CASE HISTORY – August 2015

Retinocochleocerebral vasculopathy or Susac’s syndrome. A review and a case report

Authors: Inger Noor, Kai Noor, Anu Saar, Andres Nurmiste, Andrus Kreis

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Susac’s syndrome is a rare disease vith a clinical triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusion (BRAO). It occurs more frequently among young women between the ages of 20–44. The main factor causing Susac`s syndrome is thought to be autoimmune microangiopathy and the disease has a self-limiting nature. The diagnosis of Susac’s syndrome is based primarily on the clinical picture. The treatment of Susac’s syndrome is similar to the treatment of juvenile dermatomyositis, owing to their similar immune pathogenesis. Susac’s syndrome should be considered one of the possibilities for differential diagnosis in the case of an unclear neurologic, ophthalmologic or cochlear finding. The reported case is a good example of cooperation between neurologist, radiologist, ophthalmologist, clinical psychiatrist and otorhinolaryngologist.