Search

Introducción: La parálisis cerebral es la causa más frecuente de discapacidad infantil. Los niños con PC van a asociar, en la mayoría de los casos, además de los problemas neurológicos, otros trastornos no neurológicos que serán más frecuentes a mayor grado de afectación de la PC. Objetivo: Abordar las manifestaciones clínicas del niño con parálisis cerebral y su manejo diagnóstico y terapéutico con el fin de facilitar en un único documento un enfoque integral de estos pacientes. Desarrollo: Se realiza una revisión de las manifestaciones clínicas del niño con parálisis cerebral incluyendo trastornos neurológicos, problemas ortopédicos, trastornos digestivos, problemas respiratorios, salud ósea, problemas visuales y auditivos, trastornos urológicos y sexuales, salud bucodental, sialorrea, trastorno del sueño y dolor. Conclusiones: El abordaje del paciente con PC ha cambiado en los últimos años siendo fundamental la atención por un equipo multidisciplinar especializado en los diferentes problemas que presentan estos niños. Sin embargo, desde nuestra experiencia parece fundamental la figura de un pediatra conocedor de todos los problemas asociados que coordine todo el seguimiento. Palabras clave: diagnóstico, Discapacidad, parálisis cerebral, trastornos neurológicos, Tratamiento

Yes

Introduction Passive transplacental immunity against respiratory syncytial virus (RSV) appears to mediate in the protection of the infant for the first 6 months of life. Lower environmental exposure in pregnant women to RSV epidemic may influence the susceptibility of these infants to infection by lowering the levels of antibodies that are transferred to the fetus. Objectives To contrast the risk of severe disease progression in infants with acute bronchiolitis by RSV, according to the mother's level of exposure to epidemic. Method Retrospective cohort study of previously healthy infants with RSV-acute bronchiolitis during 5 epidemics was made. We compared the severity of the infection in those born during the period of risk (when is less likely the mother's exposure to epidemic and the transfer of antibodies to the fetus: October 15th–December 15th in our latitude) with the rest of acute bronchiolitis. Bivariate analysis was performed regarding birth in period of risk and the rest of variables, using the Chi-square test. Multivariate logistic regression analysis was performed to study possible classical confounding factors. Results 695 infants were included in the study. 356 infants were born during the period of risk. Of the 56 patients requiring admission to PICU, 40 of them (71.4%) were born in this period (p = 0.002). In the multivariate analysis, the birth in the period of risk showed a 6.5 OR (95% CI: 2.13–19.7) independently of the rest of variables. Conclusions The worst clinical disease progression of the acute bronchiolitis by the RSV in less than 6 months age is related to lower exposure of the pregnant woman to the RSV epidemic.

Resumen: Introducción: En la actualidad existen pocas escalas validadas para valorar la bronquiolitis aguda (BA) y escasa información de su potencia de prueba. El objetivo del presente estudio es valorar la validez de una escala de severidad de BA (ESBA) para orientar los ingresos en UCIP. Pacientes y método: Estudio observacional prospectivo descriptivo de lactantes previamente sanos menores de un año con BA, donde se utilizó la ESBA para contrastar la gravedad con la necesidad de ingreso en UCIP. El tamaño de la muestra se estimó en al menos 175 pacientes. El equipo investigador fue entrenado en el uso de la ESBA. Todos los pacientes del estudio fueron evaluados con la ESBA diariamente y en caso de deterioro clínico. Se analizaron y compararon las puntuaciones ESBA inicial y máxima respecto a la necesidad de UCIP. Se construyó una curva operativa de receptor, se calculó el área bajo la curva y se estimó el punto óptimo de sensibilidad/especificidad. Resultados: Se incluyó a 190 pacientes (varón/mujer: 58%/42%). Precisaron UCIP 11 (6%). La puntuación media ± DE de la ESBA-máxima para pacientes que precisaron y no precisaron UCIP fue de 10,55 ±1,12 y 6,35 ±2,3, respectivamente. Esta diferencia fue significativa (p

