Abstract
Behçet’s syndrome (BS) is a multisystem disease of unknown etiology, characterized by chronic relapsing cardinal symptoms of orogenital ulcers, uveitis, and different skin lesions. Its major pathologic feature is vasculitis. Neuro-Behçet’s syndrome (NBS) is defined as a constellation of neurologic manifestations with characteristic neuropathologic findings, usually confirmed by ancillary investigations, in patients who meet the diagnostic criteria for BS. Neurologic manifestations of the syndrome are more common in male patients and have been reported to occur in anywhere from 5% to 50% of BS patients, depending on their geographical region. NBS primarily affects the central nervous system and includes parenchymal and nonparenchymal involvement. Peripheral neuropathy and myopathy are rare. Immunosuppression is widely used for treatment.
Similar content being viewed by others
References and Recommended Reading
Barnes CG: Treatment of Behcet’s syndrome. Rheumatology 2006, 45:245–247. Article reviews general aspects and therapy of Behçet’s syndrome well.
Uber rezidivierende aphthose durch ein virus verursachte Behçet H. geschwure am mund, am auge und an den genitalien [in German]. Dermatol Wochenschr 1937, 105:1152–1157.
Tunc R, Keyman E, Melikoglu M, et al.: Target organ associations in Turkish patients with Behcet’s disease: a cross sectional study by exploratory factor analysis. J Rheumatol 2002, 29:2393–2396.
Yazici H: Behcet’s syndrome: Where do we stand? Am J Med 2002, 112:75–76. An editorial from an expert in the area of Behçet’s syndrome.
Diri E, Mat C, Hamuryudan V, et al.: Papulopustular skin lesions are seen more frequently in patients with Behcet’s syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis 2001, 60:1074–1076.
Sakane T, Takeno M, Suzuki N, Inaba G: Behcet’s disease. N Engl J Med 1999, 341:1284–1291.
Kaklamani VG, Vaiopoulos G, Kaklamanis PG: Behcet’s disease. Semin Arthritis Rheum 1998, 27:197–217.
Poon W, Verity DH, Larkin GL, et al.: Behcet’s disease in patients of west African and Afro-Caribbean origin. Br J Ophthalmol 2003, 87:876–878.
Yurdakul S, Gunaydin I, Tuzun Y, et al.: The prevalence of Behcet’s syndrome in a rural area in northern Turkey. J Rheumatol 1988, 15:820–822.
Nakae K, Masaki F, Hashimoto T, et al.: Recent epidemiological features of Behçet’s disease in Japan. In: Behçet’s disease. Edited by Wechsler B, Godeau P. Amsterdam: Excerpta Medica; 1993:145–151.
Yazici H, Tuzun Y, Pararli H, et al.: Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behcet’s syndrome. Ann Rheum Dis 1984, 43:783–789.
Fietta P: Behcet’s disease: familial clustering and immunogenetics. Clin Exp Rheumatol 2005, 23(Suppl 38):S96-S105. This very comprehensive and detailed article reviews the immunogenetics of Behçet’s syndrome.
Yazici H, Chamberlain MA, Tuzun Y, et al.: A comparative study of the pathergy reaction among Turkish and British patients with Behcet’s disease. Ann Rheum Dis 1984, 43:74–75.
Bonfioli AA, Orefice F: Behcet’s disease. Semin Ophthalmol 2005, 20:199–206.
Atzeni F, Sarzi-Puttini P, Doria A, et al.: Behcet’s disease and cardiovascular involvement. Lupus 2005, 14:723–726. Article contains an in-depth review of the cardiovascular involvement in Behçet’s syndrome, as well as the authors’ own related clinical experience.
Melikoglu M, Altiparmak MR, Fresko I, et al.: A reappraisal of amyloidosis in Behcet’s syndrome. Rheumatology 2001, 40:212–215.
International Study Group for Behcet’s Disease: Criteria for diagnosis of Behcet’s disease. Lancet 1990, 335:1078-1080.
Borhani Haghighi A, Pourmand R, Nikseresht AR: Neuro-Behcet disease: a review. Neurologist 2005, 11:80–89. The authors provide a comprehensive overview of neuro-Behçet’s syndrome.
Chin RL, Latov N: Central nervous system manifestations of rheumatologic diseases. Curr Opin Rheumatol 2005, 17:91–99. This paper has a good discussion of the differential diagnosis of rheumatic disorders causing central nervous system manifestations.
Serdaroglu P, Yazici H, Ozdemir C, et al.: Neurologic involvement in Behcet’s syndrome: a prospective study. Arch Neurol 1989, 46:265–269.
Akman-Demir G, Serdaroglu P, Tasci B: Clinical patterns of neurological involvement in Behcet’s disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain 1999, 122:2171–2182. This excellent report describes in detail the neurological manifestations in a large population of patients with Behçet’s. Prognostic factors are also discussed.
Lakhanpal S, Tani K, Lie JT, et al.: Pathologic features of Behcet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol 1985, 16:790–795.
Kone-Paut I, Gorchakoff-Molinas A, Weschler B, Touitou I: Paediatric Behcet’s disease in France [letter]. Ann Rheum Dis 2002, 61:655–656.
Siva A, Kantarci OH, Saip S, et al.: Behcet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 2001, 248:95–103. This is a well-designed study describing the frequencies of the different neurologic manifestations of Behçet’s syndrome.
Mirsattari SM, McGinn GJ, Halliday WC: Neuro-Behcet disease with predominant involvement of the brainstem. Neurology 2004, 63:382–384.
