title: Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.
abstract: Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and anecdotal evidence has suggested a therapeutic role for monoclonal antibodies (Rennebohm et al. 2008, Lee and Amezcua 2009) [3,4]. We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab.
authors.affiliation: Department of Neurology, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, NSW, 2050, Australia. thardy@med.usyd.edu.au
authors.forename: T A
authors.initials: TA
authors.lastname: Hardy
authors.forename: R J
authors.initials: RJ
authors.lastname: Garsia
authors.forename: G M
authors.initials: GM
authors.lastname: Halmagyi
authors.forename: S J G
authors.initials: SJ
authors.lastname: Lewis
authors.forename: B
authors.initials: B
authors.lastname: Harrisberg
authors.forename: M J
authors.initials: MJ
authors.lastname: Fulham
authors.forename: M H
authors.initials: MH
authors.lastname: Barnett
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004829:Epilepsy, Generalized / Q000209:etiology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069285:Infliximab; D008279:Magnetic Resonance Imaging; D009483:Neuropsychological Tests; D011241:Prednisone / Q000627:therapeutic use; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012640:Seizures / Q000209:etiology; D055955:Susac Syndrome / Q000188:drug therapy* / Q000523:psychology; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D014409:Tumor Necrosis Factor-alpha; D003520:Cyclophosphamide; D000069285:Infliximab; D001379:Azathioprine; D011241:Prednisone