Introduction: At present, there are few validated scoring tests for assessing acute bronchiolitis (AB) severity, and limited information on their test power. The aim of the present study is to evaluate the validity of an acute bronchiolitis severity score (ABSS) to help in deciding PICU admission. Patients and method: Prospective, descriptive, observational study of previously healthy infants under 1 year of age with AB, where the ABSS was used to compare severity as regards the need for PICU admission. The sample size was estimated as at least 175 patients. The research team was trained in the use of ABSS. All patients in the study were evaluated with ABSS daily, as well as in the case of clinical deterioration. The initial and maximum ABSS scores were contrasted to the need for PICU admission. A receiver operative curve was constructed, and the area under the curve was calculated, and the optimum point of sensitivity/specificity was estimated. Results: The study included a total of 190 patients (male/female: 58%/42%). PICU was required in 11 (6%). The mean ± SD ABSS-maximal score for patients who required and did not require PICU was 10.55 ± 1.12 and 6.35 ± 2.3, respectively (p

Introduction: Acute bronchiolitis (AB) of the infant has a serious outcome in 6–16% of the hospital admitted cases. Its pathogenesis and evolution is related to the response of the T lymphocytes. The objective of the present study is to determine if the lower systemic lymphocytic response is related to a worse outcome of AB in hospitalised infants. Patients and method: Retrospective observational-analytical study of cases–controls nested in a cohort of patients admitted due to RSV-AB between the period from October 2010 to March 2015. Those with a full blood count in the first 48 h of respiratory distress were included. Infants with underlying disease, bacterial superinfection, and premature infants

Resumen: Introducción: La bronquiolitis aguda (BA) del lactante tiene una evolución grave entre el 6 y el 16% de los casos ingresados. Su patogenia y evolución está relacionada con la respuesta de los linfocitos T. El objetivo del presente estudio es comprobar si la menor respuesta linfocitaria sistémica está relacionada con una peor evolución de la BA en lactantes ingresados. Pacientes y método: Estudio observacional-analítico retrospectivo de casos-controles anidados en una cohorte de ingresados por BA-VRS en el periodo de octubre del 2010 a marzo del 2015. Se incluyó a aquellos con hemograma en las primeras 48 h de dificultad respiratoria. Se excluyó a los lactantes con patología de base, sobreinfección bacteriana y prematuros ≤ 32 semanas de gestación. La variable principal dicotómica fue ingreso UCIP. Otras variables fueron: sexo, edad, edad posmenstrual, exposición gestacional y posnatal al tabaco, mes de ingreso, tipo de lactancia y días de evolución del distrés respiratorio. Las cifras de linfocitos fueron categorizadas por cuartiles. Se realizó un análisis bivariante con la variable principal y posteriormente regresión logística para analizar factores de confusión. Resultados: El estudio incluyó a 252 lactantes. El 6,6% (17) precisó UCIP. La diferencia de media ± DE de linfocitos para pacientes ingresados y no ingresados en UCIP fue de 4.044 ± 1.755 y 5.035 ± 1.786, respectivamente (t de Student, p

Introduction: The presence of apnoea in acute bronchiolitis (AB) varies between 1.2% and 28.8%, depending on the series, and is one of its most fearsome complications. The aim of this study is to determine the incidence of apnoea in hospitalised patients diagnosed with AB, and to define their associated risk factors in order to construct a prediction model. Patients and method: A retrospective observational study of patients admitted to a tertiary hospital in the last 5 years with a diagnosis of AB, according to the classic criteria. Data was collected on the frequency of apnoea and related clinical variables to find risk factors in a binary logistic regression model for the prediction of apnoea. A ROC curve was developed with the model. Results: Apnoea was recorded during the admission of 53 (4.4%) patients out of a total of 1.197 cases found. The risk factors included in the equation were: female (OR 0.6, 95% CI: 0.27–1.37), caesarean delivery (OR: 3.44, 95% CI: 1.5–7.7), postmenstrual age ≤43 weeks (OR: 6.62, 95% CI: 2.38–18.7), fever (OR: 0.33, 95% CI: 0.09–1.97), low birth weight (OR: 5.93, 95% CI: 2.23–7.67), apnoea observed by caregivers before admission (OR: 5.93, 95% CI: 2.64–13.3), and severe bacterial infection (OR: 3.98, 95% CI: 1.68–9.46). The optimal sensitivity and specificity of the model in the ROC curve was 0.842 and 0.846, respectively (P

