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.

[An unusual cause of failure of weaning from mechanical ventilation in an infant]. / Una causa inusual de fracaso del destete de la ventilación mecánica en un lactante.

The weaning process is a critical phase in patients undergoing mechanical ventilation. This process can be hampered by numerous causes, such as neuromuscular diseases and spinal muscular atrophy (SMA). We present a 6-month-old boy with respiratory distress, fever, marked hypotonia without motor developmental milestones, and areflexia. The patient showed progressive respiratory distress requiring mechanical ventilation. Definitive weaning was not achieved and the boy died from respiratory failure. Partial autopsy was performed with a diagnosis of SMA and genetic study of the parents. Neuromuscular diseases are an infrequent cause of respiratory insufficiency in suckling infants. The differential diagnosis is made between axonal and motor neuron diseases. The diagnosis was confirmed by muscular biopsy and genetic study.

Una causa inusual de fracaso del destete de la ventilación mecánica en un lactante / An unusual cause of failure of weaning from mechanical ventilation in an infant

El proceso de destete es una fase crítica del paciente tratado con soporte ventilatorio que puede verse dificultada por numerosas causas entre las que se encuentran las enfermedades neuromusculares, como la atrofia muscular espinal (AME). Lactante de 6 meses que presenta dificultad respiratoria y fiebre llamando la atención una marcada hipotonía generalizada sin logros motrices y arreflexia rotuliana. El niño sufre deterioro respiratorio con necesidad de asistencia ventilatoria mecánica sin lograrse un destete definitivo. Fallece por fallo respiratorio, realizándose necropsia parcial con diagnóstico de AME y estudio genético de progenitores. Las enfermedades neuromusculares son una causa poco frecuente de insuficiencia respiratoria en lactantes. Se realiza un diagnóstico diferencial entre las enfermedades de motoneurona, enfermedades axonales y enfermedades de placa motora. El diagnóstico fue confirmado por la biopsia muscular y el estudio genético

The weaning process is a critical phase in patients undergoing mechanical ventilation. This process can be hampered by numerous causes, such as neuromuscular diseases and spinal muscular atrophy (SMA). We present a 6-month-old boy with respiratory distress, fever, marked hypotonia without motor developmental milestones, and areflexia. The patient showed progressive respiratory distress requiring mechanical ventilation. Definitive weaning was not achieved and the boy died from respiratory failure. Partial autopsy was performed with a diagnosis of SMA and genetic study of the parents. Neuromuscular diseases are an infrequent cause of respiratory insufficiency in suckling infants. The differential diagnosis is made between axonal and motor neuron diseases. The diagnosis was confirmed by muscular biopsy and genetic study

[Markers of poor prognosis in convulsive status epilepticus]. / Factores de mal pronóstico en el estado epiléptico convulsivo.

OBJECTIVE: To characterize the variables that might be associated with mortality and the development of neurological deficits in children with convulsive status epilepticus. PATIENTS AND METHODS: Children older than 1 month and younger than 15 years who were admitted to the pediatric intensive care unit of a university hospital between 2001 and 2004 were reviewed. Epidemiologic and clinical factors that might be related to neurological outcome at discharge from the unit were analyzed. RESULTS: Forty-one patients (median age 24 months) were included. A total of 48.3% developed refractory convulsive status epilepticus. Six patients died (mortality 14.6%) during their intensive care unit stay and neurologic worsening was observed in 8.6% of survivors (adverse outcome in 22%). Symptomatic epilepsy was present in all patients who died and in 88.9% of those who recovered with severe neurologic sequelae. Uni- and multivariate analysis showed that adverse outcome was related to symptomatic origin and refractory convulsive status epilepticus (p < 0.05). CONCLUSIONS: Mortality and morbidity is high in childhood convulsive status epilepticus. Refractory convulsive status and symptomatic origin were markers of poor outcome. Children who did not have symptomatic epilepsy had a favorable outcome.

Factores de mal pronóstico en el estado epiléptico convulsivo / Markers of poor prognosis in convulsive status epilepticus

