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Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.
Beuker, Carolin; Strunk, Daniel; Rawal, Rajesh; Schmidt-Pogoda, Antje; Werring, Nils; Milles, Lennart; Ruck, Tobias; Wiendl, Heinz; Meuth, Sven; Minnerup, Heike; Minnerup, Jens.
Afiliação
  • Beuker C; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Strunk D; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Rawal R; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Schmidt-Pogoda A; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Werring N; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Milles L; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Ruck T; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Wiendl H; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Meuth S; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Minnerup H; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
  • Minnerup J; From the Department of Neurology with Institute of Translational Neurology (C.B., D.S., A.S.-P., N.W., H.W., J.M.); Institute of Epidemiology and Social Medicine (R.R., H.M.), University of Münster; Department of Neurology (L.M.), University Hospital Essen, University of Duisburg-Essen; and Departme
Neurol Neuroimmunol Neuroinflamm ; 8(6)2021 11.
Article em En | MEDLINE | ID: mdl-34663675
ABSTRACT
BACKGROUND AND

OBJECTIVES:

To facilitate and improve the diagnostic and therapeutic process by systematically reviewing studies on patients with primary angiitis of the CNS (PACNS).

METHODS:

We searched PubMed, looking at the period between 1988 and February 2020. Studies with adult patients with PACNS were included. We extracted and pooled proportions using fixed-effects models. Main outcomes were proportions of patients with certain clinical, imaging, and laboratory characteristics and neurologic outcomes.

RESULTS:

We identified 46 cohort studies including a total of 911 patients (41% biopsy confirmed, 43% angiogram confirmed, and 16% without clear assignment to the diagnostic procedure). The most frequent onset symptoms were focal neurologic signs (63%), headache (51%), and cognitive impairment (41%). Biopsy- compared with angiogram-confirmed cases had higher occurrences of cognitive impairment (55% vs 39%) and seizures (36% vs 16%), whereas focal neurologic signs occurred less often (56% vs 95%). CSF abnormalities were present in 75% vs 65% and MRI abnormalities in 97% vs 98% of patients. Digital subtraction angiography was positive in 33% of biopsy confirmed, and biopsy was positive in 8% of angiogram-confirmed cases. In 2 large cohorts, mortality was 23% and 8%, and the relapse rate was 30% and 34%, during a median follow-up of 19 and 57 months, respectively. There are no randomized trials on the treatment of PACNS. The initial treatment usually includes glucocorticoids and cyclophosphamide.

DISCUSSION:

PACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Vasculite do Sistema Nervoso Central Idioma: En Ano de publicação: 2021 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Vasculite do Sistema Nervoso Central Idioma: En Ano de publicação: 2021 Tipo de documento: Article