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Behçet's Syndrome and Nervous System Involvement.
1Cerrahpaşa School of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey.
Current Neurology and Neuroscience Reports
|May 24, 2018
Summary
Neuro-Behçet's syndrome (NBS) involves brain inflammation and can present with parenchymal or venous sinus issues. New research highlights diagnostic MRI patterns and effective infliximab treatment for this rare neurological disorder.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Behçet's syndrome (BS) is rare, but neuro-Behçet's syndrome (NBS) is a key differential diagnosis for CNS inflammatory and vascular disorders.
- NBS manifests in two primary forms: parenchymal NBS (p-NBS) and extra-parenchymal NBS associated with cerebral venous sinus thrombosis (CVST).
Purpose of the Study:
- To review recent findings on the etiopathogenesis of neuro-Behçet's syndrome.
- To summarize the clinico-radiological features of NBS.
- To discuss current treatment options for NBS.
Main Methods:
- Review of recent laboratory and clinical findings.
- Analysis of diagnostic magnetic resonance imaging (MRI) patterns.
- Evaluation of therapeutic studies on immunosuppressants and infliximab.
Main Results:
- Discovery of antibodies to neuro-fibrils with cross-reactivity to bacterial heat shock proteins in NBS patients.
- Identification of two distinct MRI patterns for spinal cord involvement: 'Bagel sign' and 'motor neuron' patterns.
- Infliximab demonstrates efficacy in preventing relapses and stabilizing symptoms in patients with ongoing NBS relapses.
Conclusions:
- NBS, primarily affecting the brainstem-diencephalic region, presents with symptoms like headache, dysarthria, ataxia, and hemiparesis.
- Advances in understanding NBS include novel autoantibodies and specific MRI findings.
- Infliximab offers a promising therapeutic option for managing NBS relapses and symptoms.