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Related Experiment Videos

Behçet's Syndrome and Nervous System Involvement.

Uğur Uygunoğlu1, Aksel Siva2

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

Current Neurology and Neuroscience Reports
|May 24, 2018
PubMed
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.

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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.
Keywords:
Behçet’s syndromeCerebral venous sinus thrombosisHeadacheInfliximabParenchymal neuro-Behçet’s syndrome

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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.