Prevalence of cerebral palsy and factors associated with cerebral palsy subtype: A population-based study in Belgium.

Aim: To report on the prevalence, neuroimaging patterns, and function of children with cerebral palsy (CP) in Belgium for birth years 2007-2012, and identify distinctive risk indicators and differences in outcome between CP subtypes.

Methods: Antenatal and perinatal/neonatal factors, motor and speech function, associated impairments, and neuroimaging patterns were extracted from the Belgian Cerebral Palsy Register. Prevalence was estimated per 1000 (overall, ante/perinatal, spastic, dyskinetic CP) or 10,000 (post-neonatal, ataxic CP) live births.

Multiscale entropy as a metric of brain maturation in a large cohort of typically developing children born preterm using longitudinal high-density EEG in the first two years of life.

We aimed to analyze whether complexity of brain electrical activity (EEG) measured by multiscale entropy (MSE) increases with brain maturation during the first two years of life. We also aimed to investigate whether this complexity shows regional differences across the brain, and whether changes in complexity are influenced by extrauterine life experience duration.We measured MSE of EEG signals recorded longitudinally using a high-density setup (64 or 128 electrodes) in 84 typically developing infants born preterm (<32 weeks' gestation) from term age to two years.

A multidisciplinary approach and consensus statement to establish standards of care for Angelman syndrome.

Background: Angelman syndrome (AS) is a rare neurogenetic disorder present in approximately 1/12,000 individuals and characterized by developmental delay, cognitive impairment, motor dysfunction, seizures, gastrointestinal concerns, and abnormal electroencephalographic background. AS is caused by absent expression of the paternally imprinted gene UBE3A in the central nervous system. Disparities in the management of AS are a major problem in preparing for precision therapies and occur even in patients with access to experts and recognized clinics.

Nocturnal Transcutaneous Blood Gas Measurements in a Pediatric Neurologic Population: A Quality Assessment.

: To assess the quality of SpO2 and PCO2 recordings via transcutaneous monitoring in children with neurological conditions.: Overnight transcutaneous SpO2 and PCO2 were analyzed. The presence of drift and drift correction was noted, and the rate of disrupted recordings scored (0: absence, 1; presence).

Multicentre prospective randomised single-blind controlled study protocol of the effect of an additional parent-administered sensorimotor stimulation on neurological development of preterm infants: Primebrain.

Introduction: Preterm and very low birthweight infants are at increased risk for neurodevelopmental disorders, including cerebral palsy, sensory impairment and intellectual disability. Several early intervention approaches have been designed in the hope of optimising neurological development in this context. It seems important that the intervention takes into account parental mental health, focuses on parent-child interactions and lasts sufficiently long.

High-density electroencephalography developmental neurophysiological trajectories.

Efforts to document early changes in the developing brain have resulted in the construction of increasingly accurate structural images based on magnetic resonance imaging (MRI) in newborn infants. Tractography diagrams obtained through diffusion tensor imaging have focused on white matter microstructure, with particular emphasis on neuronal connectivity at the level of fibre tract systems. Electroencephalography (EEG) provides a complementary approach with more direct access to brain electrical activity.

Phenotypic plasticity and the perception-action-cognition-environment paradigm in neurodevelopmental genetic disorders.

Careful study of the phenotype can have implications at several levels, namely clinical diagnosis, pathophysiological reasoning, management planning, and outcome measurement. Behavioural phenotypes involve cognition, communication, social skills, and motor control. They can be documented in a host of neurodevelopmental conditions and approached with the recently refined perception-action-cognition-environment (PACE) paradigm, which focuses on the neurodevelopmental processes that underlie learning and adaption to the environment through perception, action, and cognitive processing.

Effect of ankle-foot orthoses on trunk sway and lower limb intersegmental coordination in children with bilateral cerebral palsy.

Introduction: Ankle-foot orthoses may significantly improve lower limb kinematics in the gait of children with cerebral palsy. Here we aimed to analyze the effect of ankle-foot orthoses on trunk postural control and lower limb intersegmental coordination in children with mild spastic diplegia (GMFCS I or II).

Methods: We recorded tridimensional trunk kinematics and thigh, shank, and foot elevation angles in 20 4-12 year-old children born preterm with spastic diplegia and 20 typically developing children while walking either barefoot or with ankle-foot orthoses.

Behavior and neuropsychiatric manifestations in Angelman syndrome.

Angelman syndrome has been suggested as a disease model of neurogenetic developmental condition with a specific behavioral phenotype. It is due to lack of expression of the UBE3A gene, an imprinted gene located on chromosome 15q. Here we review the main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact.

Epilepsy in Angelman syndrome.

Angelman syndrome is a neurogenetic disorder caused by lack of UBE3A gene expression from the maternally inherited chromosome 15 due to various 15q11-q13 abnormalities. In addition to severe developmental delay, virtual absence of speech, motor impairment, a behavioural phenotype that includes happy demeanor, and distinctive rhythmic electroencephalographic features, over 90% of patients have epilepsy. Many different seizure types may occur, atypical absences and myoclonic seizures being particularly prevalent.

Are there distinctive sleep problems in Angelman syndrome?

Angelman syndrome is a neurogenetic condition characterized by developmental delay, absence of speech, motor impairment, epilepsy and a peculiar behavioral phenotype that includes sleep problems. It is caused by lack of expression of the UBE3A gene on the maternal chromosome 15q11-q13. Although part of the diagnostic description, 'sleep problems' are not well characterized.

Metachromatic leukodystrophy without arylsulfatase A deficiency: a new case of saposin-B deficiency.

Metachromatic leukodystrophy (MLD) is an autosomal recessive neurodegenerative lysosomal disease characterized by accumulation of sulfatides, extensive white matter damage and loss of both cognitive and motor functions. In vivo, the catabolism of sulfatide requires both the enzyme arylsulfatase A and a specific sphingolipid activator protein, saposin-B, encoded by the PSAP gene. Arylsulfatase A activity is deficient in the classical forms of MLD, but exceedingly rare cases of MLD are due to saposin-B deficiency.

Recognition of emotional facial expressions in attention-deficit hyperactivity disorder.

In ADHD, impaired interpersonal relationships have been documented. They have been hypothesized to be secondary to impairment of receptive nonverbal language. Recognition of emotional facial expressions is an important aspect of receptive nonverbal language, and it has been demonstrated to be central to organization of emotional and social behavior.

Pediatric SUNCT Syndrome.

This report describes a 5-year-old male with sudden unilateral headache attacks (2-50 seconds) accompanied by conjunctival injection, lacrimation, and nasal congestion. The episodes occurred without a precipitating factor, never during sleep. Brain imaging was normal.

Motor strategies in standing up in children with hemiplegia.

In spastic hemiplegia, the organization of whole body movements is impaired by deficient postural control. We studied segmental motor patterns involved in standing up from supine position in 15 children with spastic hemiplegic cerebral palsy and 14 unimpaired children using a visual analysis scale previously validated for developmental research. This approach examines specific movement patterns in upper limbs, axis, and lower limbs.