Susac-syndrome - A retinocochleocerebral angiopathy

Introduction: There has been a series of case reports of otherwise healthy patients suffering from microangiopathy of the brain, retina and cochlea. M ost patients were young women presenting clinically with a subacute encepha lopathy, branch retina artery occclusions, and hearing loss. In 1994 the na me "Susac syndrome" has been proposed for this disease entity. Method: Case report and review of 64 published cases, identified through MEDLINE are gi ven. Case report: We describe a 32-year-old otherwise healthy woman present ing with a subacute encephalopathy, multiple branch retinal artery occlusio ns and bilateral hearing loss. MRI of the brain revealed multiple small whi te and grey matter lesions without contrast enhancement. CSF protein was el evated, oligoclonal bands were negative. lmmunological laboratory parameter s, microbiology, virolology, koagulation studies, SEP, AEP, VEP and cerebra l DSA were normal. Review of the literature: Of 64 identified patients 58 w ere women. The mean age of the patients was 30 years. 60 patients (94%) had arterial occlusions. which were bilateral in 39%. 48 patients reported hea ring loss, 37 patients (58%) had a global encephalopathy, but other neurolo gic manifestations were common. Conclusion: This rare syndrome has a strong young female preponderance. MRI of the brain often shows lesions suggestiv e of multiple sclerosis. Fluorescein angiography may show arteriolar wall h yperfluorescence. Patients can be identified at an early stage with a caref ul history and physical examination. Early treatment with corticosteroids i s often associated with a good prognosis. Cyclophosphamide and antiplatelet drugs may be added in complicated cases.