Resumen: Introducción: La presencia de apneas en la bronquiolitis aguda (BA) varía según las series entre el 1,2 y el 28,8%, y es una de sus complicaciones más temibles. Nuestro objetivo es conocer la incidencia de apneas en pacientes ingresados con diagnóstico de BA y definir sus factores de riesgo asociados para construir un modelo de predicción. Pacientes y método: Estudio observacional retrospectivo de los últimos 5 años de pacientes ingresados en un hospital terciario con diagnóstico de BA según los criterios clásicos. Se recogieron la frecuencia de apneas y las variables clínicas relacionadas, para encontrar factores de riesgo en un modelo de regresión logística binaria para la predicción de apneas. Para evaluar el modelo se elaboró una curva ROC. Resultados: De 1.197 casos, se registró apnea durante el ingreso en 53 (4,4%). Los factores de riesgo incluidos en la ecuación fueron: sexo femenino (OR 0,6; IC del 95%: 0,27-1,37), cesárea (OR: 3,44; IC del 95%: 1,5-7,7), edad posmenstrual ≤ 43 semanas (OR: 6,62; IC del 95%: 2,38-18,7), fiebre (OR: 0,33; IC del 95%: 0,09-1,97), bajo peso al ingreso (OR: 3,06; IC del 95%: 1,23-7,67), apneas antes del ingreso observada por los cuidadores (OR: 5,93; IC del 95%: 2,64-13,3) y sobreinfección bacteriana grave (OR: 3,98; IC del 95%: 1,68-9,46). La sensibilidad y la especificidad óptima del modelo en la curva ROC fueron de 0,842 y 0,846, respectivamente (p

Resumen: Introducción: La renovación de las guías de práctica clínica sobre la bronquiolitis aguda (BA) obliga a reevaluar las consecuencias de su implantación. Pretendemos actualizar las principales variables clínico-epidemiológicas en pacientes ingresados por BA en el sur de Europa y analizar las causas de la estancia prolongada. Pacientes y método: Estudio retrospectivo de ingresos por BA durante 5 epidemias (2010-2015), con descripción de las principales variables clínico-epidemiológicas y análisis por regresión logística de los factores asociados a mayor estancia. Resultados: El inicio de la epidemia ocurrió entre las semanas cuarta de septiembre y tercera de octubre. De los menores de 2 años (42.530 niños), el 15,21% (6.468 pacientes) acudieron a urgencias por BA y el 2,36% ingresaron (1.006 pacientes), con un 18,5% de prematuros. El 12,2% tenían peso al nacimiento inferior a 2.300 g. El 21,1% eran menores de un mes, consultaron por apnea asociada el 10,8%, ingesta inferior al 50% en el 31,1% y presentaban sobreinfección bacteriana el 13,1%. Estos factores se asociaron de forma independiente a la estancia prolongada. La mediana de estancia fue de 5 días y la proporción de ingresos en la unidad de cuidados intensivos pediátricos (UCIP) del 8,5% de los casos. Conclusiones: El inicio de la epidemia de la bronquiolitis mostró una variabilidad de hasta 4 semanas en nuestro medio. Tras 5 años de la implantación de la guía de práctica clínica, la incidencia de ingresos está en torno al 2,3% y parece estable respecto a estudios previos. La edad media de los pacientes desciende a 2,4 meses, aunque con una proporción similar de ingresos en la UCIP de un 8,5%.Los factores de riesgo independiente asociados a una estancia hospitalaria más prolongada fueron: bajo peso al nacimiento, edad menor de un mes, apneas previas al ingreso, ingesta inferior al 50% y la sobreinfección bacteriana grave, donde la infección respiratoria superó la prevalencia de infección del tracto urinario grave. Abstract: Introduction: The renewal of clinical practice guidelines on acute bronchiolitis (AB) requires the re-assessment of the consequences of their implementation. An update is presented on the main clinical and epidemiological variables in patients hospitalised due to AB in Southern Europe and an analysis made of the causes associated with longer hospital stay. Patients and method: A retrospective study was conducted on patients admitted to hospital due to AB during 5 epidemics (2010-2015), with an analysis of the major clinical and epidemiological variables. A logistic regression analysis was performed on the factors associated with a longer hospital stay. Results: The beginning of the epidemic occurred between the 4th week of September and the 3rd week of October. Of those children under 2 years (42,530), 15.21% (6,468 patients) attended paediatric emergency department due to having AB, and 2.36% (1,006 patients) were admitted. Of these, 18.5% of were premature, 12.2% had a birth weight

