[Temporal lobe epilepsy. Aetiological classification in 61 paediatric patients]. / Epilepsia del lóbulo temporal. Clasificación etiológica en 61 pacientes en edad pediátrica.

INTRODUCTION: There are very few studies on the aetiology of temporal lobe epilepsy (TLE) in childhood. The purpose of the present study is to analyse the data of 61 children diagnosed with TLE, in order to describe the aetiology of TLE in children seen in a neuropaedriatic clinic. We also discuss the currently proposed classification. PATIENTS AND METHODS: A retrospective analysis was carried out on patients diagnosed with TLE. Patients consisted of 61 children less than 15 years old. RESULTS: Patients were classified into three groups: Group 1 (symptomatic temporal lobe epilepsy) consisted of 25 patients (40.98 %) with any temporal lesion on neuroimaging (tumours, malformations or infections) or significant history; Group 2 (Mesial temporal sclerosis) consisted of 17 patients (27.86 %), a history of simple and complex febrile seizure were common in this group; and Group 3 (Cryptogenic epilepsy) consisted of 19 patients (31.15 %) with no abnormalities on neuroimaging or significant history. CONCLUSION: To our knowledge, this is the largest paediatric series of childhood new-onset TLE assessed only by MRI in the literature. We have modified the previous aetiological classification in order to make the groups more realistic.

Epilepsia del lóbulo temporal. Clasificación etiológica en 61 pacientes en edad pediátrica / Temporal lobe epilepsy. Aetiological classification in 61 paediatric patients

Introducción. La etiología de la epilepsia del lóbulo temporal (ELT) en la edad pediátrica se ha descrito en escasas ocasiones. El propósito de este estudio es analizar el diagnóstico etiológico de 61 pacientes con ELT atendidos en una consulta de neurología infantil. Analizamos y comentamos la clasificación actual. Pacientes y métodos. Se trata de un estudio de carácter retrospectivo, en el que se incluyó a 61 pacientes con ELT. Resultados. Los pacientes fueron clasificados en tres grupos en función del diagnóstico etiológico: grupo 1 (ELT sintomática), incluye a 25 pacientes (40,98 %) con lesiones en el lóbulo temporal (malformación, tumor o infección) o antecedentes significativos para presentar epilepsia; grupo 2 (esclerosis mesial temporal [EMT]), incluye a 17 pacientes (27,86 %), el antecedente de crisis febriles (tanto simples como complejas) se encontraba presente en un porcentaje elevado de pacientes con EMT; grupo 3 (ELT criptogénica), incluye a 19 pacientes (31,15 %), sin hallazgos patológicos en la resonancia magnética (RM) craneal o antecedentes significativos. Conclusión. Hasta la fecha, es la mayor serie que analiza, mediante RM, la etiología de la ELT de comienzo en la edad pediátrica. Hemos pretendido matizar la clasificación etiológica más aceptada, con el propósito de hacer grupos más flexibles y realistas

Introduction. There are very few studies on the aetiology of temporal lobe epilepsy (TLE) in childhood. The purpose of the present study is to analyse the data of 61 children diagnosed with TLE, in order to describe the aetiology of TLE in children seen in a neuropaedriatic clinic. We also discuss the currently proposed classification. Patients and methods. A retrospective analysis was carried out on patients diagnosed with TLE. Patients consisted of 61 children less than 15 years old. Results. Patients were classified into three groups: Group 1 (symptomatic temporal lobe epilepsy) consisted of 25 patients (40.98 %) with any temporal lesion on neuroimaging (tumours, malformations or infections) or significant history; Group 2 (Mesial temporal sclerosis) consisted of 17 patients (27.86 %), a history of simple and complex febrile seizure were common in this group; and Group 3 (Cryptogenic epilepsy) consisted of 19 patients (31.15 %) with no abnormalities on neuroimaging or significant history. Conclusion. To our knowledge, this is the largest paediatric series of childhood new-onset TLE assessed only by MRI in the literature. We have modified the previous aetiological classification in order to make the groups more realistic

[Intracranial venous thrombosis: reports on 11 paediatric cases]. / Trombosis venosa intracraneal:a propósito de 11 casos pediátricos.

