Abordaje neuropsicológico en una unidad pediátrica de daño cerebral adquirido del sistema público de salud / A neuropsychological approach in a paediatric acquired brain injury unit under the public health system

Introducción: El daño cerebral adquirido (DCA) pediátrico provoca dificultades cognitivo/conductuales y altera el curso del desarrollo. La unidad de DCA del Hospital Infantil Universitario Niño Jesús es la primera dentro del sistema público de salud en dar cobertura integral a pacientes y familias.ObjetivoSe pretende mostrar la metodología de trabajo con los niños y sus familias, describir las características clínicas de los pacientes atendidos y los resultados en cuanto a los tratamientos aplicados.Sujetos53 niños entre los tres meses y los 16 años y medio recibieron tratamiento. Las patologías atendidas son tumores cerebrales, accidentes cerebrovasculares, traumatismos craneoencefálicos, daño tras cirugía de la epilepsia e hipoxia.MétodoA todos los pacientes se le realizó una evaluación al ingreso y otra al alta. Los tratamientos se modulan en función de las dificultades y su gravedad, así como de la edad del niño. Las familias son atendidas tanto individualmente como en formato grupal.ResultadosUna mayor edad del niño se asocia con mejor recuperación del nivel cognitivo y menor duración del tratamiento. Las patologías tienen un impacto diferencial en el CI/CD evaluado al inicio de tratamiento, la hipoxia y las encefalitis son las que asocian mayor gravedad. Las puntuaciones al alta del CI/CD, así como las de memoria verbal y atención, mejoraron significativamente respecto a las del ingreso tras el tratamiento neuropsicológico multicomponente.ConclusionesLa atención al DCA debe incluir programas de rehabilitación neuropsicológica y proporcionar soporte emocional a la familia para que pueda participar activamente en la recuperación del niño o adolescente. (AU)

Introduction: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families.ObjectiveThis study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment.PatientsFifty-three patients aged between three months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia.MethodsAll patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy.ResultsOlder age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission.ConclusionsThe care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent. (AU)

A neuropsychological approach in a paediatric acquired brain injury unit under the public health system.

INTRODUCTION: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families. OBJECTIVE: This study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment. PATIENTS: Fifty-three patients aged between 3 months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia. METHODS: All patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy. RESULTS: Older age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission. CONCLUSIONS: The care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent.

A neuropsychological approach in a paediatric acquired brain injury unit under the public health system. / Abordaje neuropsicológico en una unidad pediátrica de daño cerebral adquirido del sistema público de salud.

INTRODUCTION: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families. OBJECTIVE: This study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment. PATIENTS: Fifty-three patients aged between three months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia. METHODS: All patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy. RESULTS: Older age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission. CONCLUSIONS: The care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent.

[Clinical and neuropsychological characteristics in congenital central hypoventilation syndrome]. / Aspectos clinicos y neuropsicologicos del sindrome de hipoventilacion central congenita.

INTRODUCTION: Congenital central hypoventilation syndrome (CCHS) syndrome is a rare disease caused by mutations in the PHOX2B gene. Patients show a reduced response to hypercapnia and hypoxia accompanied by diffuse disturbances of the autonomic nervous system and occasionaly also disturbances in neuroimaging. A specific neuropsychological profile has not been described in children and adolescents with CCHS. CASE REPORTS: We describe three cases (aged between 4 and 19 years) with different profiles of affectation in cognitive and functionality. These profiles are compared with the features described in the literature about neuropsychology in CCHS. CONCLUSIONS: The profile of functional impairment in the CCHS is variable: in case 1, a severe global developmental delay with autistic features and marked functional involvement is described. In case 2, bilateral atrophy of the hippocampus is associated with involvement in social cognition and in executive functions with moderate functional repercussion. Case 3 shows difficulties in some cognitive executive functions (planning and non-verbal fluency), but without functional repercussion. Neuropsychological assessment can help in the clinical management of these patients by determining and guiding the need for rehabilitation treatments.

TITLE: Aspectos clinicos y neuropsicologicos del sindrome de hipoventilacion central congenita.Introduccion. El sindrome de hipoventilacion central congenita (SHCC) es una enfermedad rara producida por mutaciones en el gen PHOX2B. Los pacientes muestran una reducida respuesta a la hipercapnia e hipoxia acompañada de alteraciones difusas del sistema nervioso autonomo y ocasionalmente alteraciones en neuroimagen. No se ha descrito un perfil neuropsicologico especifico en los niños y adolescentes con SHCC. Casos clinicos. Se presentan tres casos (de edades comprendidas entre 4 y 19 años) con diferente perfil de afectacion cognitiva y funcional. Se comparan los perfiles de los tres casos con los hallazgos descritos en la bibliografia sobre neuropsicologia en el SHCC. Conclusiones. El perfil de afectacion funcional en el SHCC es variable: en el caso 1 se describe un grave retraso global en el desarrollo con rasgos autistas y acusadas implicaciones funcionales. En el caso 2, la atrofia bilateral del hipocampo se asocia a deficit en cognicion social y alteraciones en funciones ejecutivas con moderada repercusion funcional. El caso 3 muestra dificultades en algunas funciones ejecutivas cognitivas (planificacion y fluidez no verbal), pero sin repercusion funcional. La evaluacion neuropsicologica puede ayudar en el manejo clinico de estos pacientes determinando y orientando la necesidad de tratamientos rehabilitadores.

Functional surgery in pediatric drug-resistant posterior cortex epilepsy: Electro-clinical findings, cognitive and seizure outcome.

PURPOSE: Epilepsies originated from the occipital, parietal and/or the posterior edge of the temporal lobe are grouped together into posterior cortex epilepsy (PCE). Our objective was firstly to describe electro-clinical and imaging findings in the presurgical evaluation of children with PCE, and secondly to identify potential factors associated with surgical and cognitive outcomes. METHOD: From the total of patients referred to the Epilepsy Monitoring Unit of 'Hospital Universitario Niño Jesús' from 2003 to 2016, 55 had drug-resistant PCE. Different variables obtained from the multimodal presurgical work-up were analyzed among patients achieving seizure freedom after surgery (ILAE class 1) and patients with persistent seizures. Categorical variables were compared with Fishers exact test and numeric variables with t-Student for independent samples, and multiple logistic regression were used to analyze predictive values. RESULTS: Median duration of epilepsy until surgery was 5 years [3-10 years]. Fifty patients showed lesions in the MRI, and 62.5% had concordant MRI-PET corregistration. 37 (67%) patients were operated (lesionectomy in 21 subjects, tailored resection based on intracranial studies in 16), and 23 (62,2%) reached ILAE class 1, with a mean follow-up period of 3.51 [1-12] years. A lower number of basal seizures and antiepileptic drugs, a well-defined lesion on the MRI, an epileptogenic zone (EZ) restricted to the posterior quadrant and the normalization of postsurgical EEGs were associated with seizure freedom (p<0.05). Additionally, 65% of patients had a long-term improvement of cognitive performances. CONCLUSIONS: Epilepsy surgery should be considered in children with drug-resistant PCE, especially in those with a restricted EZ.