Susac's syndrome: psychosis as a presenting symptom of rare angiopathy
Koenen, M.; Djadoenath, A.C.
Nederlands Tijdschrift Voor Geneeskunde 159: A8237
2015
ISSN/ISBN: 1876-8784 PMID: 25784062 Document Number: 681550
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.