Opinion statement
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•Clinical and imaging data suggest that Behçet’s disease (BD) can present with a variety of neurologic complications, which may be subclassified into two forms. One is attributable to small venous inflammatory disease with focal or multifocal central nervous system involvement and is seen in the majority of patients. It is designated Central Nervous System-Neuro-Behcet syndrome (CNS-NBS). The other form is due to cerebral venous sinus thrombosis and has limited symptoms and a better prognosis. It is very uncommon for these two types of involvement to occur in the same individual. It is very likely that the two major forms have different pathogeneses.
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•Patients with small vein inflammation should be considered for aggressive treatment. A substantial number of patients in this group will have a relapsing-remitting course; for some, this will evolve into a secondary progressive course. A few patients from this group will have progressive CNS dysfunction from the onset.
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•Acute attacks of CNS-NBS are treated with either oral prednisone (1 mg/kg/d up to 4 weeks or until improvement is observed) or high-dose intravenous methylprednisolone (IVMP), 1 g/d for 5 to 7 days. After either treatment, an oral tapering dose of glucocorticoids should be given over 2 to 3 months to avoid early relapses. Some patients may require the long-term use of low maintenance doses of glucocorticoids to prevent exacerbations.
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•Immunosuppressive agents (eg, azathioprine, cyclosporine A, cyclophosphamide, and chlorambucil), given either alone or in different combinations (eg, azathioprine with cyclosporine) for the long-term treatment of various systemic manifestations of BD, have not been shown to prevent the development of the neurologic complications of the disease, reduce its exacerbations, or stop its progression.
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•Immunomodulatory treatments such as interferon alfa and thalidomide have been shown to be effective in treating some of the systemic manifestations of BD, but there is no information on their effects on the development and progression of CNS-NBS.
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•Cerebral venous sinus thrombosis in BD is treated with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin given together with a short course of glucocorticoids. Evidence of benefit from this treatment, however, is weak.
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References and Recommended Reading
International Study Group for Behçet’s Disease: Criteria for diagnosis of Behçet’s disease. Lancet 1990, 335:1078–1080.
Sakane T, Takeno M, Suzuki N, Inaba G: Behçet’s disease. N Engl J Med 1999, 341:1284–1291. This recent article reviews current thoughts on the causation and pathophysiology of Behçet’s disease and outlines its clinical manifestations and their treatments.
Wechsler B, Vidailhet M, Bousser MG, et al.: Cerebral venous sinus thrombosis in Behçet’s disease: long term follow-up of 25 cases. Neurology 1992, 42:614–618. This article, in which a large series of patients with cerebral venous sinus thrombosis in Behçet’s disease is reported, is one of the earlier papers emphasizing that this form may represent a subtype of neurologic involvement seen in this disease.
Serdaroglu P: Behçet’s disease and the nervous system. J Neurol 1998, 245:197–205. This article presents an overview of Behçet’s syndrome and reviews Neuro-Behçet disease.
Akman-Demir G, Serdaroglu P, Tasçi B: Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain 1999, 122:2171–2181. This study, in which the clinical features, evoked potentials, cerebrospinal fluid and imaging studies, and prognostic factors of BD with neurologic involvement are evaluated retrospectively, is the largest case series reported of its kind.
Siva A, Altintas A: Neuro-Behçet Syndrome. In Intravenous Immunoglobulins in the Treatment of Neurological Disorders. Edited by Said G. London: Martin Dunitz Publishers; 2000: 115–126.
Kidd D, Steuer A, Denman AM, Rudge P: Neurological complications in Behçet’s syndrome. Brain 1999, 122:2183–2194. This article describes the clinical, imaging, laboratory, and neurophysiologic features and prognostic factors in Behçet’s disease with neurologic involvement in a case series of 50 patients.
Koçer N, Islak C, Siva A, et al.: CNS involvement in Neuro-Behçet’s syndrome: an MR study. Am J Neuroradiol 1999, 20:1015–1024. This article reports the imaging findings of CNS involvement in Behçet’s disease and emphasizes differential diagnosis in a large case series. The findings are also correlated with the current understanding of the pathophysiology of this form of neurologic involvement.
Hadfield MG, Aydin F, Lippman HR, Sanders KM: Neuro-Behçet’s disease. Clin Neuropathol 1997, 16:55–60.
O’Duffy JD, Robertson DM, Goldstein NP: Chlorambucil in the treatment of uveitis and meningoencephalitis of Behçet’s disease. Am J Med 1984, 76:75–84.
Schwartz RB, Bravo SM, Klufas RA, et al.: Cyclosporine neurotoxicity and its relation to hypertensive neuropathy: CT and MR findings in 16 cases. Am J Roentgenol 1995, 165:627–631. This article describes the clinical and radiologic findings in patients showing the neurotoxic effects of cyclosporine.
Kotake S, Higashi K, Yoshikawa K, et al.: Central nervous system symptoms in patients with Behçet disease receiving cyclosporine therapy. Ophthalmology 1999, 106:586–589. This article reports on the higher incidence of neurotoxicity seen with the use of cyclosporine in Behçet’s disease.
Akman-Demir G, Bahar S, Baykan-Kurt B, et al.: Intracranial hypertension in Behçet’s disease. Eur J Neurol 1996, 3:66–70.
