title: Cyclophosphamide and methotrexate in Susac's Syndrome: a successful sequential therapy in a case with involvement of the cerebellum.
abstract: Susac's Syndrome (SS) is a rare disease with unknown aetiology due to a microangiopathy affecting the precapillary arterioles of the brain, retina, cochlea and semicircular canals. Neurological manifestations, visual dysfunction and hearing loss represent the classical clinical triad of SS. Diagnosis is confirmed by laboratory investigations, neuroimaging findings, fluoroangiography and inner-ear studies. An early treatment with steroids and immunosuppressors limits the sequelae of disease. We report a case of SS in which the clinical triad occurred in a very short period of time. Brain MRI showed the involvement of cerebellum, this representing a rare neuroradiological finding in SS. A full remission of disease was obtained by using corticosteroids and cyclophosphamide in the acute-subacute phase and methotrexate as maintenance therapy. This latter has never been used before in SS.
authors.affiliation: Department of Internal Medicine, Immuno-Allergology Unit, University of Pisa, via Roma 67, 56126, Pisa, Italy, eleonoracatarsi@hotmail.com.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Catarsi
authors.forename: Veronica
authors.initials: V
authors.lastname: Pelliccia
authors.forename: Chiara
authors.initials: C
authors.lastname: Pizzanelli
authors.forename: Ilaria
authors.initials: I
authors.lastname: Pesaresi
authors.forename: Mirco
authors.initials: M
authors.lastname: Cosottini
authors.forename: Paola
authors.initials: P
authors.lastname: Migliorini
authors.forename: Antonio
authors.initials: A
authors.lastname: Tavoni
mesh_terms: D001921:Brain / Q000473:pathology; D002526:Cerebellar Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D002531:Cerebellum / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D055955:Susac Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008727:Methotrexate