[Moyamoya disease: revision and description of a number of pedriatic cases]. / Enfermedad de moyamoya: descripción de una serie de casos pediátricos.

INTRODUCTION: Moyamoya disease is caused because of progressive occlusion of the arterial circle of Willis, leading to a compensatory net-like abnormal vessels development. The objective is to describe the number of cases in our center (tertiary hospital). PATIENTS AND METHODS: Retrospective study. Revision of pediatric medical histories diagnosed of moyamoya disease or moyamoya syndrome (in case of predisposing disease) between 2005 and 2018. Demographic variables were collected, related to diagnosis, risk factors, treatment, and follow-up. RESULTS: Seven cases were collected with a median age of 6 years and an equitable distribution by sex. Five associated predisposing pathologies (Down syndrome, neurofibromatosis, sickle cell disease, Behcet). The main clinical diagnosis was neurological focus (five cases), followed by epileptic seizures (four), and headache (two). One was asymptomatic at diagnosis. Six strokes were documented, five of them were isquemic. The arteriography (goldstardard) was made in five patients. Five presented bilateral involvement of the vessels, the internal carotid arteries and the middle cerebral arteries were the most affected. Six received acetylsalicylic acid treatment and five of them required antiepileptic drugs. Revascularization surgery (encephaloduroarteriomyosinangiosis) was performed in four patients, and in one, strokes persisted. The most prevalent sequelae were hemiparesis and psychomotor retardation. CONCLUSIONS: The risk factors presented in our patients match to those described in the literature. The symptoms at the onset can be diverse and ischemic strokes predominate in our series. Revascularization surgery was effective in more than half of the cases. Subsequent follow-up is necessary to assess complications and sequelae.

TITLE: Enfermedad de moyamoya: descripción de una serie de casos pediátricos.Introducción. La enfermedad de moyamoya se produce por la oclusión de las arterias alrededor del polígono de Willis y genera una amplia red de vasos colaterales. El objetivo es describir la serie histórica de nuestro centro (hospital terciario). Pacientes y métodos. Es un estudio retrospectivo. Se hizo una revisión de historias clínicas de pacientes pediátricos diagnosticados de enfermedad o síndrome de moyamoya (si patología predisponente) entre 2005 y 2018. Se recogieron variables demográficas, relacionadas con el diagnóstico, factores de riesgo, tratamiento y seguimiento. Resultados. Se obtuvieron siete casos, con una mediana de edad de seis años y distribución por sexos equitativa. Cinco asociaban patologías predisponentes (síndrome de Down, neurofibromatosis, drepanocitosis y Behçet). La clínica predominante en el diagnóstico fue focalidad neurológica (cinco casos), seguida de crisis epilépticas (cuatro) y cefalea (dos). Un paciente era asintomático en el momento del diagnóstico. Se documentaron seis ictus, cinco de los cuales fueron isquémicos. La arteriografía (técnica de referencia) constaba en cinco pacientes. Cinco presentaban afectación bilateral y estaban mayormente afectadas las arterias carótidas internas y las cerebrales medias. Seis recibieron tratamiento con ácido acetilsalicílico y cinco necesitaron fármacos antiepilépticos. La cirugía de revascularización (encefaloduroarteriomiosinangiosis) se realizó en cuatro pacientes y en uno persistieron los ictus. Las secuelas más prevalentes fueron hemiparesia y retraso psicomotor. Conclusiones. Los factores de riesgo presentados en nuestros pacientes se ajustan a los descritos en la bibliografía. La clínica en el inicio puede ser diversa y predominan los ictus isquémicos en nuestra serie. La cirugía de revascularización fue efectiva en más de la mitad de los casos. Es necesario un seguimiento posterior para evaluar complicaciones y secuelas.

[Megalencephalic leukoencephalopathy with cysts -the clinical importance in the genetic era]. / Leucoencefalopatía megalencefálica con quistes: importancia de la descripción clínica en la era genética.

