title: Characteristics of Neuro-Behçet's Disease in a Case-Series from a Single Centre in Northern Portugal.
abstract: BACKGROUND
  Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology that may affect the CNS - Neuro-Behçet (NB). Our aim was to evaluate the frequency of neurological involvement and characterize a cohort of our NB patients.
  
  
  METHODS
  We retrospectively revised the clinical, laboratory and imaging data of a cohort of BD patients, followed in our hospital outpatient clinic.
  
  
  RESULTS
  We identified 138 BD patients. Twenty-five out of 138 had NB (15 female). Four patients presented with neurological symptoms. We identified a total of 37 attacks. Twenty-one attacks were classified as parenchymatous, four non-parenchymatous and 12 as other syndromes. Seventeen patients had CSF analysis performed (20 samples). Five samples were normal, 15 showed CSF pleocytosis. The most frequent finding on MRI performed in the acute phase was extensive lesions involving the brainstem. Two patients died due to the neurological involvement of BD.
  
  
  CONCLUSIONS
  We found 18.1% prevalence of NB and a higher female-to-male ratio in our group than in other series. Gastrointestinal and vascular involvement was more frequent in the NB group. The fact that neurological involvement may be the first manifestation of BD with therapeutic implications and associated morbidity points out the relevance of an early diagnosis.
authors.affiliation: Department of Neurology, Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Domingos
authors.forename: Cláudia
authors.initials: C
authors.lastname: Ferrão
authors.forename: Joana
authors.initials: J
authors.lastname: Ramalho
authors.forename: Tiago
authors.initials: T
authors.lastname: Rodrigues
authors.forename: Bruno
authors.initials: B
authors.lastname: Moreira
authors.forename: Ernestina
authors.initials: E
authors.lastname: Santos
authors.forename: Andreia
authors.initials: A
authors.lastname: Bettencourt
authors.forename: Ana
authors.initials: A
authors.lastname: Martins da Silva
authors.forename: Berta
authors.initials: B
authors.lastname: Silva
authors.forename: Paulo
authors.initials: P
authors.lastname: Pinho E Costa
authors.forename: Carlos
authors.initials: C
authors.lastname: Vasconcelos
authors.forename: João
authors.initials: J
authors.lastname: Correia
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000150:complications / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011174:Portugal; D012189:Retrospective Studies