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No area of developmental neurology is more complex than the specific learning disorders (SLD): the terminology is a morass; the epidemiology, in consequence, is extremely difficult; diagnosis is a problem due to changing symptomatology with age (3-year-olds can't be dyslexic or can they?); the extent to which investigations are initiated is financially and biologically controversial; and management may range from pharmacotherapy to educational strategies through to alternative therapies. Because these children very clearly attract the attention of educationalists and health professionals, they often fall into a divide where neither professional is fully involved. Many clinicians report that they frequently see children in late childhood/early adolescence where the biological background to a SLD has not been identified, resulting in behaviour disturbances which may be criminal.
Autism and Rett syndrome (RS) are both developmental disorders of unknown origin. Autism is a behaviourally defined syndrome. RS, which affects girls only, is characterized by a profound learning disability following early normal development, with a consistent cluster of clinical features. Differentiation of RS from infantile autism in the very early stages of the disorders is not always easy. Both syndromes still lack discriminative laboratory markers for accurate diagnosis and differentiation. We decided to compare the CSF nerve-growth factor (NGF) levels of children with infantile autism and children with RS using enzyme-linked immunosorbent assay (ELISA). Our findings of mainly normal CSF NGF in autism and low to negligible values in RS are in agreement with the different morphological and neurochemical findings (brain growth, affected brain areas, neurotransmitter metabolism) in the two syndromes. CSF NGF could be used as a biochemical marker for differentiation of patients with autism from those with RS.
The aim of the study was to identify the comorbidity of autistic spectrum disorders in a population of children with Down syndrome (DS). All children with DS within a defined population of South Birmingham were identified. The Asperger Syndrome Screening Questionnaire and the Child Autism Rating Scale were completed and diagnosis made according to ICD-10 criteria following interview and observation. Thirty-three of 58 identified children completed the measures, four of whom received a diagnosis of an autistic spectrum disorder. This is equivalent to a minimum comorbid rate of 7%. The questionnaire items concerning social withdrawal, restricted or repetitive interests, clumsiness, and unusual eye contact were associated with an autistic disorder. Of the remaining 29 participating children, 11 also displayed marked obsessional and ritualistic behaviours. The comorbid occurrence of autism and DS is at least 7%. It is important that these children are identified and receive appropriate education and support. A full assessment of social, language, and communication skills and behaviour is crucial, particularly in children with DS who appear different from other children with DS. Potential mechanisms accounting for this comorbidity are discussed.
In the current study, movement ability and underlying kinaesthetic processes of boys with attention deficit–hyperactivity disorder (ADHD) were compared with a group of control children. Two groups of 16 boys with either predominantly inattentive subtype ADHD (ADHD-PI) or combined subtype ADHD (ADHD-C) were compared with 16 control boys matched on age and verbal IQ. The findings demonstrated that the children with ADHD had significantly poorer movement ability than the control children. A high percentage of children with ADHD displayed movement difficulties consistent with developmental coordination disorder. In addition, the current study found that the type and degree of movement difficulty differed between subtypes. Children with ADHD-PI had significantly poorer fine motor skill while children with ADHD-C were found to experience significantly greater difficulty with gross motor skill. The severity of the children's inattentive symptomatology was found to be a significant predictor of motor coordination difficulties. Kinaesthetic sensitivity was not found to differ significantly between the groups.
Previous studies of grasping and object manipulation in children with cerebral palsy (CP) have suggested a dichotomy in the ability to use anticipatory control (planning) of the fingertip force output, depending on the type of sensory information (tactile or proprioceptive) on which it is based. The present study further explores this issue by testing the ability of 15 children with hemiplegic CP aged between 8 and 14 years to scale the fingertip force output in advance during the lifting of small objects whose weight and surface texture are varied. The results indicate that children with hemiplegia can use anticipatory control based on both the weight and texture of the object, but require a greater number of trials than age-matched children without CP (control children) before they can do so. We suggest that the initial lack of anticipatory control results from an indistinct internal representation of the object's physical properties due to disturbed sensory mechanisms, which may have direct implications for therapeutic intervention.
The present study examines the coordination of fingertip forces during object manipulation in both the involved and non-involved hand of 14 children with hemiplegic cerebral palsy (CP) aged between 8 and 14 years. While no impairment could be observed in the non-involved hand, subtle deficits in the sequencing of the grip–lift movement were observed in all children tested in this hand. However, they were able to scale the fingertip force output of the non-involved hand in advance (use anticipatory control) based on the object's weight. In the second experiment in this paper, we tested whether the anticipatory control can be generalized across hands. The results indicate that sensory information from the non-involved hand can be used for anticipatory scaling of isometric force increase during subsequent lifts with the contralateral involved hand. These findings suggest that the initial lack of anticipatory control usually observed in the involved hand of children with hemiplegic CP is likely to be based on disturbed sensory input.
This study compared performance on the Functional Independence Measure for Children (WeeFIMTM), the Battelle Developmental Inventory Screening Test (BDIST), and the Vineland Adaptive Behavior Scales (VABS) in children with developmental disabilities. The three instruments were administered to 205 children with identified disabilities. All 205 children were tested using the WeeFIM instrument. The BDIST was administered to 101 children and the VABS to the remaining 104 children. Administration was counterbalanced and randomized across all three instruments. A proportional sampling plan was used to select the 205 children, who ranged in age from 11 to 87 months. A variety of medical diagnoses and levels of severity of motor, cognitive, and communication impairments were systematically included in the sample. Correlations (r) among subscales for all three instruments ranged from 0.42 to 0.92. Correlations for total scores ranged from 0.72 to 0.94. Analyses of potential moderator variables found no significant relation between age and severity of disability (r=0.05) or between socioeconomic status (SES) and severity of disability (r=0.21). Correlations with age were strongest for those subscale scores involving gross and fine motor skills. Correlations with SES and subscale scores ranged from 0.03 to 0.18. The three instruments provide important information regarding childhood performance in motor, self-care, communicative, cognitive, and social skills. The WeeFIM instrument requires less administration time and provides information directly relevant to evaluating functional outcomes for children with disabilities and their families.
The aim of the study was to examine the predictive value of the variables of parental assessment score, pre-, peri-, and postpartum optimality, sex, socioeconomic status (SES), and maternal education with respect to developmental delay at 18 months, and intellectual disability and school achievement problems at 8 and 14 years. The sample studied comprised 101 children (53 low scorers and 48 controls) originally from a total population of 2783 children assessed by their parents at 18 months using a screening instrument. Data were analysed by logistic regression. The results yielded moderate but statistically significant correlations between predictor and outcome variables. Optimality score and maternal education were the best predictors of developmental delay at 18 months. At 8 years, parental assessment score and maternal education constituted the best predictors of school achievement problems. At 14 years, SES together with parental assessment score were included in the model, when school achievement problems were predicted. Parental assessment score showed the strongest association with school achievement problems at both 8 and 14 years, when children with intellectual disability were included in the analysis. The exclusion of children with intellectual disability from the analyses yielded a stronger association between maternal education (at 8 years) and SES (at 14 years) and school achievement problems. The overall classification accuracy of the models varied between 67% and 88%. Specificity varied from 65% at 18 months to 95% at 14 years. Sensitivity varied from 70% at 18 months and 55% (all cases) and 42% (children with intellectual disability excluded) at 14 years.
We report on a 31-year-old female with vitamin B6-dependent seizures whose seizure onset was in the neonatal period. Her elder brother had the same disorder and died in infancy. Administration of vitamin B6 was initiated in the postnatal period. At the age of 12 years 1 month, 2 months after withdrawal of vitamin B6, visual seizures began to occur frequently. Myoclonic seizures and occasional generalized convulsive seizures were also observed. At the same time, photoparoxysmal response and spontaneous diffuse spike–wave bursts were seen on her EEG. Myoclonic seizures were provoked by intermittent photic stimulation during the EEG. It is distinctive that visual seizures were one of the main seizure types in this patient, that her clinical course was relatively benign, and that she has normal intellectual outcome.
Convulsive status epilepticus (CSE) is the most common medical neurological emergency and continues to be associated with significant morbidity and mortality. The two primary determinants of outcome from CSE are underlying aetiology and seizure length. It is unclear whether a prolonged seizure worsens the prognosis of an underlying aetiology or whether a more severe underlying disorder is more likely to result in CSE. Acceptance of the former hypothesis means that seizures need rapid treatment as their early termination could potentially reduce brain damage and morbidity. Pathophysiology, epidemiology, and outcomes from CSE have recently been reviewed elsewhere.