Evolución clínica de la forma hiperaguda de la encefalomielitis aguda desmielinizante / Clinical outcome of the hyperacute form of acute disseminated encephalomyelitis

Introducción: La encefalomielitis aguda desmielinizante (EMAD) es una enfermedad inflamatoria mutifocal y monofásica del sistema nervioso central (SNC) que afecta a la sustancia blanca. La evolución suele ser favorable. Presentamos nuestra experiencia en el manejo de la EMAD de curso clínico hiperagudo. Pacientes y métodos: Revisión de 5 casos de EMAD que se encontraban en coma en el momento del ingreso en la Unidad de Cuidados Intensivos Pediátricos (UCIP). Se describen los resultados epidemiológicos, clínicos, microbiológicos y los hallazgos de resonancia magnética (RM), así como el tratamiento y la evolución de dichos pacientes. Resultados: La edad media fue de 5,2 años (100% varones). Ningún caso presentó criterios de leucoencefalitis aguda hemorrágica. Todos los pacientes ingresaron en coma y precisaron ventilación mecánica controlada. Todos presentaron fiebre previamente al ingreso. El intervalo entre el primer síntoma de afectación neurológica y el coma fue ≤ 24 h. En 3 casos se objetivó alteración bioquímica del LCR. El estudio inmunológico del LCR detectó bandas oligoclonales en un paciente. En la primera RM, la sustancia blanca supratentorial y núcleos basales se mostraron afectados en todos los casos y 3 presentaron afectación medular. Todos recibieron tratamiento con metilprednisolona a dosis altas. Cuatro recibieron tratamiento con inmunoglobulina G a dosis altas y uno, plasmaféresis. Dos pacientes fallecieron durante su estancia en UCIP. Las lesiones radiológicas de sustancia blanca persistieron al alta. A los 9 meses, los 3 supervivientes presentaban un Glasgow Outcome Scale de 5 puntos y no habían presentado recaídas. Conclusiones: Existen formas hiperagudas de EMAD que se acompañan de una mortalidad elevada. A corto plazo, la mejoría clínica no se acompaña de una regresión paralela de las lesiones detectables en la RM(AU)

Introduction: Acute disseminated encephalomyelitis (ADEM) is an inflammatory disease that damages the white matter of the central nervous system. Its clinical course is monophasic and multifocal. The outcome is usually favourable. We report our experience in the management of the hyperacute form of ADEM. Patients and methods: A retrospective chart review was performed on five patients admitted in coma with a diagnosis of ADEM in the Paediatric Intensive Care Unit (PICU). We describe their epidemiological, clinical, microbiological, magnetic resonance imaging features and their treatment and outcomes. Results: The mean age was 5.2 years and all were male. None of them fulfilled radiologic criteria for acute haemorrhagic leukoencephalitis. At admission all patients were in a coma, and all were on controlled mechanical ventilation support. Before their admission all patients had fever. In all cases the time between the first neurological symptom and coma was ≤ 24hours. The cerebrospinal fluid examination was abnormal in three patients, and in one case, oligoclonal bands were detected. The first brain magnetic resonance imaging (MRI) showed white matter and basal ganglia lesions in all patients, and in three cases the spinal medulla was affected by demyelination. All patients were treated with a course of high-dose methylprednisolone. Four patients were also treated with high-dose immunoglobulins, and one of them received plasmapheresis. Two patients died, and one patient had severe sequelae at discharge from the PICU. At discharge the lesions in the white matter were still present in the MRI. After nine months the three survivors had a Glasgow Outcome Scale score of five and no one relapsed. Conclusions: There is a hyperacute clinical form of ADEM which has a high mortality rate. In the short term, the clinical improvement of hyperacute ADEM is not accompanied by a decrease of severity of the brain MRI(AU)

[Clinical outcome of the hyperacute form of acute disseminated encephalomyelitis]. / Evolución clínica de la forma hiperaguda de la encefalomielitis aguda desmielinizante.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an inflammatory disease that damages the white matter of the central nervous system. Its clinical course is monophasic and multifocal. The outcome is usually favourable. We report our experience in the management of the hyperacute form of ADEM. PATIENTS AND METHODS: A retrospective chart review was performed on five patients admitted in coma with a diagnosis of ADEM in the Paediatric Intensive Care Unit (PICU). We describe their epidemiological, clinical, microbiological, magnetic resonance imaging features and their treatment and outcomes. RESULTS: The mean age was 5.2 years and all were male. None of them fulfilled radiologic criteria for acute haemorrhagic leukoencephalitis. At admission all patients were in a coma, and all were on controlled mechanical ventilation support. Before their admission all patients had fever. In all cases the time between the first neurological symptom and coma was ≤ 24hours. The cerebrospinal fluid examination was abnormal in three patients, and in one case, oligoclonal bands were detected. The first brain magnetic resonance imaging (MRI) showed white matter and basal ganglia lesions in all patients, and in three cases the spinal medulla was affected by demyelination. All patients were treated with a course of high-dose methylprednisolone. Four patients were also treated with high-dose immunoglobulins, and one of them received plasmapheresis. Two patients died, and one patient had severe sequelae at discharge from the PICU. At discharge the lesions in the white matter were still present in the MRI. After nine months the three survivors had a Glasgow Outcome Scale score of five and no one relapsed. CONCLUSIONS: There is a hyperacute clinical form of ADEM which has a high mortality rate. In the short term, the clinical improvement of hyperacute ADEM is not accompanied by a decrease of severity of the brain MRI.