Journal of Neurology

, Volume 258, Issue 5, pp 719–727

Cerebral venous thrombosis in Behçet’s disease: a systematic review

Review

Abstract

Behçet’s disease (BD) is a chronic inflammatory multisystem disorder which can involve the central nervous system (CNS). Cerebral venous thrombosis (CVT) is one of its major neurological manifestations. We aimed to review the epidemiologic and clinical features of CVT in patients with BD, as well as the available data on therapeutic interventions and prognosis. Systematic review of all observational studies of BD patients was done. Search strategy included electronic searches of MEDLINE (1966-August 2009). Occurrence of CVT in BD and Neuro-Behçet patients, occurrence of CVT as the inaugural manifestation of BD, clinical and neuro-imaging characteristics of CVT, prothrombotic evaluation, treatment options and prognosis were extracted. A meta-analysis of available results was performed when feasible. Twenty-three studies were included, with 290 cases of CVT in patients with BD. The incidence of CVT per 1,000 person-years was 3 (95% CI: 1–8), being higher in retrospective studies (3.2, 95% CI: 1–10) than in prospective studies (2.7, 95% CI: 1–13). Among patients with neurologic involvement, the incidence rate was 15.1/1,000 person-years. The onset was progressive in 77% of the patients. Intracranial hypertension syndrome was a frequent presentation of CVT in BD. The most frequent sites of occlusion were the superior sagittal and the transverse sinus. Most of the studies did not evaluate the prevalence of prothrombotic disorders. Treated CVT was associated with a good prognosis. CVT is a frequent neurological manifestation of BD. When treated, BD-associated CVT bears a good prognosis. There is insufficient information regarding the role of concomitant prothrombotic disorders and specific treatments.

