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Nervous system involvement in Behçet's syndrome.

Uğur Uygunoğlu1, Aksel Siva

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

Current Opinion in Rheumatology
|November 9, 2018
PubMed
Summary

Neuro-Behçet's syndrome (NBS) involves neurological symptoms in Behçet's syndrome patients. Distinct MRI patterns, like the "Bagel Sign," aid diagnosis, guiding treatment for this serious condition.

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Area of Science:

  • Neurology
  • Immunology
  • Radiology

Background:

  • Behçet's syndrome (BS) is a multisystem inflammatory disorder.
  • Neurological involvement (NBS) in BS carries a poor prognosis.
  • Accurate diagnosis of NBS is crucial due to differing treatment options.

Purpose of the Study:

  • To describe the differential diagnosis of neuro-Behçet's syndrome (NBS).
  • To highlight radiological patterns of NBS.
  • To outline treatment options for NBS.

Main Methods:

  • Review of neurological involvement in Behçet's syndrome.
  • Analysis of magnetic resonance imaging (MRI) findings.
  • Consideration of cerebrospinal fluid (CSF) examination.

Main Results:

  • Two distinct MRI patterns of spinal cord involvement in BS: 'Bagel Sign' and 'Motor Neuron' patterns.
  • The 'Bagel Sign' pattern shows a central lesion with specific T2-weighted characteristics.
  • The 'Motor Neuron' pattern involves symmetric anterior horn cell involvement.
  • Infliximab treatment showed efficacy in preventing further attacks and improving neurological status.

Conclusions:

  • Careful diagnosis of NBS is essential, especially when clinical and MRI findings mimic other central nervous system inflammatory disorders.
  • Radiological patterns, particularly MRI findings, are key in diagnosing NBS.
  • Effective treatment strategies, including Infliximab, can improve outcomes for patients with NBS.

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