title: Characteristics of neurological manifestations of Behçet's disease: a retrospective monocentric study in Tunisia.
abstract: OBJECTIVE
  The aim of the present study was to analyze demographic, clinical and genetic features of Behçet's disease patients with neurological involvement through a monocentric study of a homogenous group of hospitalized patients observed in the same department and to compare them with those of other ethnic and geographic groups.
  
  
  METHODS
  Four hundred and thirty Behçet's disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçet's disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations.
  
  
  RESULTS
  Neurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçet's disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1-19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis.
  
  
  CONCLUSIONS
  Clinical and epidemiological features of NBD are various. In our Tunisian cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.
authors.affiliation: Department of Internal Medicine, La Rabta University Hospital, Faculty of Medicine of Tunis, university el Manar, 1007 Tunis, Tunisia. Electronic address: houman_habib@yahoo.fr.
authors.forename: Mohamed-Habib
authors.initials: MH
authors.lastname: Houman
authors.forename: Syrine
authors.initials: S
authors.lastname: Bellakhal
authors.forename: Thouraya
authors.initials: T
authors.lastname: Ben Salem
authors.forename: Amira
authors.initials: A
authors.lastname: Hamzaoui
authors.forename: Amel
authors.initials: A
authors.lastname: Braham
authors.forename: Mounir
authors.initials: M
authors.lastname: Lamloum
authors.forename: Smiti-Khanfir
authors.initials: SK
authors.lastname: Monia
authors.forename: Imed
authors.initials: I
authors.lastname: Ben Ghorbel
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D001528:Behcet Syndrome / Q000150:complications* / Q000503:physiopathology*; D002493:Central Nervous System Diseases / Q000209:etiology; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology* / Q000503:physiopathology* / Q000523:psychology; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012737:Sex Factors; D014416:Tunisia / Q000453:epidemiology; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: Behçet's disease; Magnetic resonance imaging; Neuro-Behçet; Neurological involvement; Prognosis