Document Detail


Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.
MedLine Citation:
PMID:  21167503     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
T A Hardy; R J Garsia; G M Halmagyi; S J G Lewis; B Harrisberg; M J Fulham; M H Barnett
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-12-17
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  302     ISSN:  1878-5883     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-02-14     Completed Date:  2011-05-27     Revised Date:  2012-03-16    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  126-8     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier B.V. All rights reserved.
Affiliation:
Department of Neurology, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, NSW, 2050, Australia. thardy@med.usyd.edu.au
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MeSH Terms
Descriptor/Qualifier:
Anti-Inflammatory Agents / therapeutic use
Antibodies, Monoclonal / therapeutic use*
Azathioprine / therapeutic use
Brain / pathology
Cognition Disorders / etiology,  psychology
Cyclophosphamide / therapeutic use
Epilepsy, Generalized / etiology
Female
Humans
Immunoglobulins, Intravenous / therapeutic use
Immunosuppressive Agents / therapeutic use
Magnetic Resonance Imaging
Neuropsychological Tests
Prednisone / therapeutic use
Reverse Transcriptase Polymerase Chain Reaction
Seizures / etiology
Susac Syndrome / drug therapy*,  psychology
Tumor Necrosis Factor-alpha / antagonists & inhibitors*
Young Adult
Chemical
Reg. No./Substance:
0/Anti-Inflammatory Agents; 0/Antibodies, Monoclonal; 0/Immunoglobulins, Intravenous; 0/Immunosuppressive Agents; 0/Tumor Necrosis Factor-alpha; 0/infliximab; 446-86-6/Azathioprine; 50-18-0/Cyclophosphamide; 53-03-2/Prednisone
Comments/Corrections
Comment In:
J Neurol Sci. 2012 Mar 15;314(1-2):181-2; author reply 183   [PMID:  22178082 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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