title: [Susac syndrome].
abstract: Susac syndrome is a mysterious vasculopathy affecting brain, retina and inner ear in young women. Main features of the disease are increasingly recognized: subacute encephalopathy often mimicking psychosis and frequently heralded with unusual ophthalmic migraine; frequent subclinical meningitis; brain MRI with multiple and bilateral white and gray matter nuclei lesions, with prominent involvement of corpus callosum; bilateral involvement of central retina artery branches, not only with occlusions but also with peculiar leakage of fluorescein through arteriolar walls on late stages of angiography; non-specific bilateral cochleovestibular symptoms with audiogram showing perception hypoacousia that predominates on low frequencies. Outcome, prognosis, pathogenesis and a rational basis for treatment are discussed in this review. A key message for the clinician should be to perform brain MRI, audiogram and retinal angiography whatever the mode of entry, in order not to miss one (or two) features of this syndrome triad.
authors.affiliation: Service de médecine interne, hôpital Bichat, 46, rue Henri-Huchard, 75018 Paris, France. thomas.papo@bch.aphp.fr
authors.forename: T
authors.initials: T
authors.lastname: Papo
authors.forename: I
authors.initials: I
authors.lastname: Klein
authors.forename: K
authors.initials: K
authors.lastname: Sacré
authors.forename: S
authors.initials: S
authors.lastname: Doan
authors.forename: B
authors.initials: B
authors.lastname: Bodaghi
authors.forename: F
authors.initials: F
authors.lastname: Aubart-Cohen
mesh_terms: D000792:Angiography; D001299:Audiometry; D001921:Brain / Q000473:pathology*; D003937:Diagnosis, Differential; D007758:Ear, Inner / Q000473:pathology*; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D011379:Prognosis; D012160:Retina / Q000473:pathology*; D055955:Susac Syndrome / Q000188:drug therapy / Q000473:pathology*
chemical_list: D007155:Immunologic Factors