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Neuroimmune Diseases pp 683–697Cite as

Behçet’s Syndrome and the Nervous System Involvement

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Part of the book series: Contemporary Clinical Neuroscience ((CCNE))

Abstract

Behçet’s syndrome (BS), a distinct disease with orogenital ulceration and uveitis known as the triple-symptom complex, is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. The neurological involvement of BS is termed as neuro-Behçet’s syndrome (NBS). Direct neurological involvement of BS may be classified into two forms: (1) parenchymal (p-NBS) and (2) vascular involvement. p-NBS with the rate of 75% among neurological involvements of BS usually presents with an acute-subacute brain stem syndrome. Headache, cranial neuropathy, dysarthria, ataxia, and hemiparesis are the most prominent symptoms. The most common areas affected in p-NBS are the mesodiencephalic junction (MDJ), pons, and medulla oblongata. MDJ lesions tend to extend upward to involve the diencephalic structures and downward to involve the pontobulbar region which is the most common radiological finding observed in p-NBS. The only drug that has been shown to be effective based on the Class IV evidence is infliximab for the treatment of p-NBS.

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Authors and Affiliations

  1. Istanbul University, Cerrahpaşa School of Medicine, Department of Neurology, Istanbul, Turkey

    Ugur Uygunoglu & Aksel Siva

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  1. Ugur Uygunoglu
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Editors and Affiliations

  1. Medical Education Promotion Center, Tokyo Medical University, Tokyo, Japan

    Hiroshi Mitoma

  2. Department of Neurology, CHU-Charleroi, Charleroi, Belgium, Department of Neurosciences, University of Mons, Mons, Belgium

    Mario Manto

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Uygunoglu, U., Siva, A. (2019). Behçet’s Syndrome and the Nervous System Involvement. In: Mitoma, H., Manto, M. (eds) Neuroimmune Diseases. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-030-19515-1_22

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