Objetivo: Investigar las variables que se asocian a la mortalidad y al desarrollo de secuelas neurológicas en los niños con estado epiléptico convulsivo. Pacientes y métodos: Revisión de los casos con estado epiléptico convulsivo, en niños mayores de un mes y menores de 15 años, tratados en la unidad de cuidados intensivos pediátricos de un hospital universitario entre los años 2001 y 2004. Se analizaron factores epidemiológicos y clínicos en relación con el estado neurológico al alta de la unidad. Resultados: Se incluyeron 41 pacientes con una edad mediana de 24 meses. El 43,9 % desarrolló estado epiléptico convulsivo refractario. Fallecieron 6 pacientes (mortalidad: 14,6 %) y en el 8,6 % de los supervivientes se observó un deterioro neurológico con respecto a su situación previa (mala evolución en el 22 %). Todos los pacientes que fallecieron y el 88,9 % de los que presentaron secuelas graves eran portadores de formas sintomáticas. El análisis univariante y multivariante reflejaron que la etiología sintomática y el estado epiléptico refractario se asociaron con un mal pronóstico (p < 0,05). Conclusiones: La morbimortalidad del estado epiléptico convulsivo es elevada y se relaciona con la etiología sintomática y con el desarrollo de resistencia al tratamiento. Los niños que no presentan un estado epiléptico convulsivo de tipo sintomático presentan un pronóstico favorable

Objective: To characterize the variables that might be associated with mortality and the development of neurological deficits in children with convulsive status epilepticus. Patients and methods: Children older than 1 month and younger than 15 years who were admitted to the pediatric intensive care unit of a university hospital between 2001 and 2004 were reviewed. Epidemiologic and clinical factors that might be related to neurological outcome at discharge from the unit were analyzed. Results: Forty-one patients (median age 24 months) were included. A total of 48.3 % developed refractory convulsive status epilepticus. Six patients died (mortality 14.6 %) during their intensive care unit stay and neurologic worsening was observed in 8.6 % of survivors (adverse outcome in 22 %). Symptomatic epilepsy was present in all patients who died and in 88.9 % of those who recovered with severe neurologic sequelae. Uni- and multivariate analysis showed that adverse outcome was related to symptomatic origin and refractory convulsive status epilepticus (p < 0.05). Conclusions: Mortality and morbidity is high in childhood convulsive status epilepticus. Refractory convulsive status and symptomatic origin were markers of poor outcome. Children who did not have symptomatic epilepsy had a favorable outcome

[Replacement therapy with protein C for meningococcal sepsis and fulminant purpura in pediatric patients]. / Tratamiento sustitutivo con proteína C en sepsis meningocócica y púrpura fulminante en pacientes pediátricos.

Disseminated intravascular coagulation as associated to sepsis contributes to the development of clinical multiple organ failure by extensive thrombosis in microcirculation vessels. This condition commonly manifests itself in severe meningococcal sepsis. On the skin, its clinical manifestation is extensive purpura with necrotic lesions that usually progress to serious distal ischemia and may call for amputation. A common denominator in these events regarding hemostasis is a depletion of so-called natural anticoagulant proteins, particularly protein C. According to clinical observations replacement therapy with human plasma-derived protein C concentrates has been associated with significantly improved clinical outcome in patients with meningococcal sepsis and fulminant purpura. This paper reports a case of acquired protein C deficiency in a girl with meningococcal sepsis, fulminant purpura, disseminated intravascular coagulation, and septic shock. Fresh plasma therapy was intended to increase consumption coagulopathy-depleted coagulation factors and to provide small amounts of protein C. The inability to restore protein C concentrations above 30%, and the presence of severe thrombopenia in the setting of disseminated intravascular coagulation led to the onset of replacement therapy using a human protein C concentrate (Ceprotin), which increased plasma protein C concentrations and contributed to revert the existing hypercoagulability status. Finally, evidence available in the literature regarding fulminant meningococcal sepsis management using human protein C concentrates and recombinant activated protein C is discussed.

[Abnormalities in coagulation and fibrinolysis in septic shock with purpura]. / Alteraciones del sistema de la coagulación y la fibrinólisis en el shock séptico asociado a púrpura.

OBJECTIVE: To describe abnormalities in coagulation and fibrinolysis in septic shock with purpura and to assess the relationship between plasma plasminogen activator inhibitor-1 (PAI-1) concentrations and multiple organ system failure (MOSF). METHODS: Observational study in the pediatric intensive care unit of a tertiary care hospital. The presence of early MOSF was assessed at admission in 15 children with septic shock and purpura consecutively admitted to the pediatric intensive care unit. Blood samples were taken to determine coagulation and fibrinolysis parameters. RESULTS: At admission, MOSF was diagnosed in 7 patients (46.7 %), acute respiratory distress syndrome (ARDS) in 6 (40 %), consumption coagulopathy in 7 (46.7 %) and acute renal failure in 1 (6.7 %). The overall mortality rate was 40 %. Coagulation parameters were generally affected but statistically significant differences were found only in concentrations of fibrinogen and antithrombin III, which were lower in patients with MOSF than in those without organ dysfunction. Fibrinolysis parameters were increased in all patients but plasma PAI-1 concentrations were significantly elevated only in patients with MOSF and in those with ARDS. CONCLUSION: These data indicate that impaired fibrinolysis could play a major role in the development of MOSF in children with septic shock and purpura.