Keywords

Cerebral venous thrombosis Behçet’s disease Neuro-Behçet syndrome 

References

  1. 1.
    Siva A, Altintas A, Saip S (2004) Behçet’s syndrome and the nervous system. Curr Opin Neurol 17:347–357PubMedCrossRefGoogle Scholar
  2. 2.
    International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080Google Scholar
  3. 3.
    International Study Group for Behçet’s disease (1992) Evaluation of diagnostic (classification) criteria in Behçet’s disease—towards internationally agreed criteria. Br J Rheumatol 31:299–308CrossRefGoogle Scholar
  4. 4.
    Siva A, Saip S (2009) The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol 256(4):513–529PubMedCrossRefGoogle Scholar
  5. 5.
    Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A, Dormont D, Amoura Z, Cacoub P, Piette JC (2009) Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum 61:518–526Google Scholar
  6. 6.
    Al-Araji A, Kidd D (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8:192–204PubMedCrossRefGoogle Scholar
  7. 7.
    Ascaso FJ, Rodriguez A, Cristóbal JA (2002) Cranial hypertension as first manifestation of Behcet’s disease. Doc Ophthalmol 105:291–299PubMedCrossRefGoogle Scholar
  8. 8.
    Gumà A, Aguilera C, Acebes J, Arruga J, Pons L (1998) Meningeal involvement in Behçet’s disease. MRI Neuroradiology 40:512–515CrossRefGoogle Scholar
  9. 9.
    Daif A, Awada A, Al-Rajeh S, Abduljabbar M, Al Tahan A, Obeid T, Malibary T (1995) Cerebral venous thrombosis in adults: a study of 40 cases from Saudi Arabia. Stroke 26:1193–1195PubMedGoogle Scholar
  10. 10.
    Bousser M, Ferro JM (2007) Cerebral venous thrombosis: an update. Lancet Neurol 6:162–170PubMedCrossRefGoogle Scholar
  11. 11.
    Ferro JM, Canhão 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 35:664–670PubMedCrossRefGoogle Scholar
  12. 12.
    Manchester HC (1959) Behçet’s syndrome complicated by intracranial thrombophlebitis. Proc R Soc Med 52:1039–1040Google Scholar
  13. 13.
    Pamir N, Kansu T, Erbengi A, Zileli T (1981) Papilledema in Behçet’s syndrome. Arch Neurol 38:643–645PubMedGoogle Scholar
  14. 14.
    Rougemont D, Bousser MG, Wechsler B, Bletry O, Castaigne P, Godeau P (1982) Manifestations neurologiques de la maladie de Behçet: vingt-quatre observations. Rev Neurol (Paris) 138:493–505Google Scholar
  15. 15.
    Serdaroglu P, Yazici H, Ozdemir C, Yurdakul S, Bahar S, Aktin E (1989) Neurologic involvement in Behçet’s syndrome: a prospective study. Arch Neurol 46:265–269PubMedGoogle Scholar
  16. 16.
    Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, Yurdakul S, Yazici H (2003) The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76CrossRefGoogle Scholar
  17. 17.
    Wechsler B, Piette JC, Conard J, Huong Du LT, Blétry O, Godeau P (1985) Cerebral venous thrombosis in Behçet’s disease. Mayo Clin Proc 60:891–892PubMedGoogle Scholar
  18. 18.
    Wechsler B, Piette JC, Conard J, Huong Du LT, Blétry O, Godeau P (1987) Les thromboses veineuses profundes dans la maladie de Behçet: 106 localizations sur une série de 177 malades. Presse Méd 16:661–664PubMedGoogle Scholar
  19. 19.
    Banna M, El-Ramahi K (1991) Neurologic involvement in Behçet disease: imaging findings in 16 patients. AJNR 12:791–796PubMedGoogle Scholar
  20. 20.
    Lannuzel D, Lamaury I, Charpentier D, Caparros-Lefebvre D (2002) Neurological manifestations of Behçet’s disease in a Caribbean population: clinical and imaging findings. J Neurol 249:410–418PubMedCrossRefGoogle Scholar
  21. 21.
    Akman-Demir G, Baykan-Kurt B, Serdaroglu P, Gurvit H, Yardakul S, Yazici H, Bahar S, Aktin E (1996) Seven-year follow-up of neurologic involvement in Behçet syndrome. Arch Neurol 53:691–694PubMedGoogle Scholar