Resumen Introduccion La tosferina es una enfermedad reemergente que afecta sobre todo a lactantes. A esta edad la gravedad se puede afectar por infecciones intercurrentes como la del virus respiratorio sincitial (VRS). Objetivos Estimar la incidencia de la infeccion por el VRS durante su epoca epidemica en pacientes ingresados por tosferina. Asimismo, estudiar su repercusion sobre la gravedad observada durante el ingreso. Pacientes y metodo Estudio descriptivo de casos con diagnostico de tosferina ingresados en un hospital terciario durante 3 anos donde se analizo la presencia de coinfeccion por el VRS. La estimacion de la gravedad se confronto por la incidencia de complicaciones y el nivel de cuidados requeridos. Resultados De un total de 73 lactantes con tosferina, 34 acontecieron en epoca epidemica de bronquiolitis. En 17 de los 34 se detecto coinfeccion por el VRS. La edad media no mostro diferencias significativas respecto a los no coinfectados. La estancia media y la necesidad de cuidados intensivos fue semejante. La necesidad de cuidados de oxigenoterapia y soporte nutricional fue superior en los coinfectados. Conclusiones La coinfeccion por el VRS en lactantes ingresados con tosferina acontecio en uno de cada 2 pacientes en epoca epidemica para el VRS, en lactantes de edad similar. La gravedad en terminos de estancia, presencia de apneas e ingreso en cuidados intensivos fue semejante, pero con mayor necesidad de cuidados respiratorios y soporte nutricional.

BACKGROUND Apnea is a life-threatening complication of pertussis, now a re-emerging cause of infant hospitalization worldwide. The incidence of apnea during pertussis ranges widely. The aim of this study was therefore to determine the incidence of apnea in hospitalized infants diagnosed with pertussis and to identify relevant risk factors for apnea. METHODS This was a retrospective analytical study. We included children hospitalized with pertussis at a tertiary hospital during a 5 year period from 2010 to 2015. Nasopharyngeal aspirates (NPA) were obtained in all subjects. Real-time polymerase chain reaction was used to test NPA samples for Bordetella pertussis. The daily charts were assessed to identify inpatients with apnea. Multivariable logistic regression was performed to identify independent risk factors for inpatient apnea. RESULTS Inpatient apnea was identified in 51 of 147 infants with pertussis confirmation (34.7%: 95%CI: 27-42%). Maternal smoking during pregnancy was the only statistically significant, independent predictor of inpatient apnea (OR: 4.48; 95%CI: 1.35-14.8). No statistically significant association was found with gender, corrected age; birthweight, caregiver smoking; lactation, weight at admission

Introduction: The renewal of clinical practice guidelines on acute bronchiolitis (AB) requires the re-assessment of the consequences of their implementation. An update is presented on the main clinical and epidemiological variables in patients hospitalised due to AB in Southern Europe and an analysis is made of the causes associated with longer hospital stay. Patients and method: A retrospective study was conducted on patients admitted to hospital due to AB during 5 epidemics (2010–2015), with an analysis of the major clinical and epidemiological variables. A logistic regression analysis was performed on the factors associated with a longer hospital stay. Results: The beginning of the epidemic occurred between the 4th week of September and the 3rd week of October. Of those children under 2 years (42 530), 15.21% (6468 patients) attended paediatric emergency department due to having AB, and 2.36% (1006 patients) were admitted. Of these, 18.5% were premature, 12.2% had a birth weight

