CLINICAL, DEMOGRAPHIC AND RADIOLOGICAL FINDINGS IN NEURO-BEHÇET SYNDROME

Yıl 2021, , 6 - 13, 05.01.2021

Fatma Şimşek

https://doi.org/10.18229/kocatepetip.631344

Öz

OBJECTIVE: Neuro-Behcet's Syndrome (NBS), occurs due to central nervous system involvement of Behcet's disease (BD), and it is one of the factors that increase mortality and morbidity. In this study, we aimed to investigate the clinical, demographic and radiological data of Neuro-Behcet patients living in our region.
MATERIAL AND METHODS: In this study, the records of 14 patients who were followed up with the diagnosis of NBS at Atatürk University Neurology Clinic between January 2015 and January 2019 were reviewed retrospectively. Analyses were made by using SPSS 20 statistical program.
RESULTS: The ages of patients were between 18 and 45 years. The average age was 32,07+9,06 years, and NBS onset age was 29,9+6,8 years. % 35,7 (n=5) of patients with NBS were female, and % 64,3 (n=9) of them were male. The most common reason for admission was headache with % 57,1. % 50 of the patients with NBS in magnetic resonance imaging had parenchymal, and 50% of the patients had nonparenchymal involvement. One patient had coexistence of NBS and multiple sclerosis while one patient had an acute infarct area with diffusion restriction in the brain stem. In our study, mortality rate in NBS was % 7,1 (n=1). % 35,7 of the patients had a previously known diagnosis of BS, and % 64,3 (n=9) of them had been diagnosed BD with NBS diagnosis. The result of HLA-B51 was positive in 4 patients.
CONCLUSIONS: The rate of patients with NBS and BD at the same time is significantly higher than the literature. It was thought that the effective factor was low sociocultural level. Early diagnosis and treatment are important factors that reduce the risk of developing NBS. Rarely coexistence of NBS and MS?.

Anahtar Kelimeler

Behcet disease, Multiple sclerosis, Central nerveus system, Early diagnosis

NÖROBEHÇET SENDROMUNDA KLİNİK, DEMOGRAFİK VE RADYOLOJİK BULGULAR

Öz

AMAÇ: Nöro-Behçet sendromu (NBS), Behçet Hastalığının (BH) santral sinir sistemi tutulumuna bağlı ortaya çıkan, mortalite ve morbiditeyi arttıran faktörlerden biridir. Çalışmamızda bölgemizde yaşayan NBS tanılı hastaların klinik, demografik ve radyolojik verilerinin incelenmesi amaçlandı.
GEREÇ VE YÖNTEM: Çalışmamızda Ocak 2015-Ocak 2019 tarihleri arasında Atatürk Üniversitesi Nöroloji kliniğinde NBS tanısı ile takip edilen ve tanı kriterlerini karşılayan 14 hastanın kayıtları retrospektif olarak tarandı. Analizler SPSS 20 istatistik programı kullanılarak yapıldı.
BULGULAR: Hastaların yaşları 18-45 yaş aralığındaydı. Yaş ortalaması 32,07+9,06 yıl, NBS başlama yaşı 29,9+6,8 yıldı. NBS tanılı hastaların % 35,7’si (n=5) kadın, % 64,3 ’ü (n=9) erkekti. Hastaların en sık başvuru sebebi % 57,1 ile baş ağrısıydı. NBS tanılı hastaların manyetik rezonans görüntülemede % 50’sinde parankimal, % 50’sinde nonparankimal tutulum vardı. Bir hastada NBS ve multipl skleroz (MS) birlikteliği, bir hastada beyin sapında difüzyon kısıtlaması olan akut enfarkt alanı vardı. Çalışmamızda NBS’de mortalite oranımız % 7,1 (n=1) idi. Hastaların % 35,7’sinde öncesinde bilinen BH tanısı olup, % 64,3’ü (n=9) NBS tanısı ile birlikte BH tanısını almıştı. HLA-B51 sonucu dört hastada pozitifti.
SONUÇ: NBS ve BH tanısını aynı anda alan hasta oranımız literatüre göre belirgin yüksek olup burada etkili olan faktörün düşük sosyokültürel düzey olduğu düşünüldü. Erken tanı ve tedavi NBS gelişme riskini azaltan önemli bir faktördür. Nadiren NBS ve MS birlikteliği görülebilir.

