Visuospatial functions in preterm schoolchildren without cognitive delay: Using Pascual's Graphomotor test as a screening method.

BACKGROUND: Preterm children obtain worse scores in tests that evaluate visuospatial functions. Pascual's graphomotor test (PGMt) assesses maturity in copying drawings in childhood, quickly evaluating the graphomotor aptitude that is a partial aspect of non-verbal intelligence. AIMS: To evaluate visuospatial functions in preterm children compared to full-term children. To assess the capacity of the Pascual graphomotor test (PGMt) to detect visuospatial disorders more specifically than non-verbal intelligence quotient (IQ). STUDY DESIGN AND SUBJECTS: case and control study. CASES: preterm children between 5 and 11 years of age without cognitive delay; controls: full-term children with the same characteristics. For each child clinical history, neurological examination, language-free intelligence test Toni 2 (IQ) and Pascual's graphomotor test (PGMt) were carried out. RESULTS: 135 children were enrolled (59 cases vs. 79 controls). The mean age was 7.4 years. 55% were male. The mean gestational age of cases was 30.5 weeks with 34% extremely preterm. Cases obtained worse mean scores in both tests. The mean IQ scores were: cases 117.4, controls 125.0 (p = 0.004). The mean graphomotor quotient (GQ) scores were statistically and clinically significant (cases 76.8; controls 98.3, p = 0.001). Although we have found a positive correlation between IQ and GQ scores (cc = 0.31 p = 0.01), the differences found in the GQ between groups have been maintained regardless of the IQ in the multivariate analysis (GQ: cases 78.3 (SD 14.8), controls 98.3 (SD 12.5), p = 0.04). CONCLUSIONS: GQ is a useful tool for screening for visuospatial anomalies. GQ more specifically measures the visuoperceptive disorder regardless of non-verbal cognitive level.

Patrón punta-onda continua en el sueño lento: nuestra experiencia durante 20 años / Continuous spike-waves during slow-wave sleep: Experience during 20 years

Introducción: La punta-onda continua durante el sueño lento (POCS) es un trazado electroencefalográfico característico, que aparece en la infancia y que en ocasiones condiciona un deterioro cognitivo. Este patrón electroencefalográfico puede aparecer tanto en determinados síndromes epilépticos como en la evolución de epilepsias idiopáticas y sintomáticas. El objetivo de nuestro estudio es analizar las características epidemiológicas y clínicas de pacientes que presentan en algún momento de su evolución POCS, describir la respuesta a distintos tratamientos y estudiar factores predictores de su evolución. Métodos: Estudio observacional retrospectivo de pacientes pediátricos con POCS seguidos en un hospital terciario en el periodo de noviembre 1997 a noviembre 2017. Resultados: Obtuvimos una muestra de 25 pacientes (68% varones). El 76% presentaba de base alteraciones en pruebas de neuroimagen o retraso psicomotor (POCS secundaria). El 24% restante eran niños sanos o con epilepsias benignas (POCS primaria). La edad media al inicio de la POCS fue de 6,7 años, siendo menor en los casos secundarios. Durante el periodo de POCS, el 72% presentó alguna manifestación clínica añadida. Todos recibieron combinaciones de antiepilépticos, siendo eficaces en el 36%. La POCS cesó en el 72%, siendo más probable el cese cuanto más tarde se hubiera instaurado. Un tercio tuvo alguna secuela, principalmente alteraciones cognitivas y del comportamiento, más frecuentes en POCS secundaria y en los pacientes en que duró más tiempo. Conclusiones: El trazado electroencefalográfico de POCS, aunque infrecuente, supone un reto terapéutico. Es importante seguir a los pacientes con epilepsia, especialmente si asocia deterioro neurológico, para detectar la presencia POCS e iniciar tratamiento precoz

