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Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome
T A Hardy1, R J Garsia, G M Halmagyi
1Department of Neurology, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, NSW, 2050, Australia. thardy@med.usyd.edu.au
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.
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