[Acute disseminated encephalomyelitis. Experience of a tertiary hospital in Spain]. / Encefalomielitis aguda. Experiencia de un hospital terciario español.

INTRODUCTION: To describe the epidemiological, clinical, microbiological, neuroimaging and laboratory features, treatment, and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). PATIENTS AND METHODS: Retrospective chart review was performed of children with a diagnosis of ADEM over a 23-year period in a tertiary hospital in Spain. RESULTS: Twelve cases were identified. Ten cases (83%) occurred after 1992. Nine patients (75%) presented between April and September. The mean age was 6 years. Nine patients (75%) were male. Fifty percent of the patients had a history of infectious disease or vaccination. The most frequent nonspecific symptom was fever in 75%. The most frequent neurological manifestations were motor deficits and altered consciousness in 75%. Cerebrospinal fluid abnormalities were found in 83%. All patients had at least one brain scan and one brain magnetic resonance imaging (MRI) scan. Three patients underwent spinal MRI. The sensitivity of MRI was greater than that of the scanner in the diagnosis of ADEM. An etiologic diagnosis was made in four patients: Mycoplasma pneumoniae, beta hemolytic streptococcus group A, Epstein-Barr virus and measles-mumps-rubella vaccination. Eleven patients were treated with corticosteroids and one was treated with intravenous immunoglobulin therapy. One patient died while 75 % of the patients had a good outcome. CONCLUSIONS: ADEM is in an infrequent disease in children. The clinical features are similar to those of infectious encephalitis. Etiologic diagnosis is difficult to establish but this entity is usually preceded by an infection. The neuroimaging test of choice to establish the diagnosis is MRI. In most patients, the prognosis is good.

Encefalomielitis aguda. Experiencia de un hospital terciario / Acute disseminated encephalomyelitis. Experience of a tertiary hospital in Spain

Introducción. El objetivo de este estudio es describir las características epidemiológicas, clínicas, microbiológicas, parámetros de laboratorio, estudios de neuroimagen, tratamiento y resultado de una cohorte de niños con diagnóstico de encefalomielitis aguda diseminada (EMAD). Pacientes y métodos. Estudio retrospectivo de las historias clínicas de los niños con diagnóstico de EMAD atendidos durante 23 años en un hospital terciario español. Resultados. Se identificaron 12 casos. Diez (83 %) a partir del año 1992. Nueve (75 %) se presentaron entre abril y septiembre. La edad media fue de 6 años. El 75 % eran varones. En el 50 % hubo infección o vacunación previa. El síntoma general más frecuente fue la fiebre, en el 75 %. La afectación motora y alteración de conciencia fueron las principales manifestaciones neurológicas, presentes en el 75 %. El estudio del líquido cefalorraquídeo (LCR) fue anormal en el 83 %. En todos los pacientes se realizó al menos un escáner y una resonancia magnética (RM) craneal y en 3 casos RM espinal. La RM se mostró más sensible que el escáner para el diagnóstico. Se estableció el diagnóstico etiológico en 4 casos: Mycoplasma, estreptococo betahemolítico del grupo A (EBHGA), virus de Epstein-Barr (VEB) y vacunación triple vírica. Once pacientes recibieron tratamiento con corticoides y en 1 caso se asociaron inmunoglobulinas intravenosas. Un paciente falleció y el 75 % tuvo una evolución favorable. Conclusiones. Se trata de una enfermedad poco frecuente en la infancia. La sintomatología es superponible a la de las encefalitis agudas infecciosas. La etiología es difícil de establecer, pero suele estar precedida por un antecedente infeccioso. La RM fue la prueba de imagen de elección para establecer el diagnóstico. El pronóstico suele ser favorable

Introduction. To describe the epidemiological, clinical, microbiological, neuroimaging and laboratory features, treatment, and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). Patients and methods. Retrospective chart review was performed of children with a diagnosis of ADEM over a 23-year period in a tertiary hospital in Spain. Results. Twelve cases were identified. Ten cases (83 %) occurred after 1992. Nine patients (75 %) presented between April and September. The mean age was 6 years. Nine patients (75 %) were male. Fifty percent of the patients had a history of infectious disease or vaccination. The most frequent nonspecific symptom was fever in 75 %. The most frequent neurological manifestations were motor deficits and altered consciousness in 75 %. Cerebrospinal fluid abnormalities were found in 83 %. All patients had at least one brain scan and one brain magnetic resonance imaging (MRI) scan. Three patients underwent spinal MRI. The sensitivity of MRI was greater than that of the scanner in the diagnosis of ADEM. An etiologic diagnosis was made in four patients: Mycoplasma pneumoniae, beta hemolytic streptococcus group A, Epstein-Barr virus and measles-mumps-rubella vaccination. Eleven patients were treated with corticosteroids and one was treated with intravenous immunoglobulin therapy. One patient died while 75 % of the patients had a good outcome. Conclusions. ADEM is in an infrequent disease in children. The clinical features are similar to those of infectious encephalitis. Etiologic diagnosis is difficult to establish but this entity is usually preceded by an infection. The neuroimaging test of choice to establish the diagnosis is MRI. In most patients, the prognosis is good