Prognostic factors for relapse and poor outcome in neuro-Behcet's syndrome: results from a clinical long-term follow-up of a single centre

ŞAHİN EROĞLU D., Torgutalp M., YÜCESAN C., Sezer S., Yayla M. E., BOYVAT A., ...Daha Fazla

JOURNAL OF NEUROLOGY, cilt.269, sa.4, ss.2046-2054, 2022 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 269 Sayı: 4
  • Basım Tarihi: 2022
  • Doi Numarası: 10.1007/s00415-021-10787-0
  • Dergi Adı: JOURNAL OF NEUROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Sayfa Sayıları: ss.2046-2054
  • Anahtar Kelimeler: Behcet's syndrome, Neuro-Behcet's syndrome, Neurological involvement, Prognosis, Relapse, Poor outcome, CEREBRAL VENOUS THROMBOSIS, NEUROLOGICAL INVOLVEMENT, DISEASE, MANIFESTATIONS, PREVALENCE, DIAGNOSIS, MORTALITY, PATTERNS
  • Ankara Üniversitesi Adresli: Evet

Özet

Objective To describe the clinical characteristics of neuro-Behcet's syndrome (NBS) and to define the factors associated with relapses and poor outcome. Methods Among 2118 patients with Behcet's syndrome who fulfilled the international study group criteria, 208 (9.8%) patients had NBS. Retrospective data of 125 NBS patients (55.5% male; mean age 37.2 +/- 11.8 years) were analysed. We divided patients into two subgroups, either parenchymal (p-NBS) or non-parenchymal (np-NBS), according to international consensus recommendations for NBS. We assessed the predictor factors associated with relapse and poor outcome-which was defined as a modified Rankin score (mRS) >= 3 at last follow-up and/or death-using Cox and logistic regression analyses, respectively. Results In total, 79 (63.2%) patients presented with p-NBS and 46 (36.8%) presented with np-NBS. Ocular involvement was more common in p-NBS than np-NBS (55.7% vs. 37.0%, p = 0.04), whereas vascular involvement excluding cerebral vein thrombosis was more frequent in patients with np-NBS (19.0% vs. 52.2%, p < 0.001). Forty-two patients (33.6%) experienced at least one relapse. Factors associated with relapse were BS diagnosis at a younger age and cranial nerve dysfunction (HR 0.96 95% CI 0.93-0.99 and 2.36 95% CI 1.23-4.52, respectively). After a median of 68 (Q1-Q3: 25-125) months, 23 patients (18.4%) had a poor outcome. Indicators of a poor outcome were higher initial mRS and the progressive p-NBS type (OR 8.28 95% CI 1.04-66.20 and 33.57 95% CI 5.99-188.21, respectively). Conclusion Our findings indicate that clinical characteristics and prognosis differ between NBS subgroups, of which patients with p-NBS have worse outcomes.