Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations

Affiliations

01 March 1998

-

doi: 10.1136/jnnp.64.3.382


Abstract

Forty one patients with the clinical diagnosis of Behçet's syndrome from two teaching hospitals in Kuwait were studied. There were 34 male and seven female patients. Age at presentation ranged from 14 to 48 years. Neurological manifestations were present in 24 patients. Eleven patients showed evidence of increased intracranial pressure, and 10 of these had radiologically confirmed dural sinus thrombosis. Five patients presented with a meningoencephalitic or meningomyelitic picture, three with a stroke-like picture, and three with primarily brain stem signs. One patient developed trigeminal neuritis, and five patients exhibited (along with other features) variable degrees of psychological manifestations. All patients with neurological involvement were treated with steroids, and some also had courses of other immunosuppressant drugs and colchicine. The disease took a relatively benign course, except those patients with meningoencephalitic and meningomyelitic presentation, one of whom died from the disease. Those treated early had a better prognosis. The incidence of dural sinus thrombosis in this series of patients is unusually high. In most patients, the course of the disease was more favourable than reported in the literature. This may be attributed to early and aggressive treatment.


Similar articles

[Neurological manifestations of Behcet's disease: 22 cases among 170 patients].

Tohmé A, Koussa S, Haddad-Zébouni S, El-Rassi B, Ghayad E.Presse Med. 2009 May;38(5):701-9. doi: 10.1016/j.lpm.2008.04.015. Epub 2008 Dec 4.PMID: 19062244 French.

Neurological presentation of neuro-Behçet's syndrome: clinical categories.

Shakir RA, Sulaiman K, Kahn RA, Rudwan M.Eur Neurol. 1990;30(5):249-53. doi: 10.1159/000117356.PMID: 2269312

Neurologic onset of Behçet's disease: a diagnostic enigma in childhood.

Koné-Paut I, Chabrol B, Riss JM, Mancini J, Raybaud C, Garnier JM.J Child Neurol. 1997 Jun;12(4):237-41. doi: 10.1177/088307389701200402.PMID: 9203064

[Cerebral sinus vein thrombosis in Behçet's disease].

Borer H, Rüttimann S, Kätterer C.Schweiz Med Wochenschr. 1991 May 25;121(21):788-92.PMID: 2057745 Review. German.

[Neurological manifestations of Behçet's disease].

Bousser MG, Rougemont D, Youl BD, Wechsler B.J Mal Vasc. 1988;13(3):231-4.PMID: 3049880 Review. French.


Cited by

Increased T-bet/GATA-3 and ROR-γt /Foxp3 Ratios in Cerebrospinal Fluid as Potential Criteria for Definite Neuro-Behçet's Disease.

Belghith M, Maghrebi O, Cherif A, Bahrini K, Saied Z, Belal S, Sassi SB, Barbouche MR, Kchaou M.J Clin Med. 2022 Jul 29;11(15):4415. doi: 10.3390/jcm11154415.PMID: 35956031 Free PMC article.

Neuro-Behcet's syndrome: Case report and literature review.

Peine B, Figueroa C, Robinette N.Radiol Case Rep. 2022 Jun 21;17(9):3064-3070. doi: 10.1016/j.radcr.2022.05.070. eCollection 2022 Sep.PMID: 35769120 Free PMC article.

Variant in ERAP1 promoter region is associated with low expression in a patient with a Behçet-like MHC-I-opathy.

Dimopoulou C, Lundgren JD, Sundal J, Ullum H, Aukrust P, Nielsen FC, Marvig RL.J Hum Genet. 2020 Mar;65(3):325-335. doi: 10.1038/s10038-019-0709-y. Epub 2019 Dec 23.PMID: 31873220

The Clinical Features and Risk Factors of Parenchymal Neuro-Behcet's Disease.

Yan D, Liu J, Zhang Y, Yuan W, Xu Y, Shi J, Li C, Wang Y, Peng L, Yang Y, Zhou J, Wu D, Liu Z, Zeng X, Zhang F, Zheng W, Zhao Y.J Immunol Res. 2019 Sep 12;2019:7371458. doi: 10.1155/2019/7371458. eCollection 2019.PMID: 31612152 Free PMC article.

Clinical and Radiographic Characteristics of Neuro-Behçet's Disease in South Korea.

Kim SW, Kim TG, Oh J, Kim DY, Choi YC, Kim SM, Shin HY, Bang D.J Clin Neurol. 2019 Oct;15(4):429-437. doi: 10.3988/jcn.2019.15.4.429.PMID: 31591829 Free PMC article.


KMEL References