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Behçet’s Syndrome and Nervous System Involvement

  • Demyelinating Disorders (J Bernard and M Cameron, Section Editors)
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Abstract

Purpose of Review

Although Behçet’s syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement “neuro-Behçet’s syndrome (NBS)” is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroimaging findings support two major forms of NBS: parenchymal NBS (p-NBS) and an extra-parenchymal form that presents with cerebral venous sinus thrombosis (CVST). The present review summarizes recent findings on the etiopathogenesis and clinico-radiological features of this disorder as well as its treatment options.

Recent Findings

Recent laboratory and clinical findings include the discovery of antibodies to human and mouse neuro-fibrils showing cross-reactivity with Streptococcus spp. and Mycobacterium tuberculosis heat shock proteins (HSP) in NBS. Diagnostically, two distinct magnetic resonance imaging (MRI) patterns of spinal cord involvement have been observed: (a) “Bagel sign” and (b) “motor neuron” patterns. Therapeutic studies have shown that infliximab effectively prevents further relapses and stabilizes the symptoms of patients experiencing ongoing clinical relapses while being treated with single or multiple immunosuppressant drugs.

Summary

Primary neurological involvement referred to as NBS. The majority of patients with NBS present with parenchymal involvement that commonly affects the brain stem-diencephalic region. Headache, dysarthria, ataxia, and hemiparesis are the main clinical features of NBS.

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

    Uğur Uygunoğlu & Aksel Siva

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  1. Uğur Uygunoğlu
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Uğur Uygunoğlu declares no potential conflicts of interest.

Aksel Siva declares no potential conflicts of interest related to this article. His otherwise disclosures are honoraria and consultation fees from Novartis, Genzyme, Bayer, Biogen Idec, Merck Serono; Genzyme, Teva, and Roche; received travel and registration coverage for attending several national or international congresses or symposia, from Merck Serono, Biogen Idec/Gen Pharma of Turkey, Novartis, Teva; Genzyme and Roche.

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Uygunoğlu, U., Siva, A. Behçet’s Syndrome and Nervous System Involvement. Curr Neurol Neurosci Rep 18, 35 (2018). https://doi.org/10.1007/s11910-018-0843-5

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