The need for mechanical ventilation (MV) in acute bronchiolitis (AB) by respiratory syncytial virus (RSV) varies depending on the series (6-18%). Our goal is to determine the admissions to PICU for MV in patients under 6 months with AB and define the risk factors for building a prediction model.Retrospective study of patients younger than 6 months admitted by BA-VRS between the periods April 1, 2010 and March 31, 2015 was made. The primary variable was the admission to PICU for MV. Related addition, to find risk factors in a model of binary logistic regression clinical variables were collected. A ROC curve model was developed and optimal cutoff point was identified.In 695 cases, the need of MV in the PICU (Y) was 56 (8.1%). Risk factors (Xi) included in the equation were: 1. male sex (OR 4.27) 2. postmenstrual age (OR: 0.76) 3. Weight income less than p3 (OR: 5.53) 4. intake lees than 50% (OR: 12.4) 5. Severity by scale (OR: 1.58) 6. apneas before admission (OR: 25.5) 7. bacterial superinfection (OR 5.03) and 8. gestational age more than 37 weeks OR (0.32). The area under the curve, sensitivity and specificity were 0.943, 0.84 and 0.93 respectively.The PICU admission for MV was 8.1 in every 100 healthy infants hospitalized for AB and year. The prediction model equation can help to predict patients at increased risk of severe evolution.La necesidad de ventilación mecánica (VM) en la bronquiolitis aguda por el virus respiratorio sincitial (VRS) varía según las series entre el 6 y el 18 %. Nuestro objetivo fue conocer los ingresos en la unidad de cuidados intensivos para VM de pacientes con bronquiolitis aguda menores de 6 meses y definir los factores de riesgo con el fin de construir un modelo de predicción.Estudio retrospectivo de pacientes menores de 6 meses ingresados por bronquiolitis aguda por VRS entre los periodos 1 de abril de 2010 y 31 de marzo de 2015. La variable principal fue el ingreso en la unidad de cuidados intensivos pediátricos para ventilación mecánica. Además, se recogieron variables clínicas relacionadas para hallar factores de riesgo en un modelo de regresión logística binaria. Con el modelo se elaboró una curva ROC y se identificó un punto óptimo de corte.De 695 casos, precisaron VM 56 (8,1%). Los factores de riesgo (Xi) incluidos en la ecuación fueron: 1. Sexo varón (OR: 4,27) 2. Edad postmenstrual (OR:0,76) 3. Peso al ingreso inferior a p3(OR: 5,53) 4. Ingesta inferior a 50% (OR:12,4), 5. Gravedad por escala (OR:1,58), 6. Apneas antes del ingreso (OR:25,5) 7. Sobreinfección bacteriana (OR: 5,03) y 8. Edad gestacional superior a 37 semanas OR (0,32). El área bajo la curva, sensibilidad y especificidad fueron 0,943; 0,84 y 0,93 respectivamente.Los ingresos en UCIP para VM fueron 8,1 por cada 100 lactantes sanos hospitalizados por BA y año. La ecuación elaborada del modelo de predicción puede ser de ayuda para predecir los pacientes con mayor riesgo de evolución grave.

Aim. It has been suggested that the repetitive transcranial magnetic stimulation could be useful as a non-pharmacological treatment for spasticity. The aim of this study was to evaluate the clinical and neurophysiological effects of high-frequency intermittent theta burst stimulation (iTBS) on lower limb spasticity in patients with relapsing multiple sclerosis in a randomized, double-blind placebo controlled trial. Patients and methods. Seventeen patients in the remitting phase of the disease were randomly allocated to sham or magnetic therapy group and underwent iTBS over contralateral motor cortex of the most affected leg once a day for two weeks. Each session consisted of 10 bursts containing three pulses at 50 Hz repeated at 200 ms intervals (5 Hz) every 10 s for a total of 600 stimuli. The iTBS effect was assessed by using clinical (such as the Modified Ashworth Scale) and neurophysiological (H/M amplitude ratio and cortical silent period duration) parameters. Results. Two-week iTBS over motor cortex of the most affected leg did not produce any significant clinical effect on spasticity. However, it decreases the H/M amplitude ratio and increases duration of cortical silent period but not significantly, in patients with relapsing multiple sclerosis. Conclusion. The stimulation protocol used in this study does not have significant therapeutic effect. Therefore, we do recommend further studies as neurophysiological changes were evident.

