Small biparietal diameter and head circumference are part of the phenotype instead of independent prognostic markers in fetuses with spinal dysraphism.

Objective: The aim of this retrospective study was to assess the fetal biparietal diameter (BPD) and head circumference (HC) in the second trimester of pregnancy in fetuses with open spinal dysraphism.

Methods: BPD and HC were measured at 16-26 weeks in 74 fetuses with open spinal dysraphism and compared with reference values.

Results: BPD was smaller in fetuses with open spinal dysraphism.

Motor sequence learning in children with spina bifida.

Cognitive and motor problems are common in children with spina bifida (SB), particularly in those children with cerebral malformations (SBM). Little is known about how these conditions affect motor learning. This study examines motor sequence learning in children with SB, SBM, and healthy controls.

Contribution of the corticospinal tract to motor impairment in spina bifida.

We aimed to disentangle the proportional contributions of upper and lower motor neuron dysfunction to motor impairment in children with spina bifida. We enrolled 42 children (mean age, 11.2 years; standard deviation, 2.

Motor evoked potentials and compound muscle action potentials as prognostic tools for neonates with spina bifida.

Unlabelled: MEPs and CMAPs as prognostic tools for spina bifida.

Aim: The aim of this prospective study was to determine the prognostic value of neurophysiological investigations compared to clinical neurological examination in infants with spina bifida.

Methods: Thirty-six neonates born with spina bifida between 2002 and 2007 were evaluated and followed for 2 years.

Interobserver reliability and diagnostic performance of Chiari II malformation measures in MR imaging--part 2.

Purpose: Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of MR images of the malformation is not always straightforward. Morphometric analyses of the extent of Chiari II malformation may improve the assessment.

Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1.

Purpose: Brain MR imaging is essential in the assessment of Chiari II malformation in clinical and research settings concerning spina bifida. However, the interpretation of morphological features of the malformation on MR images may not always be straightforward. In an attempt to select those features that unambiguously characterize the Chiari II malformation, we investigated the interobserver reliability of all its well-known MR features.

Clinical practice: swallowing problems in cerebral palsy.

Cerebral palsy (CP) is the most common physical disability in early childhood. The worldwide prevalence of CP is approximately 2-2.5 per 1,000 live births.

What could predict effectiveness of Botulinum Toxin to treat drooling: a search for evidence of discriminatory factors on the level of body functions or structures.

Background: The treatment of drooling is important to families that experience the daily impact and research to elucidate clinical factors that play a role in the outcome of drooling treatment should be encouraged.

Aim: To define clinical factors that influence therapy outcome of submandibular Botulinum Toxin (BoNT-A) injections for drooling.

Methods: Prospectively collected data of 128 children with cerebral palsy were evaluated; 80 spastic and 48 dyskinetic movement disorder, mostly Gross Motor Function Classification System III and higher; over 70% had an IQ <70.

Does motor performance matter in botulinum toxin efficacy for drooling?

The aim of this study was to define factors that influence therapy outcome of submandibular botulinum toxin injections for drooling in children with cerebral palsy or mental disability. We postulated that differences in response may be explained by the variation of dysfunctions in the various cerebral palsy subtypes. Prospectively collected data were evaluated of 80 spastic and 48 dyskinetic children, of whom 70% had an IQ of <70.

Influence of birth mode on early neurological outcome in infants with myelomeningocele.

Objective: The aim of the study was to determine whether route of birth affects early neurological outcome in infants with myelomeningocele.

Study Design: In a retrospective cohort study, 95 neonates with myelomeningocele evaluated at the Radboud University Nijmegen Medical Centre between 1990 and 2006 were reviewed. The effect of delivery mode on early neurological outcome was assessed as the difference between the functional neurological level of the defect and the X-ray level (ΔFAX).

Botulinum toxin versus submandibular duct relocation for severe drooling.

Aim: Botulinum neurotoxin type A (BoNT-A) has been described as an effective intervention for drooling and is being increasingly adopted. However, its effectiveness compared with established treatments is still unknown. We undertook a within-participants observational study to examine this.

Thickened saliva after effective management of drooling with botulinum toxin A.

Aim: The aim of this study was to evaluate the rheological properties of saliva after submandibular botulinum toxin type A (BoNT-A) injections.

Method: We enrolled 15 children (11 males and six females; age range 3-17 y, mean age 9 y 10 mo) diagnosed with spastic (n=9) or dyskinetic (n=6) quadriplegic cerebral palsy (CP); Gross Motor Function Classification System level IV or V; and two children with intellectual disability (IQ<70) who experienced moderate to severe drooling. Salivary flow rate and drooling quotient were measured at baseline and at different times after BoNT-A injections up to 24 weeks.

Neuropsychological assessment of attention in children with spina bifida.

Background: Children with the severe form of spina bifida (SBM: spina bifida with myelomeningocele with accompanying hydrocephalus) may manifest attention deficits, and have a similar psychological profile to children with hydrocephalus due to other etiologies. It is unclear to what extent tests to assess attention in SBM are confounded by the accompanying cognitive or visual-motor impairments. The aim of this study was to analyse attention functions by administering two different types of attention tests, one with high and the other with low cognitive and motor requirements.

Motor profile and cognitive functioning in children with spina bifida.

Background: Spina bifida is a complex neuroembryological disorder resulting from incomplete closure of the posterior neural tube. Morbidity in the different fields of motor and cognitive neurodevelopment is variable in nature and severity, and often hard to predict.