INTRODUCTION: Megalencephalic leukoencephalopathy with cysts is a leukodystrophy of genetic origin that produces an alteration in the water and ion homeostasis in the brain, generating vacuolar forms and chronic oedema in the white matter with progressive neurological deterioration. It should be suspected in infants who present progressive macrocephaly during the first year of life, motor retardation and characteristic findings in magnetic resonance brain scans. CASE REPORT: We report the case of a girl who was followed up from the age of 9 months due to progressive macrocephaly and delayed psychomotor development and brain MRI findings consistent with megalencephalic leukoencephalopathy with cysts, and the appearance of epilepsy during its development. The usual genetic studies (new generation sequencing and array) were negative, but as the diagnostic criteria were met, a complementary messenger RNA and DNA study was conducted, which confirmed the presence of two pathogenic variants in MLC1. CONCLUSIONS: Megalencephalic leukoencephalopathy with cysts is a rare condition. Progressive macrocephaly in the first year of life, the absence of deterioration or slow deterioration, and the possibility of developing epilepsy, spasticity and ataxia are characteristic signs in its course. It is important for these patients to undergo an imaging test that shows findings that characterise this condition, which, together with the clinical features, makes it possible to differentiate it from other leukodystrophies and to establish a confirmatory diagnosis. Genetic studies can confirm the associated mutation that makes it possible to predict the clinicoradiological phenotype.

TITLE: Leucoencefalopatía megalencefálica con quistes: importancia de la descripción clínica en la era genética.Introducción. La leucoencefalopatía megalencefálica con quistes es una leucodistrofia de origen genético que produce una alteración de la homeostasis del agua e iones en el cerebro, generando formas vacuolares y edema crónico en la sustancia blanca con deterioro neurológico progresivo. Debe sospecharse en los lactantes que presentan macrocefalia progresiva durante el primer año de vida, retraso motor y hallazgos característicos en la resonancia magnética cerebral. Caso clínico. Niña en seguimiento desde los 9 meses por macrocefalia progresiva y retraso del desarrollo psicomotor con presencia en la resonancia magnética cerebral de hallazgos compatibles con leucoencefalopatía megalencefálica con quistes, y aparición de epilepsia en su evolución. Los estudios genéticos habituales (secuenciación de nueva generación y array) fueron negativos, pero, al cumplir los criterios diagnósticos, se procedió al estudio del ARN mensajero y el ADN complementario, que confirmó la presencia de dos variantes patogénicas en MLC1. Conclusiones. La leucoencefalopatía megalencefálica con quistes es una entidad infrecuente. Es característica la macrocefalia progresiva en el primer año de vida, la ausencia de deterioro o deterioro lento, y la posibilidad de desarrollar epilepsia, espasticidad y ataxia en su evolución. Cobra importancia en dichos pacientes la realización de una prueba de imagen que muestre hallazgos propios de la entidad, lo que, junto con la clínica, permite diferenciarla de otras leucodistrofias y establecer un diagnóstico confirmatorio. Los estudios genéticos pueden constatar la mutación asociada que posibilita predecir el fenotipo clinicorradiológico.

[HaNDL syndrome in a 12-year-old girl]. / Sindrome HaNDL en una niña de 12 años.

INTRODUCTION: HaNDL (headache and neurological deficits with cerebrospinal fluid lymphocytosis) syndrome is a self-limiting benign condition which, as its name suggests, causes episodes of transient neurological deficits of a motor, sensory, aphasic and, less frequently, visual nature lasting several hours, accompanied or followed by moderate-to-severe headache and lymphocytosis of the cerebrospinal fluid. Its incidence is low in adulthood, and it is extremely uncommon in the paediatric age. Recurrence of episodes usually occurs in the first three months. It has an excellent prognosis. CASE REPORT: A 12-year-old female with intense frontal headache that suddenly begins on the tenth day, with clinical signs and symptoms of dysarthria and right hemiparesis with subsequent aphasia and generalised hypotonia. After ruling out other conditions by means of complementary examinations and the clinical course, the patient was finally diagnosed with HaNDL syndrome, since it fulfilled the clinical criteria of the International Headache Society (IHS). CONCLUSIONS: The patient met the criteria established by the IHS for the diagnosis of HaNDL syndrome. The main historical, epidemiological and clinical data are analysed and the differential diagnosis of HaNDL syndrome in the paediatric age is described.

