Nöro-Behçet Hastalığında Klinik Özellikler

Özet

Giriş: Behçet hastalığında (BH) nörolojik tutulum nadir olsa da, morbiditenin ana nedenlerinden biridir. Bu çalışmadaNöro-Behçet hastalığının (NBH) klinik ve radyolojik özelliklerinin araştırılması amaçlanmıştır.Yöntemler: Ocak 2011- Aralık 2017 tarihleri arasında nöroloji kliniğinde NBH tanısı alan 22 hastanın demografik veklinik özellikleri retrospektif olarak gözden geçirilmiştir.Bulgular: Hastaların yaş ortalaması 39.18±12.25 olup, erkek/kadın oranı 1 idi. Nörolojik tutulum gelişimine kadar geçenortalama hastalık süresi 8.95±4.28 yıldı. 20 hastada (%90,9) parankimal santral sinir sistemi (SSS) tutulumu mevcutken,1 hastada (%4,5) parankim dışı SSS tutulumu ve 1 hastada (%4,5) periferik sinir sistemi tutulumu mevcuttu. Parankimaltutulumu olan 20 hastanın 16’sında (%80) beyinsapı, diensefalon ve/veya bazal gangliyon tutulumu mevcutken, 9hastada (%45) hemisferik tutulum, 1 hastada (%5) meningial tutulum ve 3 hastada (%15) spinal kord tutulumumevcuttu. 1 hasta enfeksiyon nedeni ile kaybedildi. 21 hastanın 16’sının (%76,1) 3. Ay takip verisi mevcuttu. 5 hastanınnörolojik muayenesi normal (%31,25), 1 hastada (%6,25) sadece duyusal bulgular mevcut, 7 hastada (%37,5) belirti vebulgularda kısmi düzelme olup mobilize iken, 3 hasta (%18,75) ağır sekelle immobildi.Sonuç: BH’da nörolojik tutulum farklı klinik tablolar şeklinde ortaya çıkabilmektedir. NBH’da yüksek morbidite vemortalite riski, erken ve doğru tedavinin önemi akılda tutularak BH olan hastaların nörolojik belirti ve bulguları dikkatledeğerlendirilmelidir.

Abstract

Objective: Neurological involvement in Behcet’s disease (BD) is rare, although it is one of the main cause of morbidity. The aim of this study was to investigate the clinical and radiological characteristics of Neuro-Behçet’s disease (NBD). Methods: Demographic and clinical features of 22 patients with NBD diagnosed at neurology department between January 2011 and December 2017 were reviewed retrospectively. Results: The mean age of the patients was 39.18±12.25 years and male to female ratio was 1. Average disease duration before the onset of neurological involvement was 8.95±4.28 years. Twenty patients (90.9%) had parenchymal central nervous system (CNS) involvement, one patient (4.5%) had non-parenchymal CNS involvement and one patient (4.5%) had peripheral nervous system involvement. Sixteen (80%) out of 20 patients with parenchymal involvement had brainstem, diencephalon and / or basal ganglion, 9 (45%) had hemispheric, one (5%) had meningeal and three (15%) had spinal cord involvement. One patient died because of infection. 16 (76.1%) out of 21 patients had 3 month follow-up data. Neurological examination was unremarkable in five patients (31.25%), one patient (6.25%) had only sensory signs, seven (37.5%) had partial improvement in symptoms and signs and were mobile, while three (18.75%) were immobile with severe sequelae. Conclusion: Neurological involvement in BD may present with various clinical manifestations. The neurological symptoms and signs of patients with BD should be carefully evaluated, keeping in mind the high risk of morbidity and mortality in NBH and the importance of early and correct treatment.