Diagnóstico e Tratamento do Neuro-Behçet: Uma Actualização Clínica
DOI:
https://doi.org/10.20344/amp.19734Palavras-chave:
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 farmacológicoResumo
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.
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