TITLE: Sindrome HaNDL en una niña de 12 años.Introduccion. El sindrome HaNDL (headache and neurologic deficits with cerebrospinal fluid lymphocytosis) es una entidad autolimitada y benigna que cursa, como su nombre indica, con aparicion de episodios de deficits neurologicos transitorios de naturaleza motora, sensitiva o afasica, y menos frecuentemente visual, de varias horas de duracion, acompañados o seguidos de cefalea moderada-grave y linfocitosis del liquido cefalorraquideo. Su incidencia es baja en la edad adulta, y casi excepcional en la edad pediatrica. La recurrencia de los episodios suele producirse en los primeros tres meses. El pronostico es excelente. Caso clinico. Mujer de 12 años con cefalea frontal intensa que inicia en el decimo dia, de forma brusca, clinica de disartria y hemiparesia derecha con posterior afasia e hipotonia generalizada. Tras descartarse mediante exploraciones complementarias y evolucion clinica otras entidades, fue finalmente diagnosticada de sindrome HaNDL, ya que cumplia los criterios clinicos de la Sociedad Internacional de Cefaleas (IHS). Conclusiones. La paciente reunio los criterios de la IHS para el diagnostico de sindrome HaNDL. Se analizan los principales datos historicos, epidemiologicos y clinicos, y se expone el diagnostico diferencial del sindrome HaNDL en la edad pediatrica.

[Sleep disturbances among children and adolescents with learning disabilities. Comparative study between students from a mainstream school and a special school in the Valencia Community (Spain)]. / Trastornos del sueño en niños y adolescentes con incapacidad psíquica. Análisis comparativo entre alumnos escolarizados en centros ordinarios y centros de educación especial de la Comunidad Valenciana.

INTRODUCTION: Sleep disturbances affect daytime behaviour and cognitive functions in children. These disorders make the situation worse in children who already have learning difficulties. The objective of the present study was to study the prevalence of sleep disturbances among children from special schools and carry out a comparative study with children from mainstream schools. PATIENTS AND METHODS: We use the Spanish version of the Paediatric Sleep Questionnaire, which was given out in three special schools of the Valencia Community. Results were compared with a previous study in children from mainstream schools. RESULTS: A significantly higher proportion of children in special schools had insomnia, 32.3 % against 10.5 % in main-stream school, and sleep breathing disorders, 26.8 % against 5.7 % in mainstream schools, and a lower proportion of parasomnias. Among the whole population with learning difficulties, children with cerebral palsy are the most affected. CONCLUSIONS: Our results clearly show that children with learning difficulties have a higher risk of sleep disturbances and these disturbances have repercussions on daily functioning. It is important to rule out sleep disturbances when we see children with learning difficulties.

[Sleep patterns and sleep disturbances among schoolchildren in the town of Gandia]. / Patrones normales y trastornos del sueño entre la población escolar de la ciudad de Gandía.

INTRODUCTION: Knowledge of sleep patterns and sleep disturbances among healthy children is interesting, particularly, amongst other things, for carrying out comparative studies with children with certain diseases. The objective of the present study was to study sleep patterns and the prevalence of sleep disturbances among schoolchildren. METHODS: We used the Spanish version of the Pediatric Sleep Questionnaire, which was given out to a representative sample of Gandia Town. RESULTS: The prevalence of sleep disturbances were as follows: insomnia 10.5 %, daytime sleepiness 13 %, a positive score for sleep-disorder breathing 5.7 %, snoring 5.7 %, enuresis 5.3 %, sleepwalking 12.5 %, night terrors 18.4 %, nightmares 12.8 %. As regards sleep patterns, the average time for getting up was 7:45 am, the average time for going to bed was 22:13 pm, and the average sleep duration was 9 hours and 30 minutes. CONCLUSIONS: Comparison among different populations studies is difficult due to the use of different instruments to measure the same variables. Our results are similar to other studies, with the exception of sleep-disorder breathing and snoring, where the prevalences are lower in our study. The sleep patterns also did not show any differences between other published studies.

[Attention-deficit/hyperactivity disorder and sleep disturbances. Results of an epidemiological study in schoolchildren in Gandia, Spain]. / Relación entre el trastorno por déficit de atención e hiperactividad y los trastornos del sueño. Resultados de un estudio epidemiológico en la población escolar de la ciudad de Gandía.