INTRODUCTION: Intracranial venous thrombosis (IVT) is a rare condition at the paediatric age, with a wide variety of clinical features and causations. AIM: To describe the etiopathogenic factors, the presenting symptoms, diagnosis, treatment and progress of the cases of IVT diagnosed at the Hospital 12 de Octubre. PATIENTS AND METHODS: The descriptive retrospective study involved reviewing the records of patients who were admitted to hospital between 1989 and 2005, with ages ranging between 1 month and 16 years. Those who had been diagnosed as having IVT, confirmed by neuroimaging, were selected for the study. A review of their patient records was then carried out and associated etiopathogenic factors, clinical features and neuroimaging findings, treatment and progress were analysed. RESULTS: Eleven patients fulfilled clinical and radiological eligibility criteria. Most of them presented precipitating factors of an infectious origin (45%). The most frequent presenting symptoms were headache and diminished level of consciousness (45% for each case). The superior longitudinal was the most commonly affected venous sinus (72% of cases). Thrombophilic alterations were found in 27% of the patients. Magnetic resonance imaging of the brain was the most cost-effective diagnostic test. No haemorrhagic complications were recorded in patients receiving anti-clotting therapy. In most cases progress was favourable. CONCLUSIONS: Improved diagnostic methods allow IVT to be detected increasingly more often at the paediatric age. Anti-clotting therapy in the acute phase proved to be safe and effective in this series, although further studies are needed with patients at this age to confirm this finding.

Trombosis venosa intracraneal: a propósito de 11 casos pediátricos / Trombosis venosa intracraneal: a propósito de 11 casos pediátricos

La trombosis venosa intracraneal (TVI) constituye una patología rara en la edad pediátrica, congran variabilidad clínica y etiológica. Objetivo. Describir los factores etiopatogénicos, la presentación clínica, el diagnóstico, el tratamiento y la evolución de los casos diagnosticados de TVI en el Hospital 12 de Octubre. Pacientes y métodos. Estudiodescriptivo retrospectivo en el que se revisaron los informes de pacientes ingresados entre los años 1989 y 2005, con edades comprendidas entre 1 mes y 16 años. Se seleccionaron aquéllos diagnosticados de TVI confirmada con neuroimagen. Se procedió a una revisión de su historia clínica, analizando factores etiopatogénicos asociados, clínica y hallazgos en neuroimagen,tratamiento y evolución. Resultados. Once pacientes reunieron los criterios clínicos y radiológicos de inclusión. Lamayoría presentó factores desencadenantes de etiología infecciosa (45%). Los síntomas de presentación más frecuentes fueronla cefalea y la disminución del nivel de conciencia (un 45% cada una). El seno venoso más frecuentemente afectado fue el longitudinal superior en un 72% de los casos. Se hallaron alteraciones trombofílicas en el 27% de los pacientes. La resonanciamagnética cerebral fue la prueba de mayor rentabilidad diagnóstica. No se registraron complicaciones hemorrágicas en los pacientes anticoagulados. La evolución mayoritaria fue favorable. Conclusión. Se reconoce cada vez más la TVI en la edad pediátrica gracias a las mejoras en los métodos diagnósticos. La anticoagulación en la fase aguda ha resultado segura y eficaz en esta serie, aunque se precisan más estudios en esta edad para confirmarlo

Intracranial venous thrombosis (IVT) is a rare condition at the paediatric age, with a wide variety ofclinical features and causations. Aim. To describe the etiopathogenic factors, the presenting symptoms, diagnosis, treatment and progress of the cases of IVT diagnosed at the Hospital 12 de Octubre. Patients and methods. The descriptive retrospectivestudy involved reviewing the records of patients who were admitted to hospital between 1989 and 2005, with ages ranging between 1 month and 16 years. Those who had been diagnosed as having IVT, confirmed by neuroimaging, were selected for the study. A review of their patient records was then carried out and associated etiopathogenic factors, clinical features andneuroimaging findings, treatment and progress were analysed. Results. Eleven patients fulfilled clinical and radiological eligibility criteria. Most of them presented precipitating factors of an infectious origin (45%). The most frequent presentingsymptoms were headache and diminished level of consciousness (45% for each case). The superior longitudinal was the most commonly affected venous sinus (72% of cases). Thrombophilic alterations were found in 27% of the patients. Magneticresonance imaging of the brain was the most cost-effective diagnostic test. No haemorrhagic complications were recorded in patients receiving anti-clotting therapy. In most cases progress was favourable. Conclusions. Improved diagnostic methodsallow IVT to be detected increasingly more often at the paediatric age. Anti-clotting therapy in the acute phase proved to be safe and effective in this series, although further studies are needed with patients at this age to confirm this finding

[Cerebral palsy: the concept and population-based registers]. / Parálisis cerebral: concepto y registros de base poblacional.

