Article

Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.

Department of Neurology, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, NSW, 2050, Australia.
Journal of the neurological sciences (impact factor: 2.32). 03/2011; 302(1-2):126-8. DOI:10.1016/j.jns.2010.11.016 pp.126-8
Source: PubMed

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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Keywords

anecdotal evidence
 
branch retinal artery occlusions
 
clinical triad
 
immune-mediated endotheliopathy
 
immunosuppressive therapy
 
Lee
 
monoclonal antibodies
 
Neumayer
 
sensorineural hearing loss
 
Susac's syndrome
 
therapeutic role
 
tumour necrosis factor
 
young females
 

T A Hardy