INTRODUCTION: The evaluation of comorbidity is an important factor in the treatment of children with attention-deficit/hyperactivity disorder. Sleep disturbances are one of the most common features of this disorder. OBJECTIVE: To find out the frequency of sleep disturbances among children with attention-deficit/hyperactivity disorder. MATERIAL AND METHODS: We used the Spanish version of the Paediatric Sleep Questionnaire, which was given out to a representative sample in Gandia (Spain). RESULTS: The questionnaire was answered by 887 (68 % of the sample). The sleep disturbances that showed a clear relationship with the three ADHD variables studied are: snoring, enuresis, rhythmic movement disturbances, night awakenings, and bedtime resistance. Sleep-disorder breathing, bruxism, somniloquy, day sleepiness, early awakenings and difficulty falling sleep are associated with only one of the three variables studied. CONCLUSIONS: Our study demonstrates that the population with attention-deficit/hyperactivity disorder has more sleep disturbances. The association with the enuresis is of particular interest due to the possible clinical implications.

[Spanish version of the Pediatric Sleep Questionnaire (PSQ). A useful instrument in investigation of sleep disturbances in childhood. Reliability analysis]. / Versión española del Pediatric Sleep Questionnaire. Un instrumento útil en la investigación de los trastornos del sueño en la infancia. Análisis de su fiabilidad.

INTRODUCTION: Sleep questionnaires are useful tools both for screening patients who require medical tests and for epidemiologic research. The Pediatric Sleep Questionnaire (PSQ) has two versions: a shorter one, which has been validated for sleep-related breathing disorders, and an extended version, which deals with a wider range of sleep disturbances. MATERIAL AND METHODS: The PSQ was translated into Spanish and its reliability was determined by means of test-retest and internal consistency methods among a random sample of 99 patients. RESULTS: Ninety-one percent of the questions showed good concordance, following the criteria established in the test- retest method. Internal consistency was good in parts A (nighttime and sleep behavior) and C (items for inattention and hyperactivity) of the questionnaire but was weak in part B (daytime behavior and other possible problems). Therefore, the reliability of the questionnaire can be considered to be adequate except for the internal consistency of part B. CONCLUSIONS: The Spanish version of the PSQ is a suitable tool both for screening patients who require medical tests and for epidemiological research.

Enfermedad de moyamoya: descripción de una serie de casos pediátricos / Moyamoya disease: revision and description of a number of pedriatic cases

Introducción: La enfermedad de moyamoya se produce por la oclusión de las arterias alrededor del polígono de Willis y genera una amplia red de vasos colaterales. El objetivo es describir la serie histórica de nuestro centro (hospital terciario). Pacientes y métodos: Es un estudio retrospectivo. Se hizo una revisión de historias clínicas de pacientes pediátricos diagnosticados de enfermedad o síndrome de moyamoya (si patología predisponente) entre 2005 y 2018. Se recogieron variables demográficas, relacionadas con el diagnóstico, factores de riesgo, tratamiento y seguimiento. Resultados: Se obtuvieron siete casos, con una mediana de edad de seis años y distribución por sexos equitativa. Cinco asociaban patologías predisponentes (síndrome de Down, neurofibromatosis, drepanocitosis y Behçet). La clínica predominante en el diagnóstico fue focalidad neurológica (cinco casos), seguida de crisis epilépticas (cuatro) y cefalea (dos). Un paciente era asintomático en el momento del diagnóstico. Se documentaron seis ictus, cinco de los cuales fueron isquémicos. La arteriografía (técnica de referencia) constaba en cinco pacientes. Cinco presentaban afectación bilateral y estaban mayormente afectadas las arterias carótidas internas y las cerebrales medias. Seis recibieron tratamiento con ácido acetilsalicílico y cinco necesitaron fármacos antiepilépticos. La cirugía de revascularización (encefaloduroarteriomiosinangiosis) se realizó en cuatro pacientes y en uno persistieron los ictus. Las secuelas más prevalentes fueron hemiparesia y retraso psicomotor. Conclusiones: Los factores de riesgo presentados en nuestros pacientes se ajustan a los descritos en la bibliografía. La clínica en el inicio puede ser diversa y predominan los ictus isquémicos en nuestra serie. La cirugía de revascularización fue efectiva en más de la mitad de los casos. Es necesario un seguimiento posterior para evaluar complicaciones y secuelas.(AU)