INTRODUCTION: Cerebral palsy (CP) is the most common cause of motor disability in the paediatric age. For several decades, a number of developed countries have kept registers that have been used to conduct population-based studies of CP. In Spain, however, little attention has been paid to the epidemiology of CP. AIM: To review the concept of CP today and to stimulate greater interest in researching into CP based on the experience of other countries. DEVELOPMENT: The different definitions of CP cover motor sequelae secondary to an isolated brain injury that occurs in a developing brain. CP registers were started as a means of monitoring the prevalence of CP and planning suitable care for patients. Over the last decade projects have been carried out that involve the coordinated efforts of several centres; this provides a larger population for study and reduces the chances of confusion with respect to the terminology employed. In this regard, one notable line of work is that of the European CP group (SCPE), which gathers information about children with CP in 15 countries. Spain has been taking part in this project since 2003 through a group of researchers from the Hospital 12 de Octubre in Madrid. CONCLUSIONS: From the very definition of the disorder, CP is a complex condition. A population-based study of CP in Spain should help to arouse a renewed interest in this condition in our country.

Parálisis cerebral: concepto y registros de base poblacional / Cerebral palsy: the concept and population-based registers

La parálisis cerebral (PC) es la causa más frecuente de discapacidad motora en la edad pediátrica.Desde hace décadas, varios países desarrollados han establecido registros que han servido para estudiar la PC con base poblacional.En España, en cambio, se ha prestado escasa atención a la epidemiología de la PC. Objetivo. Revisar el conceptoactual de PC y suscitar el interés por la investigación en PC a partir de la experiencia de otros países. Desarrollo. Las distintas definiciones de PC engloban secuelas motoras secundarias a una lesión cerebral puntual que acontece en un cerebro en desarrollo. Los registros de PC nacieron con el objetivo de monitorizar la prevalencia de la PC y planificar de forma adecuadala asistencia a los pacientes. En la última década se han desarrollado proyectos coordinados entre varios centros, lo que proporciona una población de estudio mayor y minimiza la confusión terminológica. En esta línea, destaca el grupo europeo de PC (SCPE), que reúne información sobre niños con PC de 15 países. Desde el año 2003, España participa en este proyectoa través de un grupo de investigadores del Hospital 12 de Octubre de Madrid. Conclusiones. La PC es una realidad compleja desde su propia definición. Estudiar la PC con base poblacional en España debería contribuir a renovar el interés en nuestro medio por esta patología

Cerebral palsy (CP) is the most common cause of motor disability in the paediatric age. For severaldecades, a number of developed countries have kept registers that have been used to conduct population-based studies of CP.In Spain, however, little attention has been paid to the epidemiology of CP. Aim. To review the concept of CP today and to stimulate greater interest in researching into CP based on the experience of other countries. Development. The different definitions of CP cover motor sequelae secondary to an isolated brain injury that occurs in a developing brain. CP registerswere started as a means of monitoring the prevalence of CP and planning suitable care for patients. Over the last decade projects have been carried out that involve the coordinated efforts of several centres; this provides a larger population for study and reduces the chances of confusion with respect to the terminology employed. In this regard, one notable line of workis that of the European CP group (SCPE), which gathers information about children with CP in 15 countries. Spain has been taking part in this project since 2003 through a group of researchers from the Hospital 12 de Octubre in Madrid. Conclusions.From the very definition of the disorder, CP is a complex condition. A population-based study of CP in Spain should help to arouse a renewed interest in this condition in our country

[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

Calcificaciones distróficas cerebrales tras la curación de aspergilosis invasiva / Dystrophic calcification in the brain after successful treatment of invasive aspergillosis

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[Unilateral isolated paralysis of the soft palate: a case report and a review of the literature]. / Parálisis aislada unilateral del velo del paladar: presentación de un caso y revisión de la literatura.

