PMID: 25784062Mar 19, 2015Paper

Susac's syndrome: psychosis as a presenting symptom of rare angiopathy

Nederlands tijdschrift voor geneeskunde
Maarten Koenen, Anthony C Djadoenath

Abstract

Susac's syndrome is a rare angiopathy characterised by the triad of encephalopathy, hearing loss and retinal artery occlusions. The diagnosis relies on the clinical picture, brain MRI scan, audiometry, fundoscopy and fluorescein angiography. Swift, intensive and prolonged treatment with immunosuppressants, immunoglobulins, plasmapheresis and anticoagulants can result in full or partial remission. A 31-year-old woman became increasingly confused while on holiday in Aruba. On returning to Holland she was admitted for a psychotic episode. Because of the atypical nature of the psychiatric symptoms and subtle neurological deficits, extensive somatic investigations were conducted. Typical lesions in the corpus callosum were found on brain MRI. After treatment with immunosuppressants the psychotic symptoms disappeared. In Susac's syndrome psychiatric symptoms can initially dominate the clinical picture. To diagnose this condition correctly, extensive multidisciplinary collaboration is essential.

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