Introduction: Moyamoya disease is caused because of progressive occlusion of the arterial circle of Willis, leading to a compensatory net-like abnormal vessels development. The objective is to describe the number of cases in our center (tertiary hospital). Patients and methods: Retrospective study. Revision of pediatric medical histories diagnosed of moyamoya disease or moyamoya syndrome (in case of predisposing disease) between 2005 and 2018. Demographic variables were collected, related to diagnosis, risk factors, treatment, and follow-up. Results: Seven cases were collected with a median age of 6 years and an equitable distribution by sex. Five associated predisposing pathologies (Down syndrome, neurofibromatosis, sickle cell disease, Behçet). The main clinical diagnosis was neurological focus (five cases), followed by epileptic seizures (four), and headache (two). One was asymptomatic at diagnosis. Six strokes were documented, five of them were isquemic. The arteriography (goldstardard) was made in five patients. Five presented bilateral involvement of the vessels, the internal carotid arteries and the middle cerebral arteries were the most affected. Six received acetylsalicylic acid treatment and five of them required antiepileptic drugs. Revascularization surgery (encephaloduroarteriomyosinangiosis) was performed in four patients, and in one, strokes persisted. The most prevalent sequelae were hemiparesis and psychomotor retardation. Conclusions: The risk factors presented in our patients match to those described in the literature. The symptoms at the onset can be diverse and ischemic strokes predominate in our series. Revascularization surgery was effective in more than half of the cases. Subsequent follow-up is necessary to assess complications and sequelae.(AU)

[Variability in the treatment of headaches in visits to the Neuropaediatrics service at six Spanish hospitals]. / Variabilidad en el tratamiento de las cefaleas en las consultas de Neuropediatría de seis hospitales españoles.

INTRODUCTION: Headache is one of the chief reasons for visiting Neuropaediatric units. Variability in clinical practice has been defined as variations in the rates a clinical procedure is employed. The aim of this work is to study the variability in the treatment of children's headaches in six Spanish hospitals. PATIENTS AND METHODS: We conducted a retrospective study involving patients who first visited because of a headache some time in the year 1999. Data were collected in December 2003. The following variables were analysed: type of headache, neuroimaging scans, beginning of prophylaxis, follow-up visits to clinic and number of patients who dropped out of the follow-up. RESULTS: In all, 372 children were attended. 63.4% of the headaches were considered as being migraine-type; 43% of the patients were submitted to neuroimaging and prophylaxis was established in 36.8%. Mean follow-up time in the clinic was 10.6 months and the mean number of visits was 3. 30% had dropped out of the follow-up. In the comparative analysis, significant differences were found in all the variables studied. CONCLUSIONS: Overall results are similar to those found in other series. The differences found between centres can be accounted for by differences in population, differences in health professionals' styles of medical practice or by the nonexistence of clear guidelines regarding a particular procedure.

[Neuropediatric practice in Spanish hospitals: supply and demand]. / Actividad asistencial neuropediátrica en los hospitales españoles: la oferta y la demanda.

BACKGROUND: No reports have been published of country-wide neuropediatric practice. OBJECTIVE: To determine neuropediatric practice in Spanish hospitals by studying supply and demand throughout the country. MATERIALS AND METHODS: A survey was performed of Spanish hospitals in 2001. Private hospitals with no state funding and hospitals without a pediatric unit were excluded. The parameter used to study supply was the existence of neuropediatric beds and that used to study demand was the annual consultation rate per 1000 inhabitants aged from 0 to 14 years. RESULTS: A questionnaire was sent to 277 hospitals and 106 (38.27 %) responded. Sixty percent of Spanish hospitals had neuropediatric beds. The mean neuropediatric consultation rate was 31 consultations per 1000 inhabitants aged from 0 to 14 years. CONCLUSIONS: In Spain, the supply of neuropediatric services is fairly widespread. The greater the number of pediatric beds, the higher the probability of neuropediatric beds. However, 42 % of small hospitals provides this service. In contrast with previously reported results on morbidity in primary care, our results show that most children with neurological processes are referred to hospital.

[Hypernatremic dehydration associated with breast-feeding]. / Deshidratación hipernatrémica asociada a lactancia materna.

