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Current Neurology and Neuroscience Reports
Published in: Current Neurology and Neuroscience Reports 7/2018

01-07-2018 | Demyelinating Disorders (J Bernard and M Cameron, Section Editors)

Behçet’s Syndrome and Nervous System Involvement

Authors: Uğur Uygunoğlu, Aksel Siva

Published in: Current Neurology and Neuroscience Reports | Issue 7/2018

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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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Metadata
Title
Behçet’s Syndrome and Nervous System Involvement
Authors
Uğur Uygunoğlu
Aksel Siva
Publication date
01-07-2018
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 7/2018
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-018-0843-5

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