Anahtar Kelimeler

Behçet hastalığı, multipl skleroz, santral sinir sistemi, erken tanı

Kaynakça

• 1. Behcet H. Uber residivierende, aphtose durch ein Virus verursachtes Gesc-hwure am Mund, am Auge und an der Genitalien. Derm Wschr 1937;105:1152– 57.
• 2. Akman Demir G. Behçet hastalığında nörolojik tutulum. Turkderm 2009;43:61-4.
• 3. Wechsler FB, Davatchi F. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. The Lancet 1990; 335(8697):1078-1080.
• 4. Siva A, Altintas A, Saip S. Behçet's syndrome and the nervous system. Curr Opin Neurol 2004;17(3):347–57.
• 5. Siva A, Kantarcı O, Saip S, et al. Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 2001;248(2):95-103.
• 6. Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications of Behcet’s syndrome. Brain 1999;122(11):2183–94.
• 7. Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behcet’s disease: evaluation of patients. The Neuro-Behcet Study Group. Brain 1999;122(11):2171–82
• 8. Koçer N, Islak C, Siva A, et al. CNS involvement in neuro-Behçet syndrome: an MR study. American Journal of Neuroradiology 1999;20(6):1015-24.
• 9. Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behçet disease. A review. Neurologist 2005;11(2):80–9.
• 10. Cavaco S, da Silva AM, Pinto P, et al. Cognitive functioning in Behçet's disease. Ann N Y Acad Sci 2009;1173(1):217–26.
• 11. Siva A, Saip S. The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J neurol 2009;256(4):513-29.
• 12. Farah S, Al-Shubaili A, Montaser A. Behçet’s syndrome. A report of 41 patients with emphasis on neurological manifestations. J Neurol Neurosurg Psychiatry. 1998; 64(3):382–384.
• 13. Saip S, Siva A, Altintas A, et al. Headache in Behçet's syndrome. Headache 2005;45:911–9.
• 14. Borhani Haghighi A, Aflaki E, Ketabchi L. The prevalence and characteristics of different types of headache in patients with Behçet's disease, a case–control study. Headache 2008;48:424–9.
• 15. Aykutlu E, Baykan B, Akman-Demir G, Topcular B, Ertas M. Headache in Behçet's disease. Cephalalgia 2006;26(2):180–6.
• 16. Kontogiannis V and Powell R. Behcet’s disease. Postgrad Med J 2000; 76(900): 629-637.
• 17. Serdaroglu P. Behçet’s disease and the nervous system. J Neurol 1998;245(4):197–205.
• 18. Motomura S, Tabira T, Kuroiwa Y (1980) A clinical comparative study of multiple sclerosis and neuro-Behçet’s syndrome. J Neurol Neurosurg Psychiatry 1980;43(3):210–213.
• 19. Massacesi L, Tramacere I, Amoroso S, et al. Azathioprine versus beta interferons for relapsing-remitting multiple sclerosis: a multicentre randomized non-inferiority trial. PLoS One 2014; 9(11):113371.
• 20. Ticha V, Kalincik T, Havrdova E. Interferon-β or azathioprine as add-on therapies in patients with active multiple sclerosis. Neurol Res 2012;34(10):923–930.
• 21. Yazici H, Fresko I, Yurdakul S. Behcet’s syndrome: disease manifestati-ons, management, and advances in treatment. Nat Clin Pract Rheumatol 2007;3(3):148–55.
• 22. Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characte-ristics, and management. Lancet Neurol 2009;8(2):192-204.
• 23. Bank I, Weart C. Dural sinus thrombosis in Behçet’s disease. Arthritis Rheum 1984;27:816–8.
• 24. Akman-Demir G, Saip S, Siva A. Behcet’s disease. Current Treatment Options in Neurology, 2011;13:290-310.