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• W2033905623 abstract "L’atteinte neurologique est une des manifestations cliniques les plus graves au cours de la maladie de Behçet (MB). Le but de cette étude a été d’analyser le profil clinique et évolutif d’une série de 154 cas de neuro-Behçet (NB), et de les comparer aux 925 patients atteints de MB colligés durant la même période, et aux données de la littérature. Il s’agissait d’une étude rétrospective portant sur 154 cas de NB. Tous les patients répondaient aux critères internationaux d’étude sur la MB. Quatre vingt treize cas de céphalées isolées ont été exclus de ce travail. Le NB a été retrouvé dans 16,6 p. 100 de l’ensemble des cas de MB. Le sex-ratio homme/femme était de 4,3. L’âge moyen de début du NB était de 29,8 ans. Quatre vingt quatorze patients (61 p. 100) avaient une atteinte parenchymateuse. Vingt-sept patients (17,5 p. 100) présentaient une atteinte non parenchymateuse : hypertension intracrânienne isolée (24 cas), atteinte des artères à destinée cérébrale (3 cas). Une atteinte mixte a été observée dans 9 cas. D’autres aspects cliniques ont été rapportés : méningite isolée (15 cas), atteinte neurologique périphérique (4 cas), atteinte isolée des nerfs crâniens (8 cas). L’atteinte parenchymateuse était la plus grave avec 6 décès parmi les 7 observés dans le NB. Le NB survient beaucoup plus fréquemment chez l’homme. Son pronostic reste sévère et particulièrement dans la forme parenchymateuse. Neurological involvement is well described in Behçet's Disease (BD), with variable prevalence of 5.3 to 30p.cent. The purpose of this retrospective study was to analyze the clinical patterns of neuro-Behçet (NB) and to compare them with different clinical features of 925 BD registered in the same period and the literature. All patients of NB fulfilled the International Study Group Criteria for the diagnosis of BD. 93 patients with headache, without other neurological symptoms were excluded. The findings were supported by cerebrospinal fluid, computed tomography scan, magnetic resonance imaging and angiography. NB was present in 16.64p.cent of BD. A sex ratio male/female: 4.31. The mean age of patients with NB was 31.76 years. The average age of onset of NB was 29.83. The mean duration of the NB disease was 3.81 years. The findings were categorised in 2 main types: parenchymal and non-parenchymal involvement. Some patients had features of both types (mixed patterns). 94 patients (61.03p.cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, pseudobulbar syndrome and cerebellar signs. 27 patients (17.53p.cent) without parenchymal CNS which were divided into: intracranial hypertension in 24 patients (15.58p.cent) presenting headache, vomiting and bilateral papilloedema; cerebro-arterial involvement in 3 cases (one of them had cerebrovascular aneurysms). Mixed patterns were observed in 9 cases. Erythema nodosum and vascular involvement were more frequent in intracranial hypertension than in BD. Other clinical features were reported: pure meningeal pattern in 15 cases, pure peripheral nervous system involvement in 4 cases, isolated cranial nerves in 8 cases, chorea in one patient and pseudotumor of cervical medulla in other patient. 6 cases of juvenile BD and one case of familial Behçet were observed. 133 patients were treated (86.36p.cent). Mean duration of treatment was 7.46 month. We used corticosteroids, immunosuppressive agents (cyclophosphamid, azathioprine, chlorambucil) and anticoagulant in intracranial hypertension. The course of disease was good in only 54.13p.cent of cases, and was bad in 18.79p.cent. NB occurred frequently in men and is more serious especially in parenchymal CNS involvement: 7 deaths (6 from parenchymal CNS)." @default.
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• W2033905623 date "2006-11-01" @default.
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• W2033905623 title "Manifestations neurologiques de la maladie de Behçet" @default.
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• W2033905623 doi "https://doi.org/10.1016/s0035-3787(06)75121-9" @default.
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