Document Detail

Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome.
MedLine Citation:
PMID:  2327738     Owner:  NLM     Status:  MEDLINE    
Thirteen patients, 11 women and 2 men, developed sensory and autonomic neuronopathies in association with features of primary Sjögren's syndrome. In 11, Sjögren's syndrome had not been previously diagnosed at the time of neurological presentation. All had prominent loss of kinesthesia and proprioception. Pain and thermal sensibility were less severely affected. Most had evidence of autonomic insufficiency. In some this was severe, with Adie's pupils, fixed tachycardia, and orthostatic hypotension. The course ranged from an abrupt, devastating onset to indolent progression over years. Stabilization or functional improvement occurred in 6 patients, 2 of whom received no drug therapy. Sensory nerve conduction studies and examination of nerve biopsy specimens demonstrated a wide spectrum in the severity of loss of large myelinated fibers. The cutaneous nerves of 6 patients had perivascular mononuclear infiltrates without necrotizing arteritis. Examination of biopsy specimens of dorsal root ganglia in 3 patients revealed lymphocytic (T-cell) infiltration in the dorsal roots and ganglia, with focal clusters around neurons. In the more mildly affected ganglia, individual sensory neurons were undergoing degeneration. In the most advanced case, very few neurons remained. The possibility of Sjögren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss.
J W Griffin; D R Cornblath; E Alexander; J Campbell; P A Low; S Bird; E L Feldman
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of neurology     Volume:  27     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1990 Mar 
Date Detail:
Created Date:  1990-05-21     Completed Date:  1990-05-21     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  304-15     Citation Subset:  IM    
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
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MeSH Terms
Ataxia / etiology*,  pathology,  physiopathology
Ganglia, Spinal / pathology,  physiopathology*
Middle Aged
Neuritis / etiology*,  pathology,  physiopathology
Sjogren's Syndrome / complications*
Spinal Cord Diseases / etiology*,  pathology,  physiopathology
Grant Support
NS23936/NS/NINDS NIH HHS; P01-22849//PHS HHS

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