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CNS Drugs
Erschienen in: CNS Drugs 8/2001

01.08.2001 | Therapy in Practice

CNS Involvement in Primary Sjögren’s Syndrome

Prevalence, Clinical Aspects, Diagnostic Assessment and Therapeutic Approach

verfasst von: Dr Marcello Govoni, Melissa Padovan, Nicoletta Rizzo, Francesco Trotta

Erschienen in: CNS Drugs | Ausgabe 8/2001

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Abstract

Among the systemic manifestations of primary Sjögren’s syndrome, neurological involvement is still an intriguing and debated issue. Although peripheral nervous system abnormalities are a well documented occurrence with a reported prevalence ranging from 10 to 20%, opinions differ as to the prevalence of CNS disease, with suggestions from ‘nonexistent’ to ‘very common’. The lack of agreement probably reflects the different populations selected, different inclusion criteria and lack of rigorous epidemiological studies. In our experience, CNS involvement was detected in 7 of 87 (8%) unselected consecutive patients observed over a period of 5 years.
The spectrum of CNS involvement is wide, including focal, diffuse, neuropsychiatric and spinal cord symptoms, frequently characterised by insidious onset, remitting course and, sometimes, progressive evolution. The diagnostic approach enabling early recognition of this complication relies on careful clinical assessment using history and physical examination combined with neuropsychological testing and instrumental, laboratory and imaging investigations such as magnetic resonance imaging, single photon emission computed tomography, electrophysiological testing and CSF analysis.
The clinical picture often shows spontaneous remission, but when overt neurological symptoms occur or become progressive, therapeutic interventions with high dose corticosteroids and cytotoxic agents, such as intravenous cyclophosphamide pulse therapy, may be indicated.
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Metadaten
Titel
CNS Involvement in Primary Sjögren’s Syndrome
Prevalence, Clinical Aspects, Diagnostic Assessment and Therapeutic Approach
verfasst von
Dr Marcello Govoni
Melissa Padovan
Nicoletta Rizzo
Francesco Trotta
Publikationsdatum
01.08.2001
Verlag
Springer International Publishing
Erschienen in
CNS Drugs / Ausgabe 8/2001
Print ISSN: 1172-7047
Elektronische ISSN: 1179-1934
DOI
https://doi.org/10.2165/00023210-200115080-00003

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