Document Detail


Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica.
MedLine Citation:
PMID:  19692434     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To evaluate clinical characteristics, aquaporin (AQP)-4 antibody results, and probability of developing symptoms of neuromyelitis optica (NMO) in patients with Sjögren's syndrome myelopathy (SSM). METHODS: We identified eight patients with spinal cord involvement from 112 patients with Sjögren's syndrome (SS) referred to the neurology department. The clinical characteristics and AQP-4 antibody status, based on immunoprecipitation of EGFP-tagged AQP-4, of the patients with SSM were assessed. RESULTS: All patients with SSM had extensive spinal cord lesions, high mean annual relapse rates, and poor response to steroid treatment. Of the eight patients with SSM, seven patients satisfied the revised diagnostic criteria for NMO or showed positive results from AQP-4 antibody testing; one patient had incomplete follow-up. The clinical manifestations and AQP-4 autoantibody status of patients with SSM did not differ significantly from those of NMO patients without SS. CONCLUSION: All patients with SSM had poor prognosis with high mean annual relapse rates, and most seemed to have the clinical and immunological characteristics of NMO. Early aggressive immune therapies should be considered in patients with SSM irrespective of the presence or absence of optic neuritis.
Authors:
S M Kim; P Waters; A Vincent; S Y Kim; H J Kim; Y H Hong; K S Park; J H Min; J J Sung; K W Lee
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Multiple sclerosis (Houndmills, Basingstoke, England)     Volume:  15     ISSN:  1352-4585     ISO Abbreviation:  Mult. Scler.     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-20     Completed Date:  2009-10-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9509185     Medline TA:  Mult Scler     Country:  England    
Other Details:
Languages:  eng     Pagination:  1062-8     Citation Subset:  IM    
Affiliation:
Department of Neurology, Seoul National University, College of Medicine, Seoul, Korea.
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MeSH Terms
Descriptor/Qualifier:
Adult
Age of Onset
Aquaporin 4 / metabolism
Brain / metabolism,  pathology
Female
Humans
Immunohistochemistry
Magnetic Resonance Imaging
Middle Aged
Myelin Sheath / metabolism,  pathology*
Neuromyelitis Optica / drug therapy,  metabolism,  pathology*
Recurrence
Retrospective Studies
Sjogren's Syndrome / drug therapy,  metabolism,  pathology*
Spinal Cord / metabolism,  pathology*
Steroids / therapeutic use
Chemical
Reg. No./Substance:
0/AQP4 protein, human; 0/Aquaporin 4; 0/Steroids

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