Hesna Bektaş MD, HESNA BEKTAŞ, Şadiye TEMEL, ORHAN DENİZ, Hava Dönmez KEKLİKOĞLU, GÖNÜL VURAL

Sinus Ven Trombozu ile Prezente Olan Nöro-Behçet Hastalığı

Behçet hastalığı (BH) etiyolojisi bilinmeyen, kronik, relapslarla seyreden, multisistem tutulumu yapabilen enflamatuvar bir hastalıktır. Tekrarlayıcı deri mukoza lezyonları ve panüveit hastalığın ana belirtileridir. Başlangıç yaşı genellikle 3. dekaddır ve erkekler kadınlara göre daha ciddi etkilenir. Vasküler tutulum olguların %40'ında görülür. Bütün çap ve tipteki damarlar etkilenmekle birlikte venleri arterlere göre daha sık etkiler. Alt ekstremite ven trombozu hastalığın en sık klinik bulgusu olup bunu vena kava trombozu, pulmoner arter anevrizması, Budd-Chiari sendromu, periferik arter anevrizması, dural sinüs trombozu ve abdominal aorta anevrizması izler. Serebral venöz trombozu %8 oranında görülür. Biz bu yazıda BH'ye bağlı sinus ven trombozu gelişen 28 yaşındaki erkek olguyu sunduk.

Neuro-Behçet Disease Presenting with Sinus Venous Thrombosis: Case Report

Behçet's disease (BD) is a rare, chronic, autoimmune, autoinflammatory disorder of unknown origin. Mucocutaneous lesions and panuveitis constitute the hallmark of Behçet's. The disease is mostly seen at the third decade and disease course is dramatically more severe for males than females. Vascular involvement can occur in up to 40% of the cases. Although veins are mostly involved, all sizes and types of vessels can be affected. The most common clinical presentation of disease is lower extremity vein thrombosis. Vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms are also frequent at the disease's course. Prevalence of cerebral venous thrombosis is 8%. In this article, we presented a 28-year-old man with sinus venous thrombosis due to BD.

PDF

___

1. Siva A, Altintas A, Saip S. Behcet's syndrome and the nervoussystem. Curr Opin Neurol 2004; 17: 347-57.
2. International Study Group for Behcet's Disease.Criteria fordiagnosis of Behcet's disease. Lancet 1990; 335: 1078-80.
3. Ascaso FJ, Rodriguez A, Cristo´bal JA. Cranial hypertension as firstmanifestation of Behcet's disease. Doc Ophthalmol 2002; 105: 291-9.
4. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A, et al. Cerebral venous thrombosis in Behcet's disease. Arthritis Rheum 2009; 61: 518-26.
5. Al-Araji A, Kidd D. Neuro-Behcet's disease: epidemiology,clinical characteristics, and management. Lancet Neurol 2009; 8: 192- 204.
6. Akman- Demir G-, Yeşilot N, Serdaroğlu P. [Neurological involvement in Behçet Disease: Clinical features, diagnosis and treatment approaches] Journal of Neurological Sciences(Turkish) 2006; 23: 003-007.
7. Hatemi G, Fresko I, Tascilar K, Yazici H. Enthesopathy is increased among Behçet's syndrome patients with acne and arthritis: an ultrasonographic study. Arthritis Rheum 2008; 58: 1539-45.
8. Yesilot N, Bahar S, Yilmazer S, Mutlu M, Kurtuncu M, Tuncay R, et al. Cerebral venous thrombosis in Behçet's disease comparedto those associated with other etiologies. J Neurol 2009; 256: 1134-42.
9. Lizarazo-Barrera JC, Jacobelli S, Mellado P, Gonzalez S, Massardo L. Extensive cerebral vein thrombosis as firstmanifestation of Behçet's disease. Report of one case. Rev Med Chil 2010; 138: 746-51.
10. Mossadeq R, Karouache A, Bouraza A, Ouhabi H, Reda R, Boutaleb N, et al. Neuro-Behçet's syndrome and thrombosis of Rosenthal's basilar vein: a report of twelve cases. Rev Neurol 2004; 160: 935-8.
11. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, GulA, et al. EULAR Expert Committee.Management of Behçet'sdisease: a systematic literature review for the EULAR evidence based recommendations EULARExpert Committee. Ann Rheum Dis 2008; 67: 1656-62.