Diagnosis and Treatment of Neuro-Behçet: A Clinical Update
Autor(a) principal: | |
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Data de Publicação: | 2023 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734 |
Resumo: | Behçet’s disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal diseaseshould be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation. |
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Diagnosis and Treatment of Neuro-Behçet: A Clinical UpdateDiagnóstico e Tratamento do Neuro-Behçet: Uma Actualização ClínicaBehcet Syndrome/complicationsBehcet Syndrome/diagnosisBehcet Syndrome/drug therapyCentral Nervous System Diseases/diagnosisCentral Nervous System Diseases/drug therapyDoenças do Sistema Nervoso Central/diagnósticoDoenças do Sistema Nervoso Central/tratamento farmacológicoSíndrome de Behçet/complicaçõesSíndrome de Behçet/diagnósticoSíndrome de Behçet/tratamento farmacológicoBehçet’s disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal diseaseshould be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation. A doença de Behçet é uma síndrome inflamatória multissistémica recidivante, caraterizada por úlceras orais e/ou genitais recorrentes, uveítes, artrite, lesões cutâneas e envolvimento gastrointestinal e neurológico. O neuro-Behçet corresponde ao envolvimento do sistema nervoso e é uma das complicações mais graves da doença de Behçet. Ocorre em 3% a 30% dos casos e categoriza-se em doença parenquimatosa (mais frequente) ou não-parenquimatosa. A manifestação mais comum do neuro-Behçet parenquimatoso é a meningoencefalite com acometimento do tronco cerebral, sendo que os doentes se apresentam com neuropatias cranianas, encefalopatia, síndromes sensitivo-motoras, epilepsia ou mielite. A principal manifestação não-parenquimatosa é a trombose venosa cerebral. O neuro-Behçet apresenta uma evolução maioritariamente subaguda, com remissão em semanas, ou com progressão clínica, em um terço dos casos. O diagnóstico é essencialmente clínico e os exames complementares auxiliam a corroborar a suspeita, a diferenciar de diagnósticos diferenciais e a excluir complicações. A ressonância magnética cerebral permite observar lesões agudas (hipo ou isointensas em T2 e hipointensas em T1) que captam contraste, e lesões crónicas caraterizadas por pequenas lesões que não captam contraste e atrofia do tronco cerebral. Na suspeita de envolvimento não-parenquimatoso deve ser realizada venoressonância magnética/tomografia computorizada cerebral. O líquido cefalorraquidiano apresenta elevação da proteinorraquia e da pleocitose no neuro-Behçet parenquimatoso e não tem alterações no não-parenquimatoso (exceto aumento da pressão de abertura). Os surtos de doença parenquimatosa devem ser tratados com corticoterapia endovenosa em alta dose, com posterior desmame para corticoterapia oral, seguida de terapêutica biológica, habitualmente anti-TNF. O tratamento da trombose venosa cerebral é controverso, podendo consistir na associação de corticoterapia e anticoagulação. Ordem dos Médicos2023-06-21info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734Acta Médica Portuguesa; Vol. 36 No. 9 (2023): September; 588-594Acta Médica Portuguesa; Vol. 36 N.º 9 (2023): Setembro; 588-5941646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734/15162Direitos de Autor (c) 2023 Acta Médica Portuguesainfo:eu-repo/semantics/openAccessSilva, LéniaCorreia, JoãoSantos, Ernestina2023-09-03T03:00:34Zoai:ojs.www.actamedicaportuguesa.com:article/19734Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T18:01:32.181946Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update Diagnóstico e Tratamento do Neuro-Behçet: Uma Actualização Clínica |
title |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
spellingShingle |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update Silva, Lénia Behcet Syndrome/complications Behcet Syndrome/diagnosis Behcet Syndrome/drug therapy Central Nervous System Diseases/diagnosis Central Nervous System Diseases/drug therapy Doenças do Sistema Nervoso Central/diagnóstico Doenças do Sistema Nervoso Central/tratamento farmacológico Síndrome de Behçet/complicações Síndrome de Behçet/diagnóstico Síndrome de Behçet/tratamento farmacológico |
title_short |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
title_full |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
title_fullStr |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
title_full_unstemmed |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
title_sort |
Diagnosis and Treatment of Neuro-Behçet: A Clinical Update |
author |
Silva, Lénia |
author_facet |
Silva, Lénia Correia, João Santos, Ernestina |
author_role |
author |
author2 |
Correia, João Santos, Ernestina |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Silva, Lénia Correia, João Santos, Ernestina |
dc.subject.por.fl_str_mv |
Behcet Syndrome/complications Behcet Syndrome/diagnosis Behcet Syndrome/drug therapy Central Nervous System Diseases/diagnosis Central Nervous System Diseases/drug therapy Doenças do Sistema Nervoso Central/diagnóstico Doenças do Sistema Nervoso Central/tratamento farmacológico Síndrome de Behçet/complicações Síndrome de Behçet/diagnóstico Síndrome de Behçet/tratamento farmacológico |
topic |
Behcet Syndrome/complications Behcet Syndrome/diagnosis Behcet Syndrome/drug therapy Central Nervous System Diseases/diagnosis Central Nervous System Diseases/drug therapy Doenças do Sistema Nervoso Central/diagnóstico Doenças do Sistema Nervoso Central/tratamento farmacológico Síndrome de Behçet/complicações Síndrome de Behçet/diagnóstico Síndrome de Behçet/tratamento farmacológico |
description |
Behçet’s disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal diseaseshould be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation. |
publishDate |
2023 |
dc.date.none.fl_str_mv |
2023-06-21 |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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publishedVersion |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734 |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734 |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19734/15162 |
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Direitos de Autor (c) 2023 Acta Médica Portuguesa info:eu-repo/semantics/openAccess |
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Direitos de Autor (c) 2023 Acta Médica Portuguesa |
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Ordem dos Médicos |
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Ordem dos Médicos |
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Acta Médica Portuguesa; Vol. 36 No. 9 (2023): September; 588-594 Acta Médica Portuguesa; Vol. 36 N.º 9 (2023): Setembro; 588-594 1646-0758 0870-399X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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