O’Duffy JD, Goldstein NP: Neurologic involvement in seven patients with Behçet’s disease. Am J Med 1976, 61:170–178.
Bang D: Treatment of Behçet’s disease. Yonsei Med J 1997, 38(6):401–410. This article reviews the various agents and forms of treatment used in Behçet’s disease.
Kiziltas S, Imeryüz N, Gürcan T, et al.: Corticosteroid therapy augments gastroduodenal permeability to sucrose. Am J Gastroenterol 1998, 93:2420–2425.
Aktulga E, Altaç M, Müüftüoglu A, et al.: A double blind study of colchicine in Behçet’s disease. Haematologica 1980, 65(3):399–402.
Yurdakul S, Mat C, Ozyazgan Y, et al.: A double blind study of colchicine in Behçet’s syndrome (BS). Arthritis Rheum 1998, 41(suppl):S356.
Yazici H, Pazarli H, Barnes CG, et al.: A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med 1990, 322(5):281–285.
Hamuryudan V, Ozyazgan Y, Hizli N, et al.: Azathioprine in Behçet’s syndrome: effects on long-term prognosis. Arthritis Rheum 1997, 40(4):769–774.
Confavreux C, Saddier P, Grimaud J, et al.: Risk of cancer from azathioprine therapy in multiple sclerosis: a case control study. Neurology 1996, 46:1607–1612.
Ben Ezra D, Cohen E, Chajek T, et al.: Evaluation of conventional therapy versus cyclosporin A in Behçet’s syndrome. Transplant Proc 1988, 20 (3 suppl 4):136–143.
Ozyazgan Y, Yurdakul S, Yazici H, et al.: Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet’s syndrome: a single masked trial. Br J Ophthalmol 1992, 4:241–243.
Masuda K, Nakajima A, Urayama A, et al.: Double masked trial of cyclosporin versus colchicine and long term open study of cyclosporin in Behçet’s disease. Lancet 1989, 1:1093–1096.
Assad-Khalil SH: Low dose of cyclosporin in Behçet’s disease: follow-up controlled study with emphasis on extraocular manifestations and Neuro-Behçet’s disease. In Behçet’s Disease: Basic and Clinical Aspects. Edited by O’Duffy JD, Kökmen E. New York: Marcel Dekker; 1991:603–612.
Abdalla MA, Bahgat NE: Long lasting remission of Behçet’s disease after chlorambucil therapy. Br J Ophthalmol 1973, 57:706–711.
Zelenski JD, Capraro JA, Holden D, Calabrase H: Central nervous system vasculitis in Behçet’s syndrome: angiographic improvement after therapy with cytotoxic agents. Arthritis Rheum 1989, 32(2):2l7–220.
Matteson EL, O’Duffy JD: Treatment of Behçet’s disease with chlorambucil. In Behçet’s Disease: Basic and Clinical Aspects. Edited by O’Duffy JD, Kökmen E. New York: Marcel Dekker; 1991:575–580.
Tessler HH, Jennings T: High dose short term chlorambucil for intractable sympathetic ophthalmia and Behçet’s disease. Br J Ophthalmol 1990, 74:353–357.
O’Duffy JD, Calamia K, Cohen S, et al.: Interferon alfa treatment of Behçet’s disease. J Rheumatol 1998, 25:1938–1944.
Zouboulis CC, Orfanos CE: Treatment of Adamantiades-Behçet disease with systemic interferon alfa. Arch Dermatol 1998, 134:1010–1016.
Kotter I, Eckstein AK, Stubiger N, Zierhut M: Treatment of ocular symptoms of Behçet’s disease with interferon alfa-2a: a pilot study. Br J Ophthalmol 1998, 82(5):488–494.
Budak-Alpdogan T, Demirçay Z, Alpdogan O, et al.: Skin hyperreactivity of Behçet’s patients (pathergy reaction) is also positive in interferon alpha-treated chronic myeloid leukemia patients, indicating similarly altered neutrophil functions in both disorders. Br J Rheumatol 1998, 37(11):1148–1151.
Kavano T, Shigehira M, Uto H, et al.: Retinal complications during interferon therapy for chronic hepatitis C. Arch Ophthalmol 1996, 91:309–313.
Hamuryudan V, Mat C, Saip S, et al.: Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med 1998, 128(6):443–450.
Fain O, Huong Du LT, Wechsler B, et al.: Pulse cyclophosphamide in Behçet’s disease. In Behçet’s Disease: Basic and Clinical Aspects. Edited by O’Duffy JD, Kökmen E. New York: Marcel Dekker; 1991:569–573.
de Merieux P, Spitler LE, Paulus HE: Treatment of Behçet syndrome with levamisole. Arthritis Rheum 1981, 24:64–67.
Yasui K, Ohta K, Kabayashi M, et al.: Successful treatment of BD with pentoxifylline. Ann Intern Med 1996, 124:891–893.
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Siva, A., Fresko, I. Behçet’s disease. Curr Treat Options Neurol 2, 435–447 (2000). https://doi.org/10.1007/s11940-000-0042-x
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DOI: https://doi.org/10.1007/s11940-000-0042-x