A 30-year experience in neuro-Behçet disease

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.