Susac syndrome

Susac syndrome (SS), also known as SICRET syndrome (small infarctions of cochlear, retinal and encephalic tissue), is a rare syndrome classically affecting females around the 2nd to 4th decades.  It is thought to be caused by a microangiopathy of the brain retina and cochlear.  It is typified by the clinical triad of acute or subacute encephalopathy, bilateral sensorineural hearing loss, and retinal arterial occlusions, although these may take up to 2 years to develop.

The condition more commonly affects females around the 2nd to 4th decades. Approximately three females are afflicted for every male case 9.

It consists of a clinical triad of:

  • acute or subacute encephalopathy which could manifest in a broad spectrum of symptoms, such as: memory impairment, confusion, behavioral disturbances, ataxia, dysarthria, paranoid psychosis, and headaches 8
  • sensorineural hearing loss at low and medium frequencies
  • retinal arterial occlusions leading to scotomata and vision distortion

The primary pathology is a precapiliary arteriole microangiopathy of the brain, retina, and inner ear 9.

MRI 

The brain study typically shows multiple, small lesions which are predominantly supratentorial. There is a predilection for the corpus callosum, though they can also be found in the cerebellum and midbrain. Callosal lesions typically involve the central fibers with relative sparing of the periphery 1.

Signal characteristics include:

  • T1 C+ (Gd): lesions frequently enhance during the acute stage 1
  • T2/FLAIR: lesions are high signal

Usually has a self limiting but fluctuating course. Some patients may have residual neurological sequelae.

Initially described by John O Susac et al in 1979 3.

Considerations on the basis of pure radiographic (MRI) appearances include:

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Article Information

rID: 10736
Section: Syndromes
Synonyms or Alternate Spellings:
  • Retinocochleocerebral vasculopathy
  • Susac's syndrome
  • RED-M
  • SICRET
  • SICRET syndrome

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