Introduction: Acute pyelonephritis (APN) is one of the most common causes of serious bacterial infection in infants. Renal scarring is the most prevalent long-term complication. Objectives: To review the incidence of renal scarring within 6 months after an episode of APN in children under 36 months and its relationship with imaging studies, clinical settings, and bacteriology. Method: A retrospective study of previously healthy patients aged one to 36 months, admitted for a first episode of APN, with a minimum follow-up of 6 months. Demographic and clinical variables were collected along with bacteriology, renal and bladder ultrasound scan, voiding cystourethrography, DMSA-scintigraphy, and re-infection events. Results: A total of 125 patients were included in the study, of which 60% were male, the large majority (92%) febrile, and due to Escherichia coli (74.6%). There was a history of prenatal ultrasound scan changes in 15.4%. Ultrasound scan found dilation of the urinary tract in 22.1%. Voiding cystourethrography was performed on 70 patients: 54.3% no abnormalities, 12.8% vesicoureteral reflux (VUR) grade i-iii, and 32.9% iv-v grade VUR. Six patients had iv-v grade VUR with a normal ultrasound scan. Adherence to DMSA-scintigraphy at 6 months was only 61% of that indicated. Renal scarring was found in 44.3% of those in which it was performed (60 cases). Conclusions: Almost half (44%) DMSA-scintigraphy in children aged one to 36 months hospitalised for APN show renal scarring at 6 months, which was found to be associated with the re-infection events and the iv-v grade VUR. There was no relationship between scarring and the bacteriology or the elevations of inflammatory biochemical markers. Resumen: Introducción: Una de las causas más frecuentes de infección bacteriana grave en lactantes es la pielonefritis aguda (PNFA), cuya secuela más prevalente a largo plazo es la instauración de cicatrices renales. Objetivos: Revisar la incidencia de cicatrices renales a los 6 meses de un episodio de PNFA en niños menores de 36 meses y su relación con la clínica, las pruebas de imagen y la bacteriología. Método: Estudio retrospectivo de pacientes de uno a 36 meses previamente sanos ingresados por un primer episodio de PNFA, con un seguimiento mínimo de 6 meses. Se recogieron las variables demográficas junto con bacteriología, ecografía, cistourografía miccional seriada, recidivas y gammagrafía-DMSA. Resultados: Se incluyeron 125 pacientes, 60% varones, la mayoría febriles al ingreso (92%), debido a E. coli (74,6%). Existía antecedente de alteración ecográfica prenatal solo en el 15,4%. La ecografía detectó dilatación de la vía urinaria en el 22,1%. En 70 pacientes se indicó cistourografía miccional seriada: 54,3% sin anomalías, 12,8% con reflujo vesicoureteral (RVU) grado i-iii y 32,9% con RVU iv-v. Seis pacientes presentaban RVU iv-v con ecografías normales. La adherencia a la gammagrafía DMSA a los 6 meses fue solo del 61% de los indicados. De los efectuados (60 casos), en un 44,3% se hallaron cicatrices renales, relacionadas significativamente con la recurrencia y el RVU iv-v, pero no con la bacteriología ni con la elevación de reactantes. Conclusiones: El 44% de las gammagrafías-DMSA de pacientes de uno a 36 meses hospitalizados por PNFA desarrolla cicatrices renales a los 6 meses. Estas se relacionaron con las recurrencias y el RVU grave, pero no con la bacteriología y los reactantes inflamatorios.

Pertussis is a re-emerging disease that mostly affects infants. At this age, the severity can be affected by intercurrent infections such as respiratory syncytial virus (RSV).To estimate the incidence of RSV infection during an epidemic period in patients hospitalized due to pertussis. The impact on the severity was also observed during hospitalization.A descriptive study of cases diagnosed with pertussis admitted to a tertiary hospital over a 3year period, where the presence of co-infection with RSV was analyzed. The estimate of severity was estimated using the incidence of complications and the level of care required.From a total of 73 infants with pertussis, 34 occurred in a bronchiolitis season epidemic. A co-infection due to RSV was detected in 17 patients. The mean age was not significantly different compared to the non co-infected. The mean stay and the need for intensive care was similar in both groups. The need for oxygen therapy care and nutritional support was higher in the coinfected patients.Coinfection with RSV in infants hospitalized with pertussis occurred in ono in 2 patients during a RSV epidemic season, in infants of similar age. Severity in terms of stay, presence of apnea and admission to intensive care was similar, but more need for respiratory care and nutritional support was found.

Septo-optic dysplasia, which consists of the association of the hypoplasia of the optic nerves and the agenesis of the septum pellucidum, is frequently associated with deficiency of hypothalamic releasing factors. In Magnetic Resonance (MR) of these patients, anomalies in the form and size of the pituitary stalk, adenohypophysis and neurohypophysis are found. Some cases show schizencephaly and it has been proposed as an added component of the syndrome by some authors. This fact has been refuted by others.We present the clinical and neuroanatomic revision of six children with septo-optic dysplasia studied by MR imaging over the last five years in our Department of Neuropediatrics. The aim was, that through the neuroembryological discussion of the morphopathological aspects of the patients, to determine the malformation and the time in which the injury, which was the underlying cause, occurred.From the six cases, in two only disruptive signs were evident with the optic nerves being affected asymmetrically, disruption of the corpus callosum, falx cerebri indemnity and effects in the cortex conformation. This was opposed to the dysgenic features in the other four cases which had no disruptive features.Our findings suggest that this entity could be the result of at least two different pathogenic processes, that is, a minor form of holoprosencephaly (dysgenesis) or a disruption which, therefore, occurs later in gestation.

