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Prognosis of Neuro-Behçet’s Syndrome

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Neuro-Behçet’s Disease

Abstract

Behçet’s syndrome is a multisystemic and inflammatory vasculitis. Major causes of mortality have been reported as large vessel disease and major organ involvement like parenchymal central nervous system (CNS) disease. Central nervous system (CNS) disease can occur in 5–25% of the patients with Behçet’s syndrome. CNS disease can be either parenchymal or nonparenchymal. Parenchymal neuro-Behçet’s syndrome (NBS) has been described with a poorer prognosis. Among patients with parenchymal NBS, initial presentation with headache and normal neurological examination, disease course with a single episode, and continuous treatment are associated with a relatively better prognosis. Presentation with brain stem, cerebellar, or spinal cord symptoms, frequent relapses, early neurological involvement, early disease progression, high cerebrospinal fluid (CSF) pleocytosis, disability and dependent status at initial presentation, a primary or secondary progressive course, relapse during steroid dose tapering, fever, meningeal signs, bladder involvement, and incomplete recovery are included in poorer prognostic factors. Furthermore, HLA-B51 positivity increases the odds of relapse. Although Behçet’s syndrome has a male predominance, most of the studies suggest that male gender does not affect the disease course and prognosis. Nonparenchymal NBS, especially cerebral venous thrombosis (CVT) cases, tends to have a better prognosis than parenchymal NBS. Poor prognostic factors of CVT in NBS are the presence of coma and intracerebral hemorrhage at presentation.

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Authors and Affiliations

  1. Faculty of Medicine, Department of Neurology, Hacettepe University, Ankara, Turkey

    Nazire Pınar Acar-Özen & Aslı Tuncer

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  1. Nazire Pınar Acar-Özen
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  2. Aslı Tuncer
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Correspondence to Aslı Tuncer .

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Editors and Affiliations

  1. Istanbul University, Istanbul, Turkey

    Erdem Tüzün

  2. Istanbul University, Istanbul, Turkey

    Murat Kürtüncü

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Acar-Özen, N.P., Tuncer, A. (2021). Prognosis of Neuro-Behçet’s Syndrome. In: Tüzün, E., Kürtüncü, M. (eds) Neuro-Behçet’s Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-55273-2_13

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