Introduction: Continuous spikes and waves during slow sleep (CSWS) is an EEG pattern that appears during childhood, and is often associated with cognitive impairment. It can appear in the course of epileptic syndromes, as well as in benign epilepsy. The aim of this study is to analyse epidemiological and clinical characteristic of patients with CSWS, in order to describe possible predictive factors in their outcome. Methods: A retrospective study was conducted on paediatric patients with CSWS treated in a third-level hospital from November 1997 to November 2017. Results: The study included 25 patients (68% male), of whom 76% had abnormalities in the neuroimaging or suffered from psychomotor development disorder (secondary CSWS). The rest were healthy, or diagnosed with idiopathic epilepsy. The mean age of onset of CSWS was 6.7 years, but earlier in the secondary CSWS cases. Symptoms were present during the CSWS episode in 72% of cases. All of them were treated with antiepileptic drugs, which were effective in 36%. CSWS stopped in 72%, and remission was longer if the CSWS onset occurred at an older age. One-third (33%) presented with sequelae, mostly cognitive and behavioural alterations. Outcome was poorer in those with secondary CSWS and, in those whose CSWS started at an earlier age and lasted longer. Conclusion: The CSWS pattern, although rare, is still a therapeutic challenge. A close follow-up of the patients with epilepsy is important, especially if associated with cognitive impairment, in order to establish an early diagnosis and treatment

[Continuous spike-waves during slow-wave sleep: Experience during 20 years]. / Patrón punta-onda continua en el sueño lento: nuestra experiencia durante 20 años.

INTRODUCTION: Continuous spikes and waves during slow sleep (CSWS) is an EEG pattern that appears during childhood, and is often associated with cognitive impairment. It can appear in the course of epileptic syndromes, as well as in benign epilepsy. The aim of this study is to analyse epidemiological and clinical characteristic of patients with CSWS, in order to describe possible predictive factors in their outcome. METHODS: A retrospective study was conducted on paediatric patients with CSWS treated in a third-level hospital from November 1997 to November 2017. RESULTS: The study included 25 patients (68% male), of whom 76% had abnormalities in the neuroimaging or suffered from psychomotor development disorder (secondary CSWS). The rest were healthy, or diagnosed with idiopathic epilepsy. The mean age of onset of CSWS was 6.7 years, but earlier in the secondary CSWS cases. Symptoms were present during the CSWS episode in 72% of cases. All of them were treated with antiepileptic drugs, which were effective in 36%. CSWS stopped in 72%, and remission was longer if the CSWS onset occurred at an older age. One-third (33%) presented with sequelae, mostly cognitive and behavioural alterations. Outcome was poorer in those with secondary CSWS and, in those whose CSWS started at an earlier age and lasted longer. CONCLUSION: The CSWS pattern, although rare, is still a therapeutic challenge. A close follow-up of the patients with epilepsy is important, especially if associated with cognitive impairment, in order to establish an early diagnosis and treatment.

Síndrome de shock tóxico en una unidad de cuidados intensivos pediátricos en los últimos 15 años / Toxic shock syndrome in a paediatric intensive care unit over the last 15 years

No disponible

Outcome of arterial ischemic stroke in children with heart disease.

Arterial ischemic strokes (AIS) are rare in childhood. Congenital and acquired heart diseases are one of the most important risk factors of AIS in children. OBJECTIVE: Study the outcome of children with heart disease that have suffered AIS and the factors that influence on prognosis. PATIENTS AND METHODS: We evaluated all children with heart disease who had suffered AIS between 2000 and 2014 in our hospital. RESULTS: Seventy-four children with heart disease suffered an arterial ischemic stroke. 20% of them died and 10% had new AIS during the study period. Fifty-two patients were evaluated an average of six years after AIS. According to the Paediatric Stroke Outcome Scale (PSOM), most of the patients had some degree of impairment, mainly in sensorimotor and in cognitive-behavioural areas. The modified Rankin scale (mRS) showed an unfavourable outcome in 70% of patients (including patients that have died). Upper limb was more functionally impaired than lower limb. Strokes in neonatal period and early life were associated with poor prognosis. Size of stroke, cortical and subcortical involvement and basal ganglia stroke were associated with an unfavourable outcome. Fever in the acute phase and hemiparesis at presentation were also poor prognostic factors. Epilepsy at time of evaluation was also associated with unfavourable outcome. On the other hand, a normal electroencephalogram was associated with favourable outcome. CONCLUSIONS: AIS in children with heart disease had an unfavourable outcome, with impairment in different areas. Epilepsy happened in one third of the patients.

Neurological complications after gamma-knife radiosurgery for hypothalamic hamartoma.

