Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature

Objective: This study reports five new cases of microangiopathy of the brain, retina, and cochlea (Susac syndrome) and reviews the world's literature. Design: Five cases were systematically studied by the authors. The cases in the literature were identified through Medline searches for Susac syndrome; microangiopathy of the brain, retina, or ear; and cross- referencing the indexes of each retrieved article. Participants: The number of new patients studied in this report was five. An additional 41 patients were culled from the literature. Intervention: Patients were treated with corticosteroids, antineoplastic agents, and other methods in a noncontrolled, nonrandomized fashion. Main Outcome Measures: With respect to therapeutic intervention, the main clinical outcome measures were return of vision, improvement of neurologic and psychiatric manifestations, and recovery of auditory function. Alterations of abnormalities observed by cranial magnetic resonance imaging also were monitored. Results: Of 46 identified patients, 39 were women. The mean age of the patients was 30 years. Forty-one patients (89%) had arterial occlusions, which were bilateral in 60%. Thirty-one patients (67%) reported hearing loss. Twenty patients (44%) had a global encephalopathy, but other neurologic manifestations were common. The mean duration of the illness was 46.7 months. Conclusion: This rare syndrome is more common than previously thought, has a strong female preponderance, and often can be identified at an early stage with a careful history and physical examination. Magnetic resonance imaging of the brain often shows lesions suggestive of multiple sclerosis. Fluorescein angiography may show arteriolar wall hyperfluorescence. Early treatment with corticosteroids often is, although not invariably, associated with a good prognosis. The disease appears to be self-limited in most patients.