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Related Experiment Video

Updated: Sep 20, 2025

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A 30-year experience in neuro-Behçet disease.

Lénia Silva1, Isabel Fonseca Silva2, Tomás Fonseca3

  • 1Neurosciences Clinic, Centro Hospitalar Universitário de Santo António (CHUdSA), ULS de Santo António, Porto, Portugal.

Journal of Neuroimmunology
|May 25, 2025
PubMed
Summary

Neuro-Behçet (NBD), a severe form of Behçet disease (BD), affects over 30% of BD patients, with brainstem and multifocal syndromes being most common. Anti-TNFα therapy shows promise for treating NBD.

Keywords:
Behçet's diseaseDiagnosisNeuro-BehçetOutcomeTreatment

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Area of Science:

  • Neurology
  • Rheumatology
  • Immunology

Background:

  • Behçet disease (BD) is a systemic vasculitis impacting multiple organs.
  • Neuro-Behçet (NBD) represents a severe manifestation of BD, associated with significant morbidity, disability, and mortality.

Purpose of the Study:

  • To analyze the prevalence, clinical characteristics, and outcomes of neurological involvement in Behçet disease patients.
  • To identify risk factors and evaluate treatment efficacy in Neuro-Behçet disease.

Main Methods:

  • Retrospective analysis of 296 Behçet disease patients from 1993 to 2023.
  • Evaluation of neurological symptoms, brain MRI findings, HLA-B*51 allele prevalence, and treatment responses.

Main Results:

  • 31.4% of BD patients had neurological evaluation; 10.1% were diagnosed with definite NBD.
  • Brainstem and multifocal syndromes were most common in definite NBD, with ataxia as a frequent sign.
  • HLA-B*51 allele was more prevalent in definite NBD patients (53.8%) compared to BD patients (31.2%).
  • Cyclophosphamide and infliximab demonstrated better outcomes compared to other treatments.

Conclusions:

  • Neuro-Behçet disease exhibits distinct features, including potential onset preceding BD diagnosis and no sex predominance.
  • The HLA-B*51 allele appears to be a risk factor for definite NBD.
  • Anti-tumor necrosis factor-alpha (Anti-TNFα) biological therapy is effective in managing NBD.