Kidd D, Steuer A, Denman AM, Rudge P: Neurological complications in Behcet’s syndrome. Brain 1999, 122:2183–2194.
Davis LE, Hodgin UG, Kornfeld M: Recurrent meningoencephalitis with recovery from Behcet’s disease. West J Med 1986, 145:238–239.
Aykutlu E, Baykan B, Akman-Demir G, et al.: Headache in Behcet’s disease. Cephalalgia 2006, 26:180–186.
Wechsler B, Vidailhet M, Piette JC, et al.: Cerebral venous thrombosis in Behcet’s disease: clinical study and long-term follow-up of 25 cases. Neurology 1992, 42:614–618.
Ho CL, Deruytter MJ: Manifestations of Neuro-Behcet’s disease: report of two cases and review of the literature. Clin Neurol Neurosurg 2005, 107:310–314.
Zelenski JD, Capraro JA, Holden D, Calabrese LH: Central nervous system vasculitis in Behcet’s syndrome: angiographic improvement after therapy with cytotoxic agents. Arthritis Rheum 1989, 32:217–220.
Dong ZH, Fu WG, Guo DQ, et al.: Endovascular repair for a huge vertebral artery pseudoaneurysm caused by Behcet’s disease. Chin Med J 2006, 119:435–437.
Yazici H, Yurdakul S, Hamuryudan V, Fresko I: Behçet’s syndrome. In Rheumatology. Edited by Hochberg MC, Silman AJ, Smolen JS, et al.: Madrid, Spain: Mosby; 2003:1665–1669.
Reske D, Petereit HF, Heiss WD: Difficulties in the differentiation of chronic inflammatory diseases of the central nervous system-value of cerebrospinal fluid analysis and immunological abnormalities in the diagnosis. Acta Neurol Scand 2005, 112:207–213. Article describes a good study of the value of cerebrospinal fluid analysis in the differential diagnosis of immunologic conditions affecting the central nervous system.
Sharief MB, Hentges R, Thomas E: Significance of CSF immunoglobulins in monitoring neurologic disease activity in Behcet’s disease. Neurology 1991, 41:1398–1401.
Saruhan-Direskeneli G, Yentur SP, Akman-Demir G, et al.:Cytokines and chemokines in neuro-Behcet’s disease compared to multiple sclerosis and other neurological diseases. J Neuroimmunol 2003, 145:127–134.
Akman-Demir G, Bahar S, Coban O, et al.: Cranial MRI in Behcet’s disease: 134 examinations of 98 patients. Neuroradiology 2003, 45:851–859. Article reports in detail the cranial MRI findings of neuro-Behçet’s syndrome.
Erdem H, Dinc A, Pay S, et al.: A neuro-Behcet’s case complicated with intracranial hypertension successfully treated by a lumboperitoneal shunt. Joint Bone Spine 2006, 73:200–201.
Motomura S, Tabira T, Kuroiwa Y: A clinical comparative study of multiple sclerosis and neuro-Behcet’s syndrome. J Neurol Neurosurg Psychiatry 1980, 43:210–213.
McLean BN, Miller D, Thompson EJ: Oligoclonal banding of IgG in CSF, blood-brain barrier function, and MRI findings in patients with sarcoidosis, systemic lupus erythematosus, and Behcet’s disease involving the nervous system. J Neurol Neurosurg Psychiatry 1995, 58:548–554.
Diri E, Espinoza CG, Espinoza LR: Spinal cord granulomatous vasculitis: an unusual clinical presentation of sarcoidosis. J Rheumatol 1999, 26:1408–1410.
Siva A, Kantarcy OH, Saip S, et al.: Behcet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 2001, 248:95–103.
Siva A, Fresko I I: Behcet’s disease. Curr Treat Options Neurol 2000, 2:435–448.
O’Duffy JD, Robertson DM, Goldstein NP: Chlorambucil in the treatment of uveitis and meningoencephalitis of Behcet’s disease. Am J Med 1984, 76:75–84.
Ranselaar CG, Boone RM, Kluin-Nelemans HC: Thalidomide in the treatment of neuro-Behcet’s syndrome. Br J Dermatol 1986, 115:367–370.
Calguneri M, Ozturk MA, Ertenli I, et al.: Effects of interferon alpha treatment on the clinical course of refractory Behcet’s disease: an open study. Ann Rheum Dis 2003, 62:492–493.
Sarwar H, McGrath H, Jr, Espinoza LR: Successful treatment of long-standing neuro-Behcet’s disease with infliximab. J Rheumatol 2005, 32:181–183.
Ribi C, Sztajzel R, Delavelle J, Chizzolini C: Efficacy of TNF alpha blockade in cyclophosphamide resistant neuro-Behcet disease. J Neurol Neurosurg Psychiatry 2005, 76:1733–1735.
Wechsler B, Gerber S, Vidailhet M, Dormont D: Neurologic manifestations of Behcet’s disease [in French]. Ann Med Interne 1999, 150:555–561.
Hirohata S, Suda H, Hashimoto T: Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet’s disease. J Neurol Sci 1998, 159:181–185.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Diri, E., Espinoza, L.R. Neuro-behçet’s syndrome: Differential diagnosis and management. Curr Rheumatol Rep 8, 317–322 (2006). https://doi.org/10.1007/s11926-006-0016-4
Issue Date:
DOI: https://doi.org/10.1007/s11926-006-0016-4