Evaluation of disease and treatment-response characteristics of patients with neurologic involvement of behcet's disease registered in Hacettepe University vasculitis research, diagnosis and treatment center

Bölek, EÇ. Evaluation of Disease and Treatment-Response Characteristics of Patients with Neurologic Involvement of Behcet's Disease Registered in Hacettepe University Vasculitis Research, Diagnosis and Treatment Center. Hacettepe University Faculty of Medicine, Thesis in Internal Medicine Department. Ankara, 2018. In this study, we aimed to evaluate disease and treatment response characteristics of patients with neurologic involvement of Behcet's Disease (BD) registered in Hacettepe University Vasculitis Research, Diagnosis and Treatment Center (HUVAC). Among 456 registered patients whose data was available, 329 fulfilled the diagnostic criteria of International Study Group (ISG) for BD. Ninety patients who did not met criteria were also considered to have incomplete BD after review of an experienced rheumatologist. In the final analysis 77/419 (18.4%) patient were classified as Neuro-Behcet's disease (NBD) according to the International Criteria of Recommendations (ICR). Of these patients, 61 (79.2%) had definite and 16 (20.8%) had possible NBD. Forty seven (61%) of the patients were male and 30 (39%) of the patients were female. The mean age at diagnosis of Behçet's disease was 27.7 ± 9.4 years. Patients with NBD had more ocular involvement than those without NBD (65.8% versus 47.2%, p = 0.003). Brainstem was the most frequently affected area and the most common neurological findings were pyramidal findings (64%), extrapyramidal findings and ataxia (58%), sensory findings (56%), respectively. Patients with acute onset isolated or mixed parenchymal disease, 58.3% had only one attack. Median event-free survival time was 113 months in the follow-up time of 36 months (min: 3,5, max: 404 months). There were fourteen death records and 9 of these patients were in the NBD group. No death was recorded in non-parenchymal NBD group. No further conclusions on the effect of treatment on outcomes could be made due to data limitations. Key words: Behcet Syndrome; Neurologic Manifestations; Magnetic Resonance Imaging, Signs and Symptoms, Event-Free Survival