INTRODUCTION: Unilateral isolated paralysis of the soft palate is a rare clinical entity. CASE REPORT: We describe the case of a 12 year old girl who presented acute dysphagia, a nasal voice and regurgitation of liquids into the nose. Exploration revealed right velopalatine insufficiency with normal gag reflex and pharyngeal sensitivity. All the complementary studies, including magnetic resonance, lumbar puncture and viral serology tests, were normal. There are 28 similar cases in the literature, with the following characteristics: acute onset, appearing in infancy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%). CONCLUSION: This is probably a case of acute cranial mononeuropathy with a viral aetiology

Parálisis aislada unilateral del velo del paladar: presentación de un caso y revisión de la literatura / Unilateral isolated paralysis of the soft palate: a case report and a review of the literature

Caso clínico. Paciente con epilepsia causada por una encefalitis límbica no relacionada con un proceso tumoral. Resultados. A lo largo de la evolución, y de forma subaguda, la paciente mostró crisis parciales y áreas variables de afectación de los lóbulos temporales, no simultáneas, que abocaron a una atrofia de las estructuras límbicas y áreas adyacentes y que dieron lugar a un estado de déficit neuropsicológico permanente, fundamentalmente en la memoria reciente verbal y visual. Tras 6 años de evolución, no se ha podido demostrar la coexistencia con un proceso neoplásico (AU)

Introduction. Unilateral isolated paralysis of the soft palate is a rare clinical entity. Case report. We describe the case of a 12-year-old girl who presented acute dysphagia, a nasal voice and regurgitation of liquids into the nose. Exploration revealed right velopalatine insufficiency with normal gag reflex and pharyngeal sensitivity. All the complementary studies, including magnetic resonance, lumbar puncture and viral serology tests, were normal. There are 28 similar cases in the literature, with the following characteristics: acute onset, appearing in infancy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%). Conclusion. This is probably a case of acute cranial mononeuropathy with a viral aetiology (AU)

[Neurological complications of systemic cancer and its treatment]. / Complicaciones neurológicas del cáncer sistémico y de su tratamiento.

INTRODUCTION: Therapeutic advances have attained excellent results in the battle against systemic cancer. This has meant cure for many and greater survival. However, these achievements have led to a dramatic increase in neurological complications due to the cancer itself and the surgical treatment on its own or combined with chemotherapy and radiotherapy. These complications, which are very common in childhood neoplasias, have had much less attention than those of adults, although recently excellent reviews have begun to be published. OBJECTIVE: In this paper we review the various complications of systemic cancer and its treatment in childhood, emphasizing the differences from that of adults.

Complicaciones neurológicas del cáncer sistémico y de su tratamiento / Neurological complications of systemic cancer and its treatment

Los avances terapéuticos han conseguido grandes éxitos en la lucha contra el cáncer sistémico, que se han traducido en la curación de muchos de ellos y en una mayor supervivencia. Sin embargo, estos logros han provocado un aumento dramático de las complicaciones neurológicas, debidas tanto al propio cáncer como al tratamiento sólo o combinado con quimioterapia y radioterapia. Estas complicaciones, que son muy comunes en las neoplasias de la infancia, han merecido una considerable menor atención que las de los adultos, aunque en los últimos años se han comenzado a publicar excelentes revisiones. En este trabajo se revisan las diversas complicaciones del cáncer sistémico y de su tratamiento en infancia, y se subrayan sus diferencias con respecto a las de los adultos (AU)

[Cerebral cavernomas in childhood. clinical presentation and diagnosis]. / Cavernomas cerebrales en la infancia. Presentación clínica y diagnóstico.

INTRODUCTION: Intracranial vascular malformations are congenital lesions due to alterations in the development of the arteriolocapillary network. Traditionally they are divided into four types according to their histological characteristics: arteriovenous malformations, cavernous angiomas or cavernomas, venous angiomas and telangectasias. The cavernomas are multilobulated lesions which are clearly delimited and contain blood at different stages of evolution. Histologically they are composed of sinusoidal spaces lined by endothelium and closely interlinked, without intervening nervous tissue. They are usually found at a supratentorial level, and less frequently in the posterior fossa. PATIENTS AND METHODS: We made a retrospective review of the histories of 11 patients aged between 2 and 10 years, diagnosed as having intracranial cavernomas in which imaging techniques lead to the diagnosis of intracranial cavernoma. In two patients there were multiple cavernomas. We analysed the method of diagnosis and clinical features on presentation. RESULTS: The clinical features were varied. In six patients the onset of the disorder was with epileptic seizures, three had progressive neurological deficits, one patient had headache and vomiting which progressed to coma and another had a history of non specific fainting and deficit. All but one of the cavernomas were supratentorial. CONCLUSIONS: Cavernomas are a type of vascular malformation with specific histological features. The usual clinical features are convulsions and parenchymatous bleeding. They are generally supratentorial. The appearance of MR has permitted diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for diagnosis.

