title: Atypical manifestations in Brazilian patients with neuro-Behçet's disease.
abstract: Type and frequency of systemic and neurologic manifestations of Behçet's disease (BD) vary with ethnicity. In Brazil, BD occurs as sporadic cases. We describe clinical and radiological features of 36 Brazilian patients of mixed ethnicity with neuro-Behçet's disease (NBD). Medical records of 178 BD patients were reviewed and 36 (20%) NBD patients were identified. Twenty-one NBD patients (58.3%) were female and 27 (75%) presented with parenchymal manifestations. Brainstem involvement was the most common neurologic syndrome (41.7%). Seizures (27.8%), isolated aseptic meningitis (16.7%), optic neuropathy (ON) (16.7%), cerebral venous thrombosis (CVT) (8.3%), peripheral neuropathy (2.8%), and spinal cord involvement (5.6%) were other neurologic manifestations observed among Brazilian NBD patients. Eighteen (50%) had at least one relapse, and isolated aseptic meningitis was the most common relapsing manifestation. No significant differences concerning the number of relapses between parenchymal and non-parenchymal groups were found. A multivariate model including disease duration, cell count in spinal fluid, cyclosporine use, immunosuppressive use at disease onset, age at NBD onset, and ON did not reveal any significant associations with NBD relapse. There was a low frequency of CVT and an unexpected higher number of isolated aseptic meningitis. Brazilian NBD patients present more parenchymal and atypical manifestations, and relapse more often than NBD patients from other populations.
authors.affiliation: General Neurology Division, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, R Pedro de Toledo, 650 Vila Clementino, São Paulo 04039-002, Brazil. liviaadutra@hotmail.com
authors.forename: Lívia Almeida
authors.initials: LA
authors.lastname: Dutra
authors.forename: Célio Roberto
authors.initials: CR
authors.lastname: Gonçalves
authors.forename: Pedro
authors.initials: P
authors.lastname: Braga-Neto
authors.forename: José Luiz
authors.initials: JL
authors.lastname: Pedroso
authors.forename: Alberto Alain
authors.initials: AA
authors.lastname: Gabbai
authors.forename: Orlando Graziani Povoas
authors.initials: OG
authors.lastname: Barsottini
authors.forename: Alexandre Wagner Silva
authors.initials: AW
authors.lastname: de Souza
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000188:drug therapy / Q000208:ethnology* / Q000473:pathology*; D001938:Brazil / Q000208:ethnology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009422:Nervous System Diseases / Q000188:drug therapy / Q000208:ethnology* / Q000473:pathology*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents