Encefalomielitis aguda diseminada y esclerosis múltiple en pediatría
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Palabras clave

encefalomielitis aguda diseminada
esclerosis múltiple (EM)
enfermedades desmielinizantes
desmielinización (Acta Neurol Colomb 2006;22:163-179).

Resumen

La encefalomielitis aguda diseminada, la esclerosis múltiple, la neuritis óptica, la mielitis transversa y la neuromielitis óptica son algunos ejemplos de trastornos desmielinizantes, cuyos criterios diagnósticos en adultos son objeto de revisión permanente. Se actualizarán los aspectos importantes de las dos enfermedades desmielinizantes más frecuentes en pediatría. La encefalomielitis aguda diseminada puede ocurrir a cualquier edad, es más frecuente en niños y presenta curso monofásico. Sin embargo, se han descrito algunas formas con recaídas, haciendo difícil su distinción de la esclerosis múltiple. Los pacientes con encefalomielitis aguda diseminada inicial que evolucionan a la esclerosis múltiple varían entre 9.5-27 por ciento. No existe tratamiento estandarizado más allá de las medidas iniciales de sostén. No se ha realizado hasta el momento un ensayo controlado y asignado al azar para su tratamiento en niños o en adultos. El tratamiento con corticoides constituye la terapia más utilizada y mejor tolerada en pacientes pediátricos.

El diagnóstico de esclerosis múltiple en pacientes menores de diez años es excepcional. Las características de las neuroimágenes en niños difieren del patrón usual del adulto. La esclerosis múltiple se define como una enfermedad del sistema nervioso central caracterizada por desmielinización, inflamación y daño axonal. La forma evolutiva más frecuente, en niños, es la esclerosis múltiple con recaídas y remisiones (80 por ciento a los diez años), seguida por las formas: secundaria progresiva (26 por ciento) y primaria progresiva (6-14 por ciento). Es necesario reconsiderar el límite inferior de edad para el diagnóstico de esclerosis múltiple, o bien, desarrollar criterios diagnósticos clínicos y radiológicos específicos para pacientes pediátricos.

Debe considerarse la particular forma de presentación en los niños menores de diez años y establecer claramente el diagnóstico diferencial con las formas recurrentes y multifásicas de encefalomielitis aguda diseminada. Como el número de pacientes pediátricos con esclerosis múltiple que requerirán tratamiento aumentará con el tiempo, existe una clara necesidad de extender el acceso a las terapias inmunomoduladoras a los niños con formas activas de esclerosis múltiple.

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