BACKGROUND: During the last 10 years the number of publications reporting a rise in the incidence of hypernatremia in children who are exclusively breast-fed has increased. OBJECTIVE: To report the results of a series of cases detected in a district hospital in the last 6 years in order to better delineate the clinical characteristics of infant hypernatremia, its optimal treatment and preventive measures. MATERIAL AND METHODS: The selection criteria for our study included all neonates aged less than 30 days with serum sodium concentrations equal to or greater than 150 mEq/mL who were exclusively breast fed. Cases were identified through the departmental database. RESULTS: Twelve newborns were identified who presented the following characteristics: mean gestational age of 39.5 weeks, mean birth weight of 3440 grams, mean percentage of weight loss from birth 13.7 % and mean serum sodium value 152.75 mEg/mL. Five neonates (42 %) were admitted from the maternity unit and seven (58 %) from the emergency department. The presenting complaints included weight loss in nine infants, irritability in six, fever in six, poor feeding in five and jaundice in two. Most of the neonates had more than one symptom. Ten of the mothers (83.33 %) were primiparous and two (16.67 %) were secundiparous. CONCLUSIONS: Hypernatremia due to breast-feeding is a consequence of inadequate breast-feeding technique in inexperienced mothers. A possible solution would be for health professionals to provide mothers with greater support.

[Diagnostic yield in studies of autism. The contribution made by neuroimaging, metabolic tests and genetic studies]. / Rendimiento del estudio diagnostico del autismo. La aportacion de la neuroimagen, las pruebas metabolicas y los estudios geneticos.

AIMS: We reviewed the literature on the diagnostic yield of the different medical tests carried out on autistic patients in order to determine the aetiology of the process. DEVELOPMENT: Although it is generally said that a causation of the process can be determined in 10 30% of cases, the actual diagnostic yield of autism when a routine diagnosis is applied is between 2 and 7%. The diagnostic yield of radiology, metabolic studies and genetic studies was analysed. Genetic tests are the ones that offer the highest efficiency, and carrying out a karyotype and molecular study of fragile X chromosome syndrome is indicated in all patients with mental retardation associated to autism. The next most efficient are metabolic studies, which become particularly important mainly in populations where no screening has previously been conducted for metabolic disorders. CONCLUSIONS: Despite their being capable of showing up a large number of disorders, neuroimaging studies are unspecific and the diagnostic yield of radiological studies is very low when they are conducted on a routine basis.

[Schizencephaly associated with porencephaly in a girl with congenital cytomegalovirus infection]. / Esquisencefalia acompañada de porencefalia en una niña con infección congénita por citomegalovirus.

INTRODUCTION: At the present time it is perfectly clear that schizencephaly is an anomaly of neuronal migration and porencephaly is the result of destruction of the cerebral cortex. Schizencephaly is the final result of a variety of lesions which occur during a critical period of the development of the brain. Imaging studies have shown many cerebral anomalies varying from lissencephaly to multicystic encephalomalacia. We report the case of a girl in whom porencephaly was associated with schizencephaly and in whom angioresonance showed hypoplasia of the right middle cerebral artery. CLINICAL CASE: The parents of an eight month old girl consulted us after observing that she had reduced movement of her left arm. On clinical examination there was left hemiparesia. Both CAT and MR showed closed lip schizencephaly of the left hemisphere and an area of right frontoparietal encephalomalacia. Study of the vascular structures showed underdevelopment of the right middle cerebral artery. CONCLUSIONS: The cytomegalovirus has been considered to be the principal infectious agent involved in the aetiology of different disorders of neuronal migration. It has been reported that the cytomegalovirus causes vasculitis which leads to reduction in perfusion and lesions due to secondary encephalomalacia. The fact that our patient had a hypoplastic middle cerebral artery, and a normal left middle cerebral artery, may be explained by subsequent recanalization of the middle cerebral artery. This would seem to indicate that the lesion causing schizencephaly is older and there has been time for recanalization.

[Epidemiology of progressive intellectual and neurological deterioration in childhood. A multicentre study in the Community of Valencia]. / Epidemiología del deterioro intelectual y neurológico progresivo en la infancia. Estudio multicéntrico en la Comunidad Valenciana.