Aims: The current study investigates the relationship between cognitive functioning, fine motor performance and motor quality in children with spina bifida myelomeningocele (SBM) and SB-only, taking into consideration the cerebral malformations.

Drooling in cerebral palsy: hypersalivation or dysfunctional oral motor control?

Aim: To investigate whether drooling in children with cerebral palsy (CP) in general and in CP subtypes is due to hypersalivation.

Method: Saliva was collected from 61 healthy children (30 males, mean age 9y 5mo [SD 11mo]; 31 females, mean age 9y 6mo [1y 2mo]) and 100 children with CP who drooled (57 males, mean age 9y 5mo [3y 11mo], range 3-19y; 43 females, mean age 10y 1mo [4y 9mo], range 4-19y), of whom 53 had spastic, 42 had dyskinetic, and five had ataxic CP. Almost all children were affected bilaterally, and 90 of them were at Gross Motor Function Classification System levels III or higher.

Compound muscle action potentials in newborn infants with spina bifida.

The aim of this study was to investigate the relationship between compound muscle action potentials (CMAPs) and neurological impairment in newborn infants with spina bifida. Thirty-one newborn infants (17 males, 14 females, mean gestational age 39 wks [SD 2]; mean birthweight 3336 g [SD 496]) with spina bifida were investigated at a median age of 2 days (range 1-18 d). Motor and sensory impairment and muscle stretch reflexes were assessed and neuroimaging was performed.

Rituximab and intravenous immunoglobulins for relapsing postinfectious opsoclonus-myoclonus syndrome.

We describe 2 children with postinfectious opsoclonus-myoclonus syndrome. Although the patients initially responded to monotherapy with methylprednisolone, intravenous immunoglobulins, or rituximab, they manifested persistent neurologic deficits and relapsing signs. Treatment with rituximab in combination with intravenous immunoglobulin, however, resulted in significant longterm clinical improvement.

Sjögren-Larsson syndrome: motor performance and everyday functioning in 17 patients.

Sjögren-Larsson syndrome (SLS) is an autosomal recessive neurometabolic disorder characterized by spasticity, learning disability, and ichthyosis. To our knowledge, there is no detailed report in the literature concerning the functional consequences of SLS. Therefore, we performed a cross-sectional study of motor performance and everyday functioning in 17 patients with this rare disorder.

Behavioral treatment of drooling: a methodological critique of the literature with clinical guidelines and suggestions for future research.

Many children with mental retardation and developmental disabilities suffer from the consequences of chronic drooling. Behavioral treatment for drooling should be considered before other, more intrusive treatments such as medication and surgery are implemented. However, empirical studies on behavioral procedures are scarce.

A descriptive analysis of studies on behavioural treatment of drooling (1970-2005).

A descriptive analysis was conducted on studies on the behavioural treatment of drooling (published between 1970 and 2005). The 17 articles that met the inclusion criteria described 53 participants (mean age 14y 7mo, [SD 4y 9mo]; range 6-28y). Sex of 87% of the participants was reported: 28 male, 18 female.

Fragmented visuospatial processing in children with pervasive developmental disorder.

Children diagnosed with Pervasive Developmental Disorder Not Otherwise Specified (PDD-NOS) and Asperger Syndrome (AS) may be characterised by a similar perceptual focus on details as children with autistic disorder (AD). This was tested by analysing their performance in a visuoperceptual task [the Children's Embedded Figure Test (CEFT)] and a graphic reproduction task [the Rey Complex Figure Task (Rey CFT)]. Control groups were children with Tourette Syndrome (TS) and typically developing children.

Chronic herpes simplex virus encephalitis in childhood.

Although herpes simplex virus is a major cause of acute encephalitis in childhood, chronic herpes simplex virus encephalitis has only rarely been reported. This report presents a case of chronic herpes simplex virus encephalitis in a 6-year-old female. Diagnosis was based on the detection of herpes simplex virus deoxyribonucleic acid by polymerase chain reaction in combination with the cerebrospinal fluid/serum ratio of herpes simplex virus-specific immunoglobulin G, the presence of herpes simplex virus-specific oligoclonal immunoglobulin G bands in cerebrospinal fluid, and calcifications in the temporal regions found on cerebral computed tomographic scan.

Drooling in children with cerebral palsy: a qualitative method to evaluate parental perceptions of its impact on daily life, social interaction, and self-esteem.

Research on the treatment of drooling applies measures such as salivary flow rate, qualitative observations of drooling severity in standardized situations, and anecdotal or one-dimensional parental and teacher reports. To assess drooling severity in a range of everyday conditions, and its impact on the daily life of children and their families, two parent questionnaires were constructed. Results of baseline measurements of 43 children with cerebral palsy showed that the questionnaires measured the variation in drooling severity across daily life conditions, and enabled evaluation of the impact of drooling on the ability to eat, drink and speak, on daily care, economic consequences, and social interactions.

Responses to lumbar magnetic stimulation in newborns with spina bifida.

Searching for a tool to quantify motor impairment in spina bifida, transcranial and lumbar magnetic stimulation were applied in affected newborn infants. Lumbar magnetic stimulation resulted in motor evoked potentials in both the quadriceps muscle and the tibialis anterior muscle in most (11/13) subjects. However, transcranial magnetic stimulation did not lead to any response at all.