BACKGROUND: The Gamma-knife technique is a safe and effective option for the treatment of hypothalamic hamartomas that produce epileptic seizures refractory to medical treatment and/or serious behavioral disorders. After this type of radiosurgery, an adequate symptomatic control is normally achieved, with notable decrease or even disappearance of the seizures. Radiological changes, such as a decrease in the size of the tumor or adjacent edema secondary to non-necrotizing radioinduced inflammatory reaction are unusual consequences. Side effects and neurological complications are also rare events. CASE PRESENTATION: This report describes an unusual case of complete radiological resolution of a hypothalamic hamartoma as well as neurological complications after Gamma-knife surgery (receiving 13 Gy to the 85% isodose line, 1 cm(3) of tumor volume) in a 8-year-old boy who suffered from severe refractory seizures. After radiosurgery, the patient experienced a notable improvement in his symptoms, achieving seizure cessation within 3 months. However, 4 months after the procedure he presented drowsiness, fever and decreased level of consciousness due to a direct effect on the hypothalamus with local and regional edema secondary to the radiosurgery that was performed. He was successfully treated with corticosteroids (with a total duration of 11 months), and twelve months after the surgery, complete disappearance of both the nodular lesion and the secondary edema was observed. The patient remains seizure-free in the last 16 months, with remarkable changes in his behavior. CONCLUSIONS: The present case shows that complete radiological resolution of a hypothalamic hamartoma after Gamma-knife technique is unusual but possible, without long-term neurological consequences. Nevertheless, despite its low incidence, if a patient presents neurological symptoms, primarily during the first year after intervention, possible complications of this type of surgery must be taken into account.

Infección grave por Pneumocystis jirovecii en lactantes con síndrome de West tratados con hormona adrenocorticotropa intramuscular / Classification of structural lesions in magnetic resonance imaging. Surgical implications in drug-resistant epilepsy patients

No disponible

Pharmacological interaction between valproic acid and carbapenem: what about levels in pediatrics?

UNLABELLED: Valproic acid (VPA) is the most commonly used antiepileptic drug in pediatric patients, but its major drawback is its multiple pharmacological interactions. OBJECTIVE: To study children who had been simultaneously treated with carbapenems and valproic acid, considering drug levels, pharmacological interactions and clinical follow-up. MATERIAL AND METHODS: Retrospective study of children who simultaneously received treatment with VPA and carbapenems between January 2003 and December 2011. Demographic variables, indication of treatment, dose, VPA plasma levels, interactions, clinical manifestations and medical management were analyzed. RESULTS: 28 children with concomitant treatment with both drugs were included in the study. 64.3% were males. 78.6% of the interactions were observed in the Intensive Care Unit. 60.7% of children had been previously treated VPA and its major indication were generalized seizures. Basal plasma levels of VPA were recorded in 53% and at 24 h after admittance in 60%. "40% of basal VPA levels were below therapeutic range prior to the administration of carbapenem. After the introduction of carbapenem 88% of level determinations were below therapeutic range". 54.5% of the patients that were chronically receiving VPA and had good control of epilepsy before admission had seizures during the coadministration. One patient that was on VPA before admission but with bad control of epilepsy worsened, and one patient that acutely received VPA did not achieve seizure freedom. In these cases it was necessary to either increase VPA dose or change to a different antiepileptic drug. CONCLUSIONS: Little is known about the mechanism of pharmacologic interactions between carbapenems and VPA, but it leads to a reduction in plasma levels that may cause a loss of seizure control, so simultaneous use of both drugs should be avoided when possible. If not, VPA levels should be monitored.

Funciones visuoespaciales y prematuridad / Visuospatial functions and prematurity

Durante la infancia, las funciones visuoespaciales son importantes en los procesos de aprendizaje y en el desarrollo del pensamiento abstracto. Diferentes estudios muestran que los niños prematuros o con bajo peso al nacer obtienen menores puntuaciones en los tests que valoran las funciones cognitivas, siendo estas diferencias más pronunciadas durante el primer año de vida. Con el tiempo, estas diferencias se van atenuando, pero persiste un retraso madurativo que afecta a la memoria de trabajo y a los procesos visuoespaciales. No está claro cuáles son los factores implicados en el desarrollo de estas funciones y qué factores pre o perinatales pueden interferir en su buen desarrollo, pero se han descrito diferencias anatómicas y fisiológicas entre el cerebro del niño pretérmino y el término que podrían explicar, en parte, alguna de estas alteraciones. La diferente vulnerabilidad selectiva a la hipoxia entre el cerebro inmaduro, en el que predominan las neuronas de la subplaca y los preoligodendrocitos, y el cerebro maduro del niño nacido a término determinan diferencias en el patrón de lesión por hipoxia con mayor afectación de la sustancia blanca periventricular en el niño pretérmino. Este patrón lesional conlleva una disfunción en los procesos atencionales y visuoespaciales debido a la mayor vulnerabilidad de las regiones que intervienen en la ruta dorsal del procesamiento visual (AU)

