ISCHEMIC STROKE / ETIOLOGY

Susac syndrome

Created 18/02/2022, last revision 09/09/2022

  • a rare microangiopathy affecting arteries of the brain, retina, and cochlea (retino-cochleo-cerebral dysimmune vasculopathy)
    • in partial forms of this syndrome, only 2 of 3 components of the syndrome are clinically manifest. Examples of this are reports of subacute bilateral sensorineural hearing loss with bilateral retinal artery occlusion but without obvious CNS signs
  • described by John Susac in 1979 (but the first reports appeared in 1973)  (Pfaffenbach, 1973)
  • affects mainly women in the 2nd-4th decade
  • the exact cause is unknown; autoimmune vasculitis is considered

Clinical presentation

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Diagnostic evaluation

Brain imaging (MRI)

  • leukoencephalopathy, mostly supratentorial
  • microinfarcts in the capsula interna – “string of pearls
  • small lesions periventricularly, in the centrum semiovale, cerebellum and the brainstem
  • specific lesions in the corpus callosum – “snowball lesions
  • grey matter involvement and leptomeningeal enhancement may be present
    • such findings are useful in DDx of MS and ADEM
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Ophthalmological examination

  • widefield fluorescein angiogram can show artery occlusion
    • often peripheral branch retinal artery occlusions – BRAOs
    • segmental vessel wall hyperfluorescence/leakage, capillary dropout, peripheral nonperfusion and peripheral capillary changes
  • search for signs of uveitis

Differential diagnosis

  • demyelinating diseases
    • multiple sclerosis
    • ADEM
  • multi-infarct disorders

Management

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