Publicação
A 30-year experience in neuro-Behçet disease
| dc.contributor.author | Silva, Lénia | |
| dc.contributor.author | Silva, Isabel Fonseca | |
| dc.contributor.author | Fonseca, Tomás | |
| dc.contributor.author | Pinto, Luísa Serpa | |
| dc.contributor.author | Leal, Bárbara | |
| dc.contributor.author | Pinho e Costa, Paulo | |
| dc.contributor.author | Igreja, Liliana | |
| dc.contributor.author | Moreira, Bruno | |
| dc.contributor.author | Santos, Ernestina | |
| dc.contributor.author | Vasconcelos, Carlos | |
| dc.contributor.author | Marinho, António | |
| dc.contributor.author | Correia, João Araújo | |
| dc.date.accessioned | 2026-02-16T12:44:46Z | |
| dc.date.available | 2026-02-16T12:44:46Z | |
| dc.date.issued | 2025-05-22 | |
| dc.description.abstract | Background: Behçet disease (BD) is a systemic vasculitis affecting multiple organs with a wide range of severity. Neuro-Behçet (NBD) is a severe form, characterized by high morbidity, disability, and mortality rates. Methods: Retrospective analysis (1993-2023) of neurological involvement in BD patients at a tertiary center. Results: Of 296 BD patients, 93(31.4 %) underwent neurological evaluation. Definite NBD was identified in 30(10.1 %), probable NBD in 2(0.5 %) and "other neurological symptoms in BD" in 26(8.6 %) patients. The definite NBD group (median age: 36 years, 50 % female) had 44 neurological attacks: 24(55 %) parenchymatous and 20(45 %) non-parenchymatous. The most common syndromes were brainstem (27.3 %) and multifocal (25.6 %), with ataxia being the most frequent sign (40.9 %). One-third had a relapsing course. NBD onset concurred with BD diagnosis in 50 % of cases, followed in 30 %, and preceded in 20 %. Brain MRI revealed predominant involvement of the brainstem and diencephalic regions. The HLA-B*51 allele was more prevalent in definite NBD versus BD patients (53.8 % vs 31.2 %, p = 0.036). Treatments included corticosteroids (70.5 %), cyclophosphamide (15.9 %), infliximab (9.1 %), and conventional synthetic disease-modifying antirheumatic drugs (13.6 %). Better outcomes were achieved with cyclophosphamide and infliximab. The probable NBD and "other neurological symptoms in BD" groups (median age: 37 years) were mostly female (92.9 %). Headache (85 %) and cognitive complaints (23 %) were common symptoms. | eng |
| dc.description.abstract | Highlights: -10.1 % of Behçet patients had definite neuro involvement in a 30-year study. -Neurological symptoms preceded Behcet diagnosis in 20 % of cases. -Chronic headaches were frequent in Behcet patients, but unrelated to direct CNS involvement. -HLA-B*51 was linked to definite neuro-Behcet, with OR = 2.56 (p = 0.036). -Anti-TNFα drugs and cyclophosphamide were highly effective treatments. | eng |
| dc.identifier.citation | J Neuroimmunol. 2025 Sep 15:406:578647. doi: 10.1016/j.jneuroim.2025.578647. Epub 2025 May 22 | |
| dc.identifier.doi | 10.1016/j.jneuroim.2025.578647 | |
| dc.identifier.eissn | 1872-8421 | |
| dc.identifier.issn | 0165-5728 | |
| dc.identifier.pmid | 40414042 | |
| dc.identifier.uri | http://hdl.handle.net/10400.18/10942 | |
| dc.language.iso | eng | |
| dc.peerreviewed | yes | |
| dc.publisher | Elsevier | |
| dc.relation.hasversion | https://www.sciencedirect.com/science/article/pii/S0165572825001286?via%3Dihub | |
| dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/4.0/ | |
| dc.subject | Behçet's Disease | |
| dc.subject | Diagnosis | |
| dc.subject | Neuro-Behçet | |
| dc.subject | Outcome | |
| dc.subject | Treatment | |
| dc.subject | Doenças Genéticas | |
| dc.title | A 30-year experience in neuro-Behçet disease | eng |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| oaire.citation.startPage | 578647 | |
| oaire.citation.title | Journal of Neuroimmunology | |
| oaire.citation.volume | 406 | |
| oaire.version | http://purl.org/coar/version/c_970fb48d4fbd8a85 |