Visuospatial functions are very important in learning process and development of abstract thought during childhood. Several studies show that preterm and low birth weight infants obtain lower scores in test that assess cognitive functions, specially in the first year of life. These differences are attenuated over time, but a developmental delay that affects working memory and visuospatial process still persists. It is unclear what factors are involved in development of these functions, and pre- or perinatal factors may interfere with the proper conduct of the same, but have been described anatomical and physiological differences between the preterm and term brain that could explain somewhere in these alterations. The different selective vulnerability to hypoxia between immature brain in which preoligodendrocytes and subplate neurons predominate, and mature brain, determine differences in the pattern of injury from hypoxia with greater involvement of the periventricular white matter in preterm children. This lesional pattern leaves to a dysfunction in attentional and visuospatial process, due to the increased vulnerability of the regions involved in the dorsal pathway of visual processing (AU)

Utilidad de la resonancia magnética muscular en el diagnóstico diferencial de las distrofias musculares / The value of muscular magnetic resonance imaging in the differential diagnosis of muscular dystrophies

No disponible

Periventricular nodular heterotopia and dystonia due to an ARFGEF2 mutation.

BACKGROUND: Heterotopias are a neuronal migration disorder caused by extrinsic factors or by genetic mutations. When the location is periventricular, the most frequent genetic cause is the mutation in the "filamin A2 gene", which is X-linked. New genes for periventricular nodular heterotopia with an autosomal inheritance pattern have been recently discovered. PATIENTS: We describe two siblings. The girl, who was prenatally diagnosed ventriculomegaly, had delayed development. At 6 months, she had no head control and variable muscle tone, alternating low axial tone with jerking movements. She became microcephalic. Magnetic resonance imaging at 12 months of age revealed enlarged lateral ventricles, periventricular nodular heterotopia, thin corpus callosum, a T2-hyperintensity of the putamen and the thalamus, and a loss of volume of lenticular nucleus. At 18 months, she developed sporadic myoclonic seizures that were well controlled with valproic acid. Her younger brother also developed progressive microcephaly and psychomotor delay by 6 months. He exhibited axial hypotonia with a prominent dystonic-athetoid component. Magnetic resonance imaging at 15 months of age revealed asymmetric ventriculomegaly plus diffuse nodules lining the temporal horns, a thin corpus callosum, and hyperintensity signal in putamens. He had no seizures. RESULTS: Because of the association of microcephaly, developmental delay with dystonic movements, the imaging results, and the probable autosomal recessive inheritance pattern, genetic analysis was requested. This detected a homozygous nonsense mutation in ARFGEF2 gene, at the DNA level c.388C>T in exon 4. CONCLUSIONS: The presence of dyskinetic movements in individuals with acquired microcephaly could be a manifestation of periventricular nodular heterotopia due to ARFGEF2 mutation.

Acute hemicerebellitis in children: case report and review of literature.

Acute hemicerebellitis in childhood is an extremely rare unilateral presentation of cerebellitis mimicking a tumour. Its aetiology is unknown, although an inflammatory or postinfectious origin is presumed. Its clinical outcome is generally good and a self-limited evolution, in the absence of specific treatment, is usually expected. MRI findings can be misunderstood leading to erroneous diagnosis and invasive treatments. Clinical improvement and regression of the pathological findings in serial MRI will help differentiate acute hemicerebellitis from a neoplastic process. Surgical procedures should be performed only in case of clinical deterioration. We present a case of pseudotumoral hemicerebellitis in an eight-year-old girl, presenting with severe headache. This paper provides a review on hemicerebellitis and highlights the clinical, diagnostic, therapeutic features and outcome of this entity.