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Cerebral venous thrombosis in Behçet’s disease compared to those associated with other etiologies

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Abstract

Cerebral venous thrombosis (CVT) is caused by various etiologies. In Mediterranean and Middle Eastern countries, Behçet’s disease (BD) is one of the leading causes of CVT. We aimed to evaluate any differences in CVT patients with and without BD. All registered patients with CVT were evaluated retrospectively. Clinical, neuroradiological findings and follow-up data were compared between patients with BD and patients with other etiologies. There were 36 patients with CVT and BD, and 32 patients with CVT related to other etiological causes. BD patients were younger (median age at onset 26 vs. 39 years; P < 0.001), and there was a male preponderance (28 males, 8 females) as compared to the non-BD group (10 males, 22 females; P < 0.001). Onset was frequently acute in the non-BD group, and it was subacute or chronic in the BD group. Hemi/quadriparesis, aphasia and seizures were significantly more common (P < 0.001) in the non-BD group. In the BD group 94% of the patients presented with symptoms of isolated intracranial hypertension (P < 0.001). Venous infarcts were observed in 63% of the patients with other causes and in 6% of the patients with BD (P < 0.001). At admission 97% of the patients in the BD group and 41% of the patients in the non-BD group had a modified Rankin score of 0–2. Outcome was good in all of the patients with BD and in 91% of patients with other causes. Clinical recurrences were seen in six patients with BD and in one patient without BD. CVT associated with BD has a subacute onset, mostly presents with signs of isolated intracranial hypertension and venous infarction rarely develops; these features distinguish CVT due to BD from those with other causes.

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References

  1. Criteria for diagnosis of Behcet’s disease (1990) International Study Group for Behcet’s Disease. Lancet 335:1078–1080

    Google Scholar 

  2. Akman-Demir G, Bahar S, Baykan-Kurt B, Gurvit IH, Serdaroglu P (1996) Intracranial hypertension in Behcet’s disease. Eur J Neurol 3:66–70

    Google Scholar 

  3. Akman-Demir G, Serdaroglu P, Tasci B (1999) Clinical patterns of neurological involvement in Behcet’s disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain 122(Pt 11):2171–2182

    Article  PubMed  Google Scholar 

  4. Bahar S, Coban O, Gurvit IH, Akman-Demir G, Gokyigit A (1993) Spontaneous dissection of the extracranial vertebral artery with spinal subarachnoid haemorrhage in a patient with Behcet’s disease. Neuroradiology 35:352–354

    Article  PubMed  CAS  Google Scholar 

  5. Barnes CG, Yazici H (1999) Behcet’s syndrome. Rheumatology (Oxford) 38:1171–1174

    Article  CAS  Google Scholar 

  6. Biousse V, Ameri A, Bousser MG (1999) Isolated intracranial hypertension as the only sign of cerebral venous thrombosis. Neurology 53:1537–1542

    PubMed  CAS  Google Scholar 

  7. Bousser MG, Chiras J, Bories J, Castaigne P (1985) Cerebral venous thrombosis—a review of 38 cases. Stroke 16:199–213

    PubMed  CAS  Google Scholar 

  8. Bousser MG, Ferro JM (2007) Cerebral venous thrombosis: an update. Lancet Neurol 6:162–170

    Article  PubMed  CAS  Google Scholar 

  9. Bousser MG, Rougemont D, Youl BD, Wechsler B (1988) Neurological manifestations of Behcet’s disease. J Mal Vasc 13:231–234

    PubMed  CAS  Google Scholar 

  10. Breteau G, Mounier-Vehier F, Godefroy O, Gauvrit JY, Mackowiak-Cordoliani MA, Girot M, Bertheloot D, Henon H, Lucas C, Leclerc X, Fourrier F, Pruvo JP, Leys D (2003) Cerebral venous thrombosis 3-year clinical outcome in 55 consecutive patients. J Neurol 250:29–35

    Article  PubMed  CAS  Google Scholar 

  11. Coban O, Bahar S, Akman-Demir G, Tasci B, Yurdakul S, Yazici H, Serdaroglu P (1999) Masked assessment of MRI findings: is it possible to differentiate neuro-Behcet’s disease from other central nervous system diseases? (corrected). Neuroradiology 41:255–260

    Article  PubMed  CAS  Google Scholar 

  12. de Bruijn SF, Stam J (1999) Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis. Stroke 30:484–488

    PubMed  Google Scholar 

  13. Dentali F, Gianni M, Crowther MA, Ageno W (2006) Natural history of cerebral vein thrombosis: a systematic review. Blood 108:1129–1134

    Article  PubMed  CAS  Google Scholar 

  14. Ferro JM, Canhao P, Stam J, Bousser MG, Barinagarrementeria F (2004) Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 35:664–670