  22. 22.
    Tunc R, Saip S, Siva A, Yazici H (2004) Cerebral venous thrombosis is associated with major vessel disease in Behçet’s syndrome. Ann Rheum Dis 63:1693–1694PubMedCrossRefGoogle Scholar
  23. 23.
    Akman-Demir G, Serdaroglu P, Tasçi B, The Neuro-Behçet Study-Group (1999) Clinical patterns of neurologic involvement in Behçet-disease: evaluation of 200 patients. Brain 122:2171–2181PubMedCrossRefGoogle Scholar
  24. 24.
    Wechsler B, Vidailhet M, Piette JC, Bousser MG, Dell Isola B, Blétry O, Godeau P (1992) Cerebral venous thrombosis in Behçet’s disease: clinical study and long-term follow-up of 25 cases. Neurology 42:614–618PubMedGoogle Scholar
  25. 25.
    Yesilot N, Bahar S, Yilmazer S, Mutlu M, Kurtuncu M, Tuncay R, Coban O, Akman-Demir G (2009) Cerebral venous thrombosis in Behçet’s disease compared to those associated with other etiologies. J Neurol 256:1134–1142PubMedCrossRefGoogle Scholar
  26. 26.
    Wechsler B, Dell’Isola B, Vidailhet M, Dormont D, Piette JC, Blétr O, Godeau P (1993) MRI in 31 patients with Behçet’s disease and neurologic involvement: prospective study with clinical correlation. J Neurol Neurosurg Psychiatry 56:793–798PubMedCrossRefGoogle Scholar
  27. 27.
    Mossadeq R, Karouache A, Bouraza A, Ouhabi H, Reda R, Boutaleb N, Jidane A (2004) Thrombose de la veine basilaire de Rosenthal et angio-Behçet cérébral: étude de 12 cas. Rev Neurol (Paris) 160:935–938Google Scholar
  28. 28.
    Houman MH, Neffati H, Braham A, Harzallah O, Khanfir M, Miled M, Hamzaoui K (2007) Behçet’s disease in Tunisia. Demographic, clinical and genetic aspects in 260 patients. Clin Exp Rheumatol 25(Suppl 45):S58–S64PubMedGoogle Scholar
  29. 29.
    Joseph F, Scolding N (2007) Neuro-Behçet’s disease in Caucasians: a study of 22 patients. Eur J Neurol 14:174–180Google Scholar
  30. 30.
    Al-Araji A, Sharquie K, Al-Rawi Z (2003) Prevalence and patterns of neurological involvement in Behcet’s disease: a prospective study from Iraq. J Neurol Neurosurg Psychiatry 74:608–613PubMedCrossRefGoogle Scholar
  31. 31.
    Agnelli G, Verso M (2008) Epidemiology of cerebral vein and sinus thrombosis. Front Neurol Neurosci 23:16–22PubMedGoogle Scholar
  32. 32.
    Stam J (2005) Thrombosis of the cerebral veins and sinuses. N Engl J Med 352:1791–1798PubMedCrossRefGoogle Scholar
  33. 33.
    Bouchez B, Arnott G, Caron JC, Guerouaou D, Clarisse G, Blondel M, Hannebique G (1984) Thromboses veineuses cérébrales avec atteinte du sinus caverneux primière expression d’une maladie de Behçet? Rev Otoneuroophtalmol 56:447–453PubMedGoogle Scholar
  34. 34.
    Saltik S, Saip S, Kocer N, Siva A, Alçinkaya C (2004) MRI findings in pediatric Neuro-Behçet’s disease. Neuropediatrics 35:190–193PubMedCrossRefGoogle Scholar
  35. 35.
    Alper G, Yılmaz Y, Ekinci G, Kose O (2001) Cerebral vein thrombosis in Behçet’s disease. Pediatr Neurol 25:332–335PubMedCrossRefGoogle Scholar
  36. 36.
    Can E, Kara B, Somer A, Keser M, Salman N, Yalcın I (2006) Neuro-Behcet disease presenting as secondary pseudotumor syndrome: case report. Eur J Paediatr Neurol 10:97–99PubMedCrossRefGoogle Scholar
  37. 37.
    Budin C, Ranchin B, Glastre C, Fouilhoux A, Canterino I, David L (2002) Atteinte neurologique révélatrice d’une maladie de Behçet: deux observations pédiatriques. Arch Pediatr 9:1160–1162PubMedCrossRefGoogle Scholar
  38. 38.
    Rahil AI, Errayes M, Salem KM (2009) Cerebral venous thrombosis as the initial presentation of Behcet’s disease. Chang Gung Med J 32:220–223PubMedGoogle Scholar
  39. 39.
    Humberclaude V, Vallée L, Sukno S, Vamecq J, Dhellemmes S, Pruvo JP, Nuyts JP (1993) Thrombose veineuse cérébrale révélant une maladie de Behçet. Arch Fr Pediatr 50:603–605PubMedGoogle Scholar
