- Neuro-Behçet’s disease has protean manifestations, and a high degree of suspicion is needed for prompt diagnosis.
- Headache is a common symptom in Behçet’s disease and often a presenting symptom of neuro-Behçet’s disease.
- When interviewing a patient with Behçet’s disease and new-onset headache, reports of morning headache, a positional component, worsening with Valsalva and refractoriness to therapy should prompt an evaluation for CVST.
- Consider a workup for a hypercoagulable state in patients with Behçet’s disease and CVST.
- Treat with high-dose steroids and immunosuppression, and consider anticoagulation, if not contraindicated, based on the severity of the thrombotic event.
A multidisciplinary approach, including neurology, vascular medicine, hematology and rheumatology, is key in therapeutic decision making. Current guidelines seem flexible and based on expert opinion.
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Explore This IssueDecember 2018
Javaneh Lyons, MD, MSc, is a second-year fellow in adult rheumatology at the University of Vermont Medical Center, Burlington.
Alana Nevares, MD, is an assistant professor in the Division of Rheumatology and Clinical Immunology, Robert Larner, MD, College of Medicine, at the University of Vermont Medical Center, Burlington.
The authors extend special thanks to Scott Legunn, MD, and Maria Sayeed, MD, who were the treating rheumatologists at the time of disease onset and established a prompt diagnosis.
- Akman-Demir G, Saip S, Siva A. Behçet’s disease. Curr Treat Options Neurol. 2011 Jun;13(3):290–310.
- Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1–11.
- Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease. Lancet. 1990 May 5;335(8697):1078–1080.
- Davatchi F, Assaad-Khalil S, Calamia KT, et al for the International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). The International Criteria for Behcet’s Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014 Mar;28(3):338–347.
- Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703–1723.
- Al-Araji A, Kidd DP. Neuro-Behçet’s disease: Epidemiology, clinical characteristics, and management. Lancet Neurol. 2009 Feb;8(2):192–204.
- Saip S, Siva A, Altintas A, et al. Headache in Behçet’s syndrome. Headache. 2005 Jul–Aug;45(7):911–919.
- Aguiar de Sousa D, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet’s disease: A systematic review. J Neurol. 2011 May;258(5):719–727.
- Luo Y, Tian X, Wang X. Diagnosis and treatment of cerebral venous thrombosis: A review. Front Aging Neurosci. 2018 Jan 30;10:2.
- Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008 Dec;67(12):1656–1662.
- Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018 Jun;77(6):808–818.
- Saadoun D, Wechsler B, Resche-Rigon M, et al. Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum. 2009 Apr;61(4):518–526.
- Shi J, Huang X, Li G, et al. Cerebral venous sinus thrombosis in Behçet’s disease: A retrospective case-control study. Clin Rheumatol. 2018 Jan;37(1):51–57.
- Desbois AC, Wechsler B, Resche-Rigon M, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet’s disease. Arthritis Rheum. 2012 Aug;64(8):2753–2760.
- Tayer-Shifman OE, Seyahi E, Nowatzky J, Ben-Chetrit E. Major vessel thrombosis in Behçet’s disease: The dilemma of anticoagulant therapy—the approach of rheumatologists from different countries. Clin Exp Rheumatol. 2012 Sep–Oct;30(5):735–740.