  • Treatment with corticosteroids or immunosuppression.

Differential diagnosis

  • Multiple sclerosis.
  • Acute disseminated encephalomyelitis.