  40. 40.
    Bank I, Weart C (1984) Dural sinus thrombosis in Behçet’s disease. Arthritis Rheum 27:816–818PubMedCrossRefGoogle Scholar
  41. 41.
    Stern JM, Kesler SM (1989) Raised intracranial pressure in a 16-year-old boy: report of a case of Behçet’s disease. S Afr Med J 75:243–244PubMedGoogle Scholar
  42. 42.
    Camoin-Jau J, Kone-Paut I, Chabrol B, Sampol J, Dignat-George F (2000) Circulating endothelial cells in Behçet’s disease with cerebral thrombophlebitis. Thromb Haemost 83:631–632PubMedGoogle Scholar
  43. 43.
    Turk U, Aribas E, Aydin N (1994) Cerebral venous thrombosis in Behçet’s disease. Tokai Exp Clin Med 19:19–22Google Scholar
  44. 44.
    Harper CM, O’Neill B, O’Duffy JD, Forbes GS (1985) Intracranial hypertension in Behçet’s disease: demonstration of sinus occlusion with use of cerebral venous angiography. Mayo Clin Proc 60:419–422PubMedGoogle Scholar
  45. 45.
    Swerdlow RH, Hanna GR (1996) Behçet’s disease: presentation with sagittal sinus thrombosis diagnosed noninvasively. Headache 3:115–118CrossRefGoogle Scholar
  46. 46.
    Bousser MG, Bletry, Launay M, Portier E, Guillard A, Castaigne P (1980) Thromboses veineuses cérébrales au cours de la maladie de Behçet. Rev Neurol (Paris) 136:753–762Google Scholar
  47. 47.
    El-Ramahi KM, Al-Kawi MZ (1991) Papilloedema in Behçet’s disease: value of MRI in diagnosis of dural sinus thrombosis. J Neurol Neurosurg Psychiatry 54:826–829PubMedCrossRefGoogle Scholar
  48. 48.
    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(7):592–606PubMedCrossRefGoogle Scholar
  49. 49.
    Kidd D, Steuer A, Denman AM, Rudge P (1999) Neurological complications in Behçet’s syndrome. Brain 122:2183–2194PubMedCrossRefGoogle Scholar
  50. 50.
    Borhani-Haghighi A, Samangooie S, Ashjazadeh N, Nikseresht A, Shariat A, Yousefipour G, Safari A (2006) Neurological manifestations of Behçet’s disease. Saudi Med J 27:1542–1546PubMedGoogle Scholar
  51. 51.
    Farah S, Al-Shubaili A, Montaser A, Hussein JM, Malaviya AN, Mukhtar M, Al-Shayeb A, Khuraibet AJ, Khan R, Trontelj JV (1998) Behçet’s syndrome: a report of 41 patients with emphasis on neurological manifestations. J Neurol Neurosurg Psychiatry 64:382–384PubMedCrossRefGoogle Scholar
  52. 52.
    Benamour S, Naji T, Alaoui F, El Kabli H, El Aidouni S (2006) Manifestations neurologiques de la maladie de Behçet. Rev Neurol (Paris) 162:1084–1090Google Scholar
  53. 53.
    Tohmé A, Koussa S, Haddad-Zébouni S, El-Rassi B, Ghayad E (2009) Étude de 22 observations de Neuro-Behçet dans une série de 170 maladies de Behçet. Press Med 38:701–709CrossRefGoogle Scholar
  54. 54.
    Mnif N, Rajhi H, Mlika N, Kechaou S, Abdallah Nejmeddine B, Hamza R (2006) MRI findings in neuro-Behçet’s disease. J Neuroradiol 33:250–254PubMedCrossRefGoogle Scholar
  55. 55.
    Ramos C, Sá G, Tedim Cruz V, Lopes A, Xavier J, Cruz R (2006) Neuro-Behçet: MR study of a group of patients. Acta Med Port 19:494–498PubMedGoogle Scholar
  56. 56.
    Tohmé A, Haddad F, Ghayad E (1997) Manifestations neurologiques de la maladie de Behçet: seize observations dans une cohort de 110 malades. Ann Med Interne 148:118–124Google Scholar
  57. 57.
    Al-Fahad SA, Al-Araji AH (1999) Neuro-Behcet’s disease in Iraq: a study of 40 patients. J Neurol Sci 170:105–111PubMedCrossRefGoogle Scholar
  58. 58.
    Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, Koçer N, Yazici H (2001) Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248:95–103PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  1. 1.Department of Neurosciences (Neurology), Hospital de Santa MariaUniversity of LisboaLisbonPortugal

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