BACKGROUND: Progressive intellectual and neurological deterioration (PIND) in childhood has an incidence of 0.5 per thousand live births. Its impact on morbimortality is important. The aim of this work is to study the epidemiology of PIND and the most important causes of this process. METHODS: A retrospective and multicentre study was carried out in different hospitals of the Valencian Community (Spain). CASE DEFINITION: age less than 18 years with a progressive loss of intellectual or development abilities, previously acquired for at least 3 months. RESULTS: Nine hospitals participated in the study and a total of 85 cases were included. The mean age at the onset of symptoms was 1.8 years, and the mean age at diagnosis was 3.6 years. In 68% of cases there was an intellectual or developmental delay before the onset of symptoms. The mortality rate was 16.4%. A diagnosis was achieved in 74% of the cases. The most frequently found cause was Rett syndrome. More than two-thirds (68%) of diagnosed cases were due to a metabolic disease. DISCUSSION: The existence of a previous development delay and the early onset of the PIND can make it difficult to identify. Like the rest of the series, the largest number of diagnosed cases involved a metabolic disease. CONCLUSIONS: PIND has an early onset, and in a large percentage of cases the origin remains unknown and with a high mortality rate.

[Encephalopathy due to human herpesvirus 6 in a western immunocompetent patient]. / Encefalopatia por virus herpes humano de tipo 6 en una paciente inmunocompetente occidental.

TITLE: Encefalopatia por virus herpes humano de tipo 6 en una paciente inmunocompetente occidental.

[Cerebrovascular disease in childhood: a retrospective analysis of hospital admissions in a tertiary hospital in the community of Valencia in the last ten years]. / Enfermedad cerebrovascular en la infancia. Análisis retrospectivo de los ingresos hospitalarios en los últimos diez años en un hospital terciario de la Comunidad Valenciana.

INTRODUCTION: There has been increasing interest in stroke in children in the last few years. A literature review produced little information on risk factors and other clinical questions. The aim of this study is to describe the characteristics of stroke in children, mainly in order to identify the risk factors, clinical presentation and outcomes. PATIENTS AND METHODS: A retrospective study was conducted on patients admitted to the Hospital La Fe in Valencia between January 2000 to September 2010 with the diagnosis of ischaemic or haemorrhagic stroke. RESULTS: A total of 76 patients were identified, of whom 44.7% had an ischaemic stroke and 55.3% had a haemorrhagic one. The average age of presentation was 6.8 years; 8.4 years for haemorrhagic strokes and 4.7 years for ischaemic strokes. Headache was the most frequent symptom of presentation. The most frequent risk factor was vascular malformations in haemorrhagic cerebral stroke, and vascular and cardiac disorders in ischaemic stroke. A study of prothrombotic factors was conducted on 34 patients, which was positive in 64.7% of them. As regards outcome, 17% of the patients died; only 3 patients had a secondary epilepsy, and 31% and 60% of the haemorrhagic and ischaemic stokes, respectively, had a hemiparesis. CONCLUSIONS: In this study we identified the principal risk factors as well as, the age of presentation, symptomatology and outcome. We would like to emphasise that the age of presentation was earlier in ischaemic strokes than in haemorrhagic ones.

[Methylphenidate and sleep: Results of a multicentre study on a population of children with attention deficit hyperactivity disorder]. / Efecto del metilfenidato sobre el sueño. Resultados de un estudio multicéntrico en una población infantil afecta de trastorno por déficit de atención e hiperactividad.

INTRODUCTION: The attention-deficit/hyperactivity disorder (ADHD) is the most common neuro-behavioural disorder in children and adolescents. Methylphenidate (MPH) is the drug most often used in the treatment of this disorder. It is important to know the effects methylphenidate can have on sleep due to the repercussions that insufficient sleep can have on the behaviour and cognitive function of children and adolescents. OBJECTIVES: The purpose of this study was to find out the repercussions that methylphenidate can have on sleep, using a questionnaire. METHODOLOGY: This is a multicentre study in which six hospitals in the Valencian Community took part. All those patients who were diagnosed with ADHD over a period of 6 months were given a questionnaire, before starting treatment, and after three months of this treatment. The questionnaire was a version of the Paediatric Sleep Questionnaire. The differences found were analysed. RESULTS: A total of 114 children were studied. Significant differences were found in the questions associated with enuresis, somnambulism and night-wakings, these sleep disorders decreasing after starting the treatment. The rest of variables did not show any changes. CONCLUSIONS: According to our results we can say that the MPH not only does not make worse the sleep, but that it improves the quality of the sleep in those patients with sleep disorders.