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• W3201118540 abstract "Despite progresses in autoimmune encephalitis and related disorders (AERD), they remain a major challenge in daily clinical practice. To describe a tertiary hospital's longitudinal experience in AERD between 2005 and 2020. Single-centre retrospective analysis of 43 patients. 43 patients were included, 55.8% with positive antibodies (10 antiGAD, 4 antiNMDAR, 2 antiGABABR, 2 antiLGI1, 2 antiCASPR2, 1 antiIgLON5, 1 antiHu, 1 antiYo, 1 antiCV2 and 1 antiMa2, detecting coexisting antibodies in one patient: antiNMDAR + antiGABABR); 28% with negative antibodies; and 16.2% with steroid responsive encephalitis associated to antithyroid antibodies (SREAT). The median age was 62 years-old [14–88]. Females (62.8%) outnumbered males (37.2%). Limbic encephalitis was the most common clinical syndrome (60.5%), followed by SREAT (16.2%), autoimmune cerebellitis (9.3%), stiff-person syndrome (7%), antiNMDAR-encephalitis (5%) and antiIgLON5 encephalopathy (2%). CSF showed pleocytosis and/or hyperproteinorrhachia in 54.2%. MRI was unremarkable in 60%. Brain SPECT/PET showed hyperperfusion/hypermetabolism of limbic areas in 60% of patients to whom it was performed. Antibody-positivity was significantly associated with satisfaction of diagnostic criteria at high levels of certainty (p < 0.001). Treatment escalation was more frequent in antibody-positive patients. The creation of a Neuroimmunology Unit improved diagnostic and treatment approaches. AERD's diagnosis and treatment have significantly improved over the last years. However, several limitations remain, particularly concerning antibody-negative AERD. The proposed diagnostic criteria might be still too reliant on antibody-positivity. Antibody-status seems to condition treatment escalation. The creation of a Neuroimmunology Unit optimized AERD's management in clinical practice. Describir la experiencia longitudinal de un hospital terciario en encefalitis autoinmunes y trastornos relacionados entre 2005 y 2020. Análisis retrospectivo unicéntrico de 43 pacientes. Se incluyen 43 pacientes, 55.8% con anticuerpos positivos (10 antiGAD, 4 antiNMDAR, 2 antiGABABR, 2 antiLGI1, 2 antiCASPR2, 1 antiIgLON5, 1 antiHu, 1 antiYo, 1 antiCV2 y 1 antiMa2, detectándose anticuerpos coexistentes en 1 paciente: antiNMDAR + antiGABABR); 28% con anticuerpos negativos; 16.2% con encefalopatía con respuesta a esteroides y anticuerpos antitiroideos (SREAT). Edad media = 62 años [14–88], predominio femenino (62.8%/37.2%). La encefalitis límbica fue el síndrome más frecuente (60.5%), seguida por SREAT (16.2%), cerebelitis autoinmune (9.3%), síndrome de la persona rígida (7%), encefalitis antiNMDAR (5%) y encefalopatía antiIgLON5 (2%). Un 54.2% presentó plecotosis/hiperproteinorraquia en LCR. La RM fue normal en un 60%. El SPECT/PET demostró hiperperfusión/hipermetabolismo límbico en un 60% de los casos a los que se realizó. La positividad a anticuerpos se asoció con la satisfacción de criterios diagnósticos de alta certeza (p < 0.001). En pacientes con anticuerpos positivos fue más frecuente la escalada terapéutica. La Unidad de Neuroinmunología optimizó el abordaje diagnóstico-terapéutico. El diagnóstico y tratamiento de las encefalitis autoinmunes y trastornos relacionados se ha desarrollado significativamente en los últimos años. Sin embargo, persisten numerosas limitaciones, particularmente afectando al grupo con anticuerpos negativos. Los criterios diagnósticos propuestos podrían ser aún demasiado dependientes de la presencia de anticuerpos, la cual parece condicionar también el tratamiento. La creación de Unidades de Neuroinmunología puede optimizar el manejo de estas enfermedades en la práctica clínica." @default.
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• W3201118540 date "2021-10-01" @default.
• W3201118540 modified "2023-10-01" @default.
• W3201118540 title "Autoimmune encephalitis and related disorders: A retrospective study of 43 cases in a tertiary hospital" @default.
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• W3201118540 doi "https://doi.org/10.1016/j.neurop.2021.09.001" @default.
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