Diagnosing cause of pain in children with severe cognitive impairments is difficult due to their problems with communication. Identification of risk factors for specific pain etiologies might help professionals in this task. The aim of this study was to determine whether child-related characteristics increase risk for specific types of pain. Participants were the caregivers of 41 females and 53 males with moderate to profound mental retardation, who were aged 3 to 18 years 8 months (mean 10:1, SD 4:4) but who communicated at the level of a typical child of 13.8 months (SD 10 months): 44 of the children had cerebral palsy (CP) and 59 a seizure disorder. Caregivers reported the cause of children's episodes of pain for four 1-week periods over 1 year. Logistic regression analyses were used to predict occurrence of specific types of pain using children's demographic, medical, and physical characteristics. Children had 406 episodes of pain due to accident, gastrointestinal conditions, musculoskeletal problems, infection, recurrent conditions, and common childhood causes. Results indicated that a unique set of risk factors predicted each pain type in this sample. Significant risk factors for pain included: lack of visual impairment and leg impairment (accidental pain); seizures, leg impairment, and greater number of medications (non-accidental pain); being male and tube fed (musculoskeletal pain); age <7 years, absence of CP, visual impairment, and less frequent medical monitoring (infection pain); being female and with arm impairment (gastrointestinal pain); and being tube fed and taking fewer medications (common childhood pains). In most cases, models were more specific than sensitive, indicating that the significant predictors are more useful for eliminating potential pain causes. These results suggest that population risk factors may be helpful in structuring diagnostic investigations for individual children with severe cognitive impairments.

The present study sought to describe the profile of sleep disturbance reported in children with autistic spectrum disorders (ASDs) and to document any sleep disorders underlying reports of sleeplessness. Sixty-nine children aged 5 to 16 years (mean 9 years 4 months, SD 2 years 7 months; 14 females) with an ASD were assessed by detailed sleep histories taken from parents, the Simonds and Parraga Sleep Questionnaire, a 2-week sleep diary, and actigraphs worn by the child for five nights. Parent-reported sleeplessness featured prominently (64%). Sleep disorders underlying the sleeplessness were most commonly behavioural (i.e. to do with inappropriate sleep-related behaviours), although sleep-wake cycle disorders and anxiety-related problems were also seen. In addition, the sleeplessness patterns of a large minority of children could not be classified by conventional diagnostic criteria. Sleep patterns measured objectively did not differ between those children with or without reported sleeplessness, but the sleep quality of all children seemed to be compromised compared with normal values.

The aim of this study was to investigate the relationship between motor performance, attention deficit, impulsiveness, and hyperactivity in children with attention-deficit–hyperactivity disorder (ADHD). Participants were 42 school-aged children with ADHD (36 males, 6 females; mean age 8 years 2 months, SD 1 year 2 months; range 6 years to 11 years), and 42 age- and sex-matched children without ADHD (mean age 8 years 3 months, SD 1 year 1 month; range 6 years to 11 years). Motor abilities were assessed with the Bruininks-Oseretsky Test of Motor Proficiency. Attention and impulse control were measured with the Gordon Diagnostic System, and assessment of activity level was based on two Activity Level Rating Scales that were completed by parents and teachers. Analysis by t-test revealed a significant difference between children with and without ADHD in fine and gross motor skills, impulse control, and attention. Stepwise regression indicated that attention, impulse control, and parent ratings of activity level were the three best predictors of gross motor skills for children with ADHD, accounting for 55.9% of the variance. Attention and impulse control were the two best predictors of fine motor skills, accounting for 45.7% of the variance. Attention and impulse control were consistently found to be important predictors of both fine and gross motor skills in children with ADHD. However, the fact that activity level was a predictor for gross motor proficiency but not for fine motor tasks suggests that different behavioral processes are involved in fine and gross motor performance to different extents.

Parental report may provide an inexpensive alternative to standardized assessments of children's development. We have adapted and validated a parental questionnaire on cognitive development for use with very preterm infants. Sixty-four 2-year-olds (28 males, 36 females) born less than 30 weeks' gestation (median 28.5 weeks, range 23 to 31 weeks), median birthweight 980g (range 500 to 1905g) were assessed using the Mental Development Index (MDI) of the Bayley Scales of Infant Development-II and the parental questionnaire. Significant correlations between parent report and MDI scores (r=0.54–0.68, p<0.001) indicated good concurrent validity. Diagnostic use of the parent report for predicting an MDI score of less than 70 was assessed by using receiver operating characteristic (ROC) curves. The optimal cut off produced equal sensitivity and specificity (81%), indicating good discriminatory power in diagnosing developmental delay. Test–retest reliability was demonstrated and accuracy of parent reporting was not affected by sociodemographic factors. Our questionnaire provides a valid outcome measure for use in randomized trials or large population surveys.

Effects of perinatal exposure to polychlorinated biphenyls (PCBs) on auditory P300 latencies and amplitudes were evaluated in children from a Rotterdam cohort. From this cohort of healthy, term babies, the 26 lowest and 26 highest prenatally PCB-exposed children from the breastfed and the formula-fed groups (n=104) were invited for P300 assessment when they were 9 years of age. For P300 assessment an auditory simple odd-ball paradigm was used. In the 83 participating children, 60 assessments (32 males, 28 females) satisfied the measurement criteria and were included in the data analyses. After adjusting for confounding variables, children with high prenatal exposure were found to have longer P300 latencies than children with low prenatal exposure. Lactational exposure to PCBs through breastfeeding milk was not related to P300 latencies. P300 latencies were shorter in children breast-fed for at least 16 weeks than in children breastfed for 6 to 16 weeks and formula-fed children. P300 amplitudes were not related to perinatal PCB exposure nor breastfeeding. Results of this exploratory study suggest that prenatal exposure to environmental levels of PCBs and related compounds delays mechanisms in the central nervous system that evaluate and process relevant stimuli, whereas breastfeeding accelerates these mechanisms.

This study explored the effects of a flexible supramalleolar orthosis (SMO), indicated to decrease pronation associated with hypotonia, on postural stability in children with Down syndrome. Seventeen children with Down syndrome (nine males, eight females; mean age 5 years 10 months, SD 17.2 months; range 3 years 6 months to 8 years) were tested three times in a 10-week period (weeks 1, 3, and 10) using the Standing and the Walking, Running, and Jumping dimensions of the Gross Motor Function Measure (GMFM), and the Balance subtest of the Bruininks-Oseretsky Test of Motor Proficiency (BOTMP). Range of motion measurements were used to explore the influence of joint laxity. Significant improvement was found with SMOs compared with shoes only in the Standing dimension (p=0.001) and the Walking, Running, and Jumping dimension (p=0.0001) of the GMFM, both at the time of fitting (week 3) and after 7 weeks of wearing SMOs (week 10). For the BOTMP Balance subtest, significant improvement (p=0.027) was seen only at the end of the 7-week study period. Amount of joint laxity did not influence response to orthotic intervention. This study showed that young children with Down syndrome showed immediate and longer-term (after 7 weeks of use) improvement in postural stability with the use of flexible SMOs.

The purpose of this study was to determine and measure the factors that affect the remodeling of the proximal femur after varus osteotomy in children with spastic cerebral palsy (CP). Seventy-five varus proximal osteotomies were performed on 44 children (26 females, 18 males) for hip dysplasia. Thirty-nine patients had spastic CP and five had spastic diplegia; 35 patients used a wheelchair for mobility and nine could walk. Mean age at time of surgery was 8 years (SD 4 years), range 2 years 6 months to 15 years. Preoperative, immediate postoperative, and follow-up (0 to 6 months, 6 months to <1 year, 1 year to <2 years, 2 years to <4 years, 4 years to <6 years, and 6 years to <8 years) radiographs were reviewed. Neck-shaft angles were measured. Correlations to examine the relationship between age at time of surgery and ambulatory status with postoperative changes in neck-shaft angles were reviewed. Remodeling of the proximal femur does occur after varus osteotomy as long as the physes of the proximal femur show growth potential. Remodeling was quite variable between patients: children younger than 4 years old at the time of surgery remodeled more than children who were older than 4 years. Ambulatory status has little effect on the magnitude of remodeling. This study reinforces the need to follow these patients with periodic hip radiographs until completion of growth.

Many children and young adults with cerebral palsy (CP) have diminished bone mineral density (BMD) and a propensity to fracture with minimal trauma. The aim of this study was to identify variables which are routinely assessed as part of standard clinical care and that might be used to identify those individuals with CP who are most likely to have low BMD. One hundred and seven participants (ages 2 years 1 month to 21 years 1 month; mean age 10 years 11 months, SD 4 years 2 months) with moderate to severe spastic CP were assessed in detail. This included gathering clinical data, taking anthropometric measures of growth and nutrition, as well as dual energy X-ray absorptiometry measures of BMD. Seventeen participants were ambulatory with assistance (Gross Motor Function Classification System [GMFCS] level III), and 90 were capable of little or no ambulation even with assistance (26 GMFCS level IV and 64 GMFCS level V). Weight z score proved to be the best predictor of BMD z score. Declining BMD z scores also correlated with increasing age and greater severity of involvement. It can be predicted, with reasonable reliability, that a 10-year-old non-ambulatory child with quadriplegic CP and a ‘typical’ weight z score of –2 will have a BMD z score that is at best –2. Prior fractures, use of anticonvulsants, and feeding difficulties further reduce predicted BMD.

The aim of this study was to examine the prevalence of secondary impairments in young adults with spina bifida and to relate the prevalence to the type of spina bifida and the level of lesion. This cross-sectional study is part of the ASPINE (Adolescents with Spina Bifida in the Netherlands) study. Data were collected on medical history, hydrocephalus (shunt: yes/no), neurological level of lesion (International Standards for Neurological and Functional Classification of Spinal Cord Injury), visual acuity (Landolt rings), spasticity (Modified Ashworth Scale), contractures (range of motion), scoliosis (deviation from perpendicular), ambulation (Hoffer criteria), pressure sores and blood pressure (physical examination), epilepsy, pain, incontinence and sexuality (questionnaire), and cognitive functioning (Raven Standard Progressive Matrices). In total, 179 patients with spina bifida participated (41% male, age range 16 to 25 years, mean 20 years 9 months, SD 2 years 11 months). These were 37 patients with spina bifida occulta, 119 patients with spina bifida aperta and hydrocephalus (AHC+) and 23 patients with spina bifida aperta without hydrocephalus (AHC–). Of our patient group, 73 had a high-level lesion (L2 and above), 68 a mid-level lesion (L3 to L5), and 38 a low-level lesion (S1 and below). Both subdivisions were strongly related with patients with higher lesions more often having hydrocephalus. Most secondary impairments were found for patients with AHC+, and patients with AHC– were mostly comparable to patients with spina bifida occulta. According to level of lesion, most medical problems were found in the high-level lesion group. However, all subgroups suffered from health problems.

Identical female twins (age 11 years) with congenital ocular motor apraxia and generalized idiopathic epilepsy are reported. Their presenting symptoms were a long history of abnormal head and eye movements. One twin developed partial sensory seizures. The patients underwent 16-channel EEG, electro-oculographic recordings, MRI of the brain, and genetic and metabolic investigations. EEG findings were consistent with idiopathic generalized epilepsy. Electro-oculographic recordings of the saccades confirmed an inability to elicit horizontal saccades without preceding head movement; saccades to the left were better than saccades to the right. MR scans for one twin showed normal findings, however, for the twin who had meningitis they revealed asymmetry between the right and left temporal lobes but no specific abnormality. DNA analysis using a series of autosomal polymorphic markers confirmed the monozygocity of the twins. White blood cell enzyme analysis excluded Sandhoff disease, Tay–Sachs disease, GM1 gangliosidosis, metacromatic leucodystrophy, Gaucher disease, Niemann-Pick disease (A and B), and Krabbe leucodystrophy. Albumin and immunoglobulin (IgA, IgG, and IgM) levels were normal. It is concluded that autosomal recessive inheritance seems the most likely explanation here, as recent studies have found insertion and missense mutations of the aprataxin gene which have been related to an early onset form of ataxia with ocular motor apraxia and hypoalbuminaemia.