RESUMEN
INTRODUCTION: Acute disseminated encephalomyelitis (ADE) is an inflammatory disorder of the central nervous system that is mediated immunologically and of unknown pathogenesis. It can present at any age, but is much more frequent in children. ADE has no specific biological marker and diagnosis is based on findings from clinical and neuroimaging studies. AIM: To enhance our knowledge of the clinico-radiological profile of this disease. PATIENTS AND METHODS: This retrospective study involved patients under 14 years of age who were admitted to a tertiary hospital over the last 15 years with a diagnosis of ADE. History, clinical presenting symptoms, lab findings from blood/cerebrospinal fluid analyses and radiological semiology were reviewed. In 16 cases an average follow-up of 25 months was performed. RESULTS: The study examined 20 patients, 70% children, with a mean age of 4.4 years. Forty per cent had a previous febrile episode. Eighty-five per cent presented fever or vomiting, and 70% had altered states of mind. Motor deficits (45%), convulsions (35%) and involvement of the cranial nerves (30%) were predominant. Three children progressed with relapses and three others were left with motor sequelae. Magnetic resonance imaging showed hyperintense lesions in T2, with a pattern of scarce/no enhancement, which were predominantly located in the thalamus (70%), the spinal cord (67%) and the white matter of the sub-cortex (50%). Haemorrhagic ADE was diagnosed in two patients. CONCLUSIONS: ADE is a condition with an important degree of general involvement and neurological repercussions, as well as considerable potential to leave the patient with sequelae. Clinico-analytical data and magnetic resonance scans of the head and spinal cord are relevant for the initial diagnosis and follow-up of patients with ADE.
TITLE: Perfil clinicorradiologico de la encefalomielitis aguda diseminada en la poblacion infantil. Analisis retrospectivo de una serie de 20 pacientes de un hospital terciario.Introduccion. La encefalomielitis aguda diseminada (EAD) es un trastorno inflamatorio del sistema nervioso central mediado inmunologicamente y de patogenia desconocida. Puede presentarse en cualquier edad, pero es mucho mas frecuente en niños. La EAD no tiene marcador biologico especifico y el diagnostico se basa en hallazgos clinicos y neurorradiologicos. Objetivo. Mejorar el conocimiento del perfil clinicorradiologico de esta enfermedad. Pacientes y metodos. Estudio retrospectivo con inclusion de pacientes menores de 14 años ingresados en un hospital terciario en los ultimos 15 años con el diagnostico de EAD. Se revisaron antecedentes, signos clinicos de presentacion, datos analiticos en sangre/liquido cefalorraquideo y la semiologia radiologica. En 16 casos se realizo un seguimiento medio de 25 meses. Resultados. Se revisaron 20 pacientes, un 70% niños, con una edad media de 4,4 años. El 40% tuvo un episodio febril previo. El 85% presento fiebre o vomitos, y el 70%, afectacion del estado de consciencia. Predominaron los deficits motores (45%), las convulsiones (35%) y la afectacion de pares craneales (30%). Tres niños presentaron una evolucion recidivante, y otros tres, secuelas motoras. Los estudios de resonancia magnetica mostraron lesiones hiperintensas en secuencias T2, con patron de realce escaso o nulo, que predominaron en los talamos (70%), la medula (67%) y la sustancia blanca subcortical (50%). En dos pacientes se diagnostico EAD hemorragica. Conclusiones. La EAD representa una entidad con importante afectacion general y repercusion neurologica, que muestra un potencial secuelar considerable. Los datos clinicoanaliticos y la resonancia magnetica cerebral y medular son relevantes para el diagnostico inicial y seguimiento de pacientes con EAD.