    Article  PubMed  Google Scholar 

  15. Ferro JM, Correia M, Pontes C, Baptista MV, Pita F (2001) Cerebral vein and dural sinus thrombosis in Portugal: 1980–1998. Cerebrovasc Dis 11:177–182

    Article  PubMed  CAS  Google Scholar 

  16. Ferro JM, Lopes MG, Rosas MJ, Fontes J (2005) Delay in hospital admission of patients with cerebral vein and dural sinus thrombosis. Cerebrovasc Dis 19:152–156

    Article  PubMed  Google Scholar 

  17. Hamuryudan V, Er T, Seyahi E, Akman C, Tuzun H, Fresko I, Yurdakul S, Numan F, Yazici H (2004) Pulmonary artery aneurysms in Behcet syndrome. Am J Med 117:867–870

    Article  PubMed  Google Scholar 

  18. Houman MH, Hamzaoui-B’Chir S, Ben Ghorbel I, Lamloum M, Ben Ahmed M, Abdelhak S, Miled M (2002) Neurologic manifestations of Behcet’s disease: analysis of a series of 27 patients. Rev Med Interne 23:592–606

    Article  PubMed  CAS  Google Scholar 

  19. Kidd D, Steuer A, Denman AM, Rudge P (1999) Neurological complications in Behcet’s syndrome. Brain 122(Pt 11):2183–2194

    Article  PubMed  Google Scholar 

  20. Krespi Y, Akman-Demir G, Poyraz M, Tugcu B, Coban O, Tuncay R, Serdaroglu P, Bahar S (2001) Cerebral vasculitis and ischaemic stroke in Behcet’s disease: report of one case and review of the literature. Eur J Neurol 8:719–722

    Article  PubMed  CAS  Google Scholar 

  21. Preter M, Tzourio C, Ameri A, Bousser MG (1996) Long-term prognosis in cerebral venous thrombosis. Follow-up of 77 patients. Stroke 27:243–246

    PubMed  CAS  Google Scholar 

  22. Serdaroglu P, Yazici H, Ozdemir C, Yurdakul S, Bahar S, Aktin E (1989) Neurologic involvement in Behcet’s syndrome. A prospective study. Arch Neurol 46:265–269

    PubMed  CAS  Google Scholar 

  23. Siva A, Fresko II (2000) Behcet’s disease. Curr Treat Options Neurol 2:435–448

    Article  PubMed  Google Scholar 

  24. Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, Kocer N, Yazici H (2001) Behcet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248:95–103

    Article  PubMed  CAS  Google Scholar 

  25. Stam J (2005) Thrombosis of the cerebral veins and sinuses. N Engl J Med 352:1791–1798

    Article  PubMed  CAS  Google Scholar 

  26. Stam J, De Bruijn SF, DeVeber G (2002) Anticoagulation for cerebral sinus thrombosis. Cochrane Database Syst Rev: CD002005

  27. Stephens MA (1974) EDF statistics for goodness of fit and some comparisons. J Am Stat Assoc 69:730–737

    Article  Google Scholar 

  28. Wechsler B, Piette JC, Conard J, Huong Du LT, Bletry O, Godeau P (1987) Deep venous thrombosis in Behcet’s disease. 106 localizations in a series of 177 patients. Presse Med 16:661–664

    PubMed  CAS  Google Scholar 

  29. Wechsler B, Vidailhet M, Piette JC, Bousser MG, Dell Isola B, Bletry O, Godeau P (1992) Cerebral venous thrombosis in Behcet’s disease: clinical study and long-term follow-up of 25 cases. Neurology 42:614–618

    PubMed  CAS  Google Scholar 

  30. Yazici H (2002) Behcet’s syndrome: where do we stand? Am J Med 112:75–76

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, Istanbul Medical School, Capa, 34293, Istanbul, Turkey

    N. Yesilot, S. Bahar, S. Yılmazer, M. Mutlu, M. Kurtuncu, R. Tuncay, O. Coban & G. Akman-Demir

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  1. N. Yesilot
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  2. S. Bahar
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  3. S. Yılmazer
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  4. M. Mutlu
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  5. M. Kurtuncu
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  6. R. Tuncay
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  7. O. Coban
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  8. G. Akman-Demir
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Correspondence to N. Yesilot.

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Yesilot, N., Bahar, S., Yılmazer, S. et al. Cerebral venous thrombosis in Behçet’s disease compared to those associated with other etiologies. J Neurol 256, 1134–1142 (2009). https://doi.org/10.1007/s00415-009-5088-4

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  • DOI: https://doi.org/10.1007/s00415-009-5088-4

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