Sarcoidosis is a multisystemic granulomatous disease of unknown origin that often affects the lungs, eyes, and lymphatic system. Neurological manifestations are rare (5-6%). A case is reported of neurosarcoidosis that presented with anterior uveitis, lymph node enlargement, lymphocytic meningitis, cochleovestibular disease, and right peripheral facial nerve paralysis. Current diagnostic and therapeutic methods in this hard-to-diagnose entity are discussed.

Sciatic nerve paralysis is a rare entity in the newborn. Few reference in specialized tests indicate that in the majority of cases the sciatic palsy has been observed after misplaced injections into the buttocks. The prognosis is variable and appears to be better after umbilical vessel catheterization for injection of medications than after misplaced muscular injections. In case of recovery it takes place within 3 to 12 months.The objective of the present study is to know the evolution of neonatal sciatic palsy and to determine their injury noxe in regard to perinatal factors, and their relationship with long-time outcome, and to look for prognostic clues of clinical utility.We evaluated perinatal factors of newborn children with sciatic nerve paralysis, followed for more than 18 months of clinical evolution, in a neuropediatric centre.Twenty one newborn with such criteria were evaluated. Gestational age was within 32 and 42 weeks (median 38.2). The birth weight was between 2,100 and 4,100 g (median 2,973). The majority of cases obtained total recovery (16 of 21). The time of recovery was 4 to 14 months (median 8.8). Free ambulation was obtained by all cases (at 10 to 24 months). No apparent cause was observed in the majority of cases. Cesarean delivery was more frequent specially in cases with permanent consequences.Long-time prognostic of neonatal sciatic palsy is generally good. In our series all the cases with consequences were associated to cesarean delivery. The duration of cesarean intervention and the anesthesic hypotony of the newborn could be implicated in the sciatic nerve injury of poor outcome.

Optic neuritis is rare in childhood. Frequently (35-52% of all cases depending on the series) they have, during their clinical course, foci of demyelination leading to the clinical picture of multiple sclerosis (MS). Since 1993, the optic neuritis study group has recommended treatment with high doses of corticosteroids, since this seemed to stop progression, improve long-term results and delay the appearance of MS. The course of our patient was better than we expected.A 10 year old prepubertal girl complained of progressive loss of vision and slight pain in the right eye for 26 days before admission to hospital. On examination there was obvious papillitis of the right ocular fundus with total loss of the pupillary light reflex, together with consensual hyporeflexia of the left eye. Study of the visual evoked potentials (VEP) showed that there was marked delay of the P-100 wave, and a lower amplitude in the right eye. Magnetic resonance imaging did not show any demyelinated focus. Serological testing for neurotropic viruses was negative.After the initial phase of intravenous treatment (third day) there was subjective recovery of vision and the pupillary light reflex returned. VEP studies showed marked recovery. Thirty days after treatment was started there was almost complete subjective and VEP recovery. This rapid progress, as compared to that of other paediatric cases published, suggests a mechanism involving decompression of the optic nerve.

A 35-week new born with cerebral dysplasia (type II lissencephaly) and muscular dystrophy is reported. The central nervous system (CNS) findings were typical of those previously described in lissencephalic cerebromuscular disorders, and were characterized by global lissencephaly, hydrocephalus, and cerebral cortical dysplasia. The eyes appeared normal on examination and no encephalocele was found. The changes in the CNS in Walker-Warburg syndrome are similar to, but more severe than those found in Fukuyama congenital muscular dystrophy, and both represent a failure of constraint of neuronal migration. Whether the syndromes characterized by cerebro-ocular dysplasia and muscular dystrophy are genetically heterogeneous or allelic variations is controversial. Our case is a form of presentation between Fukuyama and Walker-Warburg syndrome in a family with fourth affected children.In our opinion, cases as the ours supports the theory that Fukuyama and Walker-Warburg could be genetically allelic.