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La enfermedad de Behçet (EB) es una vasculitis inflamatoria multisistémica de etiología incierta e infrecuente, clasificada como una enfermedad rara en Estados Unidos y países europeos. El síndrome de Neuro-Behçet (SNB) ocurre cuando se presenta compromiso neurológico en pacientes con EB, en los cuales se ha descartado otras etiologías neurológicas. El espectro de afectación neurológica en la EB es muy amplio, sin embargo, las manifestaciones clínicas y los hallazgos de neuroimagen guían hacia dos grandes grupos de SNB: parenquimatoso y no parenquimatoso. La EB se debe incluir en el diagnóstico diferencial de enfermedades inflamatorias, vasculares y desmielinizantes del SNC.
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