Dieta cetogénica: fundamentos y resultados / The ketogenic diet: basics and results

Alrededor de un tercio de los pacientes con epilepsia no controlan satisfactoriamente las crisis, a pesar de recibir un tratamiento farmacológico adecuado. La dieta cetogénica (DC) se ha utilizado en el tratamiento de las epilepsias refractarias, sobre todo en niños, desde 1920. Su empleo cayó en desuso con la aparición de los fármacos anticomiciales hasta recientemente. Consiste, de manera esencial, en una dieta rica, en grasas y pobre en hidratos de carbono y proteínas, que se traduce en la aparición de cetosis y un mejor control de las crisis. Se desconoce el mecanismo último de acción de la dieta, aunque la mayoría de hipótesis indican la modificación del metabolismo cerebral, que varía de estar basado en la glucosa a depender de los cuerpos cetónicos. Las tres variantes más conocidas de la dieta se apoyan en este principio: la dieta cetogénica clásica, la más utilizada, proporciona 1 g de hidratos de carbono + proteínas por cada 3, 4 ó 5 g de grasa; la dieta con triglicéridos de cadena media (TCM) incorpora grandes cantidades de estos aceites y es peor tolerada, y la dieta cetogénica combinada, con TCM, consiste en el 30 por ciento del aporte energético como TCM, el 41 por ciento como rasas de la dieta el 10 por ciento proteínas y el 19 por ciento hidratos de carbono. Esta última es la empleada en nuestro Centro. Las complicaciones asociadas al uso de la dieta son, por lo general, de pequeña magnitud y bien toleradas. La instauración de la dieta ha de hacerse con el paciente ingresado y con la estrecha supervisión del dietista, que ajustará la dieta a las particularidades de cada niño, al mismo tiempo que educará a los padres en el control de la dieta y en la prevención de complicaciones. Desde 1998, 15 niños (7 niños y 8 niñas) con una mediana de edad de 3 años y medio (rango: 18 meses-9 años) han recibido la dieta. Todos nuestros pacientes habían sido diagnosticados de síndrome de Lennox-Gastaut; 7 de ellos con retraso mental profundo. A los 6 mese de iniciada la dieta, la mitad de los niños presentó una reducción en el número diario de crisis igual o superior al 50 por ciento. La mitad de ellos no tenía ninguna crisis o alguna muy ocasional. Tras 2 años, la mitad de los pacientes continuaba con la dieta, con una reducción en el número diario de crisis >90 por ciento. La dieta fue bien tolerada en todos los casos, manteniendo cetonuria superior a dos cruces en la mayoría de pacientes. Sólo se observaron efectos adversos. Conclusiones: La dieta cetogénica, generalmente en combinación con fármacos antiepilépticos, es eficaz en el control de la epilepsia refractaria de la infancia, sobre todo en pacientes diagnosticados de síndrome de Lennox-Gastaut. Hasta el 50 por ciento de los que comenzaron la dieta mantenía un buen control de las crisis al cabo de 2 años de tratamiento con la dieta. Su excelente tolerancia y los escasos efectos adversos animan al empleo de la dieta en los casos de difícil control (AU)

Cavernomas cerebrales en la infancia: presentación clínica y diagnóstico / Cerebral cavernomas in childhood. Clinical presentation and diagnosis

Introducción. Las malformaciones vasculares intracraneales son lesiones congénitas debidas a alteraciones en el desarrollo de la red arteriolocapilar. Tradicionalmente se dividen en cuatro tipos según sus características histológicas: malformaciones arteriovenosas, angiomas cavernosos o cavernomas, angiomas venosos y telangiectasias. Los cavernomas son lesiones multilobuladas, bien delimitadas, que contienen sangre en distintos estadios evolutivos. Histologicamente se componen de espacios sinusoidales tapizados por endotelio y unidos estrechamente entre sí, sin que exista tejido nervioso interpuesto. Generalmente se localizan en zona supratentorial y, con menos frecuencia, en fosa posterior. Pacientes y métodos. Se han revisado retrospectivamente las historias de 11 pacientes con edades comprendidas entre los 2 y los 10 años, diagnosticados de cavernomas intracraneales, en los que con la prueba de imagen se llegó al diagnóstico de cavernoma intracraneal, en dos de ellos de cavernomas múltiples. Se ha analizado el método diagnóstico y la clínica de presentación. Resultados. La clínica inicial fue variada: seis comenzaron con crisis epilépticas, tres con déficit neurológico progresivo, una paciente tuvo cefalea y vómitos que progresaron a coma, y otro tenía una historia de mareos inespecíficos. Todos, excepto uno, se localizaron en zona supratentorial. Conclusiones. Los cavernomas son un tipo de malformación vascular con unas características histológicas determinadas. Los síntomas clínicos habituales son convulsiones y hemorragia parenquimatosa. Generalmente se localizan en zona supratentorial. La aparición de la RM ha permitido el diagnóstico de cavernomas asintomáticos y actualmente se considera la técnica de elección para el diagnóstico (AU)

