Atypical manifestations in Brazilian patients with neuro-Behcet's disease

Atypical manifestations in Brazilian patients with neuro-Behcet's disease

Author Dutra, Livia Almeida Autor UNIFESP Google Scholar
Goncalves, Celio Roberto Google Scholar
Braga-Neto, Pedro Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Gabbai, Alberto Alain Autor UNIFESP Google Scholar
Barsottini, Orlando Graziani Povoas Autor UNIFESP Google Scholar
Silva de Souza, Alexandre Wagner Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Hosp Israelita Albert Einstein
Universidade de São Paulo (USP)
Abstract Type and frequency of systemic and neurologic manifestations of Beh double dagger et'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-Beh double dagger et'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.
Keywords Behcet's syndrome
Neuro-Behcet's disease
Peripheral neuropathy
Cerebral venous thrombosis
Optic neuropathy
Demyelinating syndrome
Language English
Date 2012-06-01
Published in Journal of Neurology. Heidelberg: Springer Heidelberg, v. 259, n. 6, p. 1159-1165, 2012.
ISSN 0340-5354 (Sherpa/Romeo, impact factor)
Publisher Springer
Extent 1159-1165
Access rights Closed access
Type Article
Web of Science ID WOS:000304859300021

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