| Neurological manifestations of primary Sjogren's syndrome. | |
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MedLine Citation:
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PMID: 20689426 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE OF REVIEW: This review summarizes our current understanding of the neurological manifestations of primary Sjogren's syndrome (PSS), their pathophysiology, and treatment. RECENT FINDINGS: Prevalence of neurological manifestations in PSS varies widely from 10 to 60%, with pure or predominantly sensory polyneuropathies as the most common neurologic manifestation (e.g. sensory ataxic or small fiber sensory painful neuropathy). Mononeuropathy multiplex, polyradiculopathy, symptomatic dysautonomia, cranial neuropathy, myopathy, and central nervous system involvement are less common. PSS-associated sensory neuropathy is often the presenting feature of Sjogren's syndrome and, therefore, a high index of suspicion is required, particularly in female patients with nonlength-dependent, painful, or ataxic sensory neuropathies or those with trigeminal sensory and autonomic involvement. The pathophysiological basis of PSS-associated neuropathy is still unclear. Dorsal root ganglionitis and peripheral nerve vasculitis have been observed on histological examination of biopsy and autopsy samples. A few studies have explored the fundamental role of humoral autoimmune mechanisms. Small, uncontrolled, treatment trials with numerous immunomodulatory agents have reported variable benefit in PSS-associated neuropathy, particularly corticosteroids for mononeuritis multiplex and intravenous immunoglobulin for small fiber or sensory ataxic neuropathy. SUMMARY: The clinical and histological spectrum of neurological manifestations of Sjogren's syndrome is becoming clear. The field needs further exploration of basic neuroimmunological mechanisms of neural injury, and controlled treatment trials. |
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Authors:
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Josiah Chai; Eric L Logigian |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Current opinion in neurology Volume: 23 ISSN: 1473-6551 ISO Abbreviation: Curr. Opin. Neurol. Publication Date: 2010 Oct |
Date Detail:
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Created Date: 2010-09-02 Completed Date: 2010-12-14 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9319162 Medline TA: Curr Opin Neurol Country: England |
Other Details:
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Languages: eng Pagination: 509-13 Citation Subset: IM |
Affiliation:
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Department of Neurology, National Neuroscience Institute, Singapore. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Humans Muscular Diseases / immunology, pathology, physiopathology, therapy Peripheral Nerves / pathology, physiopathology Peripheral Nervous System Diseases / immunology, pathology, physiopathology*, therapy Sjogren's Syndrome / immunology, pathology, physiopathology*, therapy |
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