[Intracranial arachnoid cysts. A study of a series of 35 cases]. / Quistes aracnoideos intracraneales. Estudio de una serie de 35 casos.

INTRODUCTION: Arachnoid cysts are cavities with a content similar to cerebrospinal fluid, frequently communicating with the subarachnoid space. They make up 1% of the intracranial space occupying lesions, and although typically seen in children (13%), they may be undiagnosed until the patient has become adult. PATIENTS AND METHODS: We review a series of 35 cases of congenital intracranial arachnoid cysts in children, which had been investigated in the Neuropaediatric Department during 1987 1999. The patients had had cranial CAT and MR, and transcranial echography studies. RESULTS: In 85.7% there was a single cyst. The Sylvan fissure and posterior fossa were the commonest sites. The age of diagnosis was under 1 year in 54.3% and at birth in 25.7%. There were other associated malformations of the central nervous system in 31.4%, with agenesis of the corpus callosum in 45.5%. The most frequent clinical features on presentation of the condition were macrocephaly (31.4%) and epileptic crises (25.7%). The surgical treatment most often used was cystoperitoneal shunt. CONCLUSIONS: Congenital arachnoid cysts are caused by alteration in the embryogenesis of the central nervous system, and hence their association with other malformations and prevalence in children. The most frequent clinical findings were macrocephaly, due to the size of the cyst or the associated hydrocephaly, and epileptic seizures secondary to cortical irritation caused by pressure. Management of symptomatic lesions is surgical. The cystoperitoneal shunt was generally used in our series, since this was followed by clinical improvement in most cases.

[The return to the ketogenic diet. What role does it play in the treatment of refractory seizures of infancy?]. / La vuelta a la dieta cetogénica. ¿Qué papel desempeña en el tratamiento de las convulsiones infantiles refractarias?

UNLABELLED: About one third of the patients with epilepsy are not satisfactorily controlled in spite of correct anticonvulsive treatment. Although the ketogenic diet has been used for refractory epilepsy since the 1920s, over the past ten years it has been used much more. OBJECTIVE: To review the effectiveness, tolerance and adverse effects in 12 paediatric patients who have been on this diet for over three months. PATIENTS AND METHODS: We assessed 15 children, of whom only 12 are included in this review ( 5 boys and 7 girls). After initial joint evaluation by the neuropaediatrician and the Clinical Nutrition Unit the diet was started in hospital with a period of fasting (24 48 hours) until ketosis occurred. The diet was continued over three days before the child was sent home. In our centre we used a diet with modified MCT, in which 30% of the energy was given in the form of MCT and 40% as natural fats. The effectiveness of the diet was found by comparing the number of seizures suffered by the patient before starting the diet with the number at different times later (1, 3, 6, 12, 18 months). RESULTS: The median age when starting on the diet was 3 years and 5 months (range 18 months to 9 years). All had been diagnosed as having the Lennox Gastaut syndrome, six having cryptogenic disease. Six children had severe mental retardation. Six months after starting the diet, half the children had over 50% reduction in seizures whilst a third had no seizures at all or had their frequency of occurrence reduced by over 90%. After one year three families had given up the diet, two because of its inefficacy in controlling the seizures. The diet was well tolerated in all cases, with levels of ketonuria maintained at 2+. Mild adverse effects were seen in only three patients and transient rise in the plasma cholesterol level in four children. CONCLUSIONS: The ketogenic diet is still an effective treatment for epilepsy, especially in patients in whom the drugs available have not led to improvement. Its excellent tolerance and few short term side effects encourages its use in most cases of refractory epilepsy.