[Susac syndrome: neurologic, psychiatric, ophthalmologic otorhinolaryngologic and neuroradiologic features of a rare autoimmune disease].

UNLABELLED: Encephalopathy, recurrent occlusion of retinal arteries and hearing loss comprise the clinical picture of Susac's syndrome. The correct diagnosis is frequently missed because of incomplete clinical signs or negligence of previous symptoms. Early diagnosis and treatment can halt the progression and prevent permanent disability. METHODS: Here, we describe a Hungarian case and review the clinical characteristics, diagnostic procedures and current concepts of therapy. RESULTS: A 30-year-old female was admitted to our neurology department because of change in her personality, apathy, and difficulty in concentration. Brain MRI indicated multiple hyperintense T2-weighted lesions including cerebellum and corpus callosum. Protein content of the CSF was markedly elevated. The recurrent bilateral loss of vision and hearing along with migraine in her previous 2,5-year-long medical history suggested Susac's syndrome. Fundoscopy and fluorescein angiography indicated multiple occlusions of the retinal arteries, audiography revealed bilateral hearing loss. Systemic autoimmune and connective tissue diseases and thrombophilia were excluded. The markedly elevated protein in the cerebrospinal fluid supported Susac's syndrome. Chronic treatment with methylprednisolone resulted in remission of clinical signs. DISCUSSION: Consideration of multiple clinical signs is an important key to the diagnosis of rare clinical entities like Susac's syndrome.