Hip adductor spasticity and strength in participants with cerebral palsy (CP) were quantified before and after selective dorsal rhizotomy (SDR) and intensive physical therapy. Twenty-four participants with cerebral palsy (CP group) and 35 non-disabled participants (ND controls) were tested with a dynamometer (CP group: mean age 8 years 5 months, 13 males, 11 females; ND group: mean age 8 years 6 months, 19 males, 16 females). According to the Gross Motor Function Classification System (GMFCS), of the 24 participants with CP, eight were at level I, six were at level II, and 10 participants were at level III. For the spasticity measure, the dynamometer quantified the resistive torque of the hip adductors during passive abduction at 4 speeds. The adductor strength test recorded a maximum concentric contraction. CP group spasticity was significantly reduced following SDR and adductor strength was significantly increased after surgery. Both pre- and postoperative values remained significantly less than the ND controls. Spasticity results agreed with previous studies indicating a reduction. Strength results conflicted with previous literature subjectively reporting a decrease following SDR. However, results agreed with previous objective investigations examining knee and ankle strength, suggesting strength did not decrease following SDR.

The purpose of this study was to determine the effect clinically prescribed ankle–foot orthoses (AFOs) have on the temporal-spatial parameters of gait, as compared with barefoot walking in children with cerebral palsy. A retrospective chart review of data collected between 1995 and 1999 in our motion analysis laboratory was performed. A retrospective chart review of 700 patients revealed 115 patients (mean age 9 years) who had a primary diagnosis of CP (diplegia n=97, hemiplegia n=18). All were wearing clinically prescribed hinged or solid AFOs at the time of undergoing a three dimensional gait analysis. In line with our standard clinical practice, data for both conditions (braced and barefoot walking) were collected the same day by the same examiner. Statistical analyses indicated the temporal and spatial gait parameters of velocity, stride length, step length, and single limb stance were significantly increased (p<0.001) with the use of AFOs versus barefoot walking. Cadence was the only parameter found to not be statistically different.

The aims of the study were: (1) to determine the feasibility of a rehabilitation program combining intensive therapy periods (4 times/week for 4 weeks) with periods without therapy (8 weeks) over a 6-month period in severely impaired children with cerebral palsy (CP); and (2) to measure changes in gross motor function after intensive therapy periods (immediate effects) and rest periods (retention). A convenient sample included five children (two females, three males; mean age 22.6 months [SD 9.9]) with severe forms of CP with impairment of four limbs and trunk (GMFCS levels IV and V). A multiple-baseline design was used. Changes in motor performance were assessed by a blind evaluator using the Gross Motor Function Measure. Visual and statistical analyses followed. Level of compliance during intensive therapy was 93.1%. Children received a mean of 30 treatments over the 24 weeks of the experimental phase compared with the 48 treatments they would have received routinely. Increases in GMFM scores (mean 9.2%; range 3 to 15%) were significant in three children (p<0.05) and all participants maintained their motor performance during the two 8-week rest periods. Results showed that four treatments per week over a 4-week period were well tolerated when separated by rest periods. The intermittent program led to improvements in motor function that were maintained over the rest periods. Results underline the need to reconsider the organization of physical rehabilitation programs. A regime that is intensive enough without being tiring and one that provides practice conditions for consolidating motor skills learned during the intensive therapy period may best optimize motor training.

In this study parents' systematic accounts of the health status of 408 school-aged children with cerebral palsy (CP) are reported (221 males, 187 females; mean age 8 years 5 months, SD 1 year 11 months; range 5 to 13 years), as are relations between severity of functional motor impairment and eight functional health status domains. Data were collected as part of a longitudinal study of the motor development of a population-based, stratified, random sample of children with CP from across Ontario, Canada. The Gross Motor Function Classification System (GMFCS) was used to classify severity of CP and functional health status was described with the eight-level Health Utilities Index – Mark 3. Rates of functional limitations in Mobility, Dexterity, Speech, and Vision were statistically significantly associated with GMFCS levels (all p<0.01), with correlation values (tau-b) of 0.82, 0.58, 0.46, and 0.36, respectively. Functional limitations in hearing (tau-b=0.16; p=0.04) and cognition (tau-b=0.27; p<0.01) were both statistically significantly associated with GMFCS levels, though correlations were low. Neither emotion (tau-b=0.03; p=0.24) nor pain (tau-b=0.07; p=0.37) was associated with degree of functional limitation as described by the GMFCS. Clinical and epidemiological implications of findings are discussed.

The medical findings from a population-based study of Prader-Willi syndrome (PWS) are discussed (in which birth incidence of PWS was estimated at 1:22000 and death rate at over 3% per annum). In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated. Of all people with a possible diagnosis of PWS, only those meeting clinical criteria and/or with a confirmed genetic diagnosis were included in the study. Sixty-six individuals, 40 males and 26 females with a mean age of 19 years (range of 0 to 46 years) agreed to participate in the population-based study group. A prevalence rate of 25% for non-insulin dependent diabetes mellitus (NIDDM) was found in adults. Mean age at onset was 20 years. Those with NIDDM had a higher past maximum body weight and a greater likelihood of positive family history. Nearly 50% across the age groups reported a history of recurrent respiratory infections. High rates of fractures (29%), leg ulceration (22% in adults), sleep disorders (20%), and severe scoliosis (15% in childhood) were also reported. It is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections. Other causes of morbidity, in particular the high rates of NIDDM, may be due to a failure to manage over-eating resulting in severe obesity. Early diagnosis and clear guidance to families about these risks and how they might be prevented is recommended. It is hypothesized that the high pain threshold may result in the presence of some illness not being apparent.

A postal study was completed by 138 members of the Tuberous Sclerosis Association for individuals with severe learning disabilities from tuberous sclerosis complex (TSC) (78 males, median 20 years of age; 60 females median 19 years 6 months of age) to investigate the abilities and care needs of these adolescents and young adults. Results were compared with a Salford community survey of young people with severe learning disability (SLD) from a variety of causes (42 males, median 17 years; 29 females, median 17 years 8 months of age). Those with SLD associated with TSC had a higher level of verbal disability and were more dependent for managing toileting and bathing. The young people with TSC were less able to interact socially, showing autistic behaviour. Sexually, they were less aware and less active. Only 20 were thought by their parents to have received sex education.

The Test of Infant Motor Performance (TIMP) is a test of functional movement in infants from 32 weeks' post-conceptional age to 4 months postterm. The purpose of this study was to assess in 96 infants (44 females, 52 males) with varying risk, the relation between measures on the TIMP at 7, 30, 60, and 90 days after term age and percentile ranks (PR) on the Alberta Infant Motor Scale (AIMS). Correlation between scores on the TIMP and the AIMS was highest for TIMP tests at 90 days and AIMS testing at 6 months (r=0.67, p=0.0001), but all comparisons were statistically significant except those between the TIMP at 7 days and AIMS PR at 9 months. In a multiple regression analysis combining a perinatal risk score and 7-day TIMP measures to predict 12-month AIMS PR, risk, but not TIMP, predicted outcome (21% of variance explained). At older ages TIMP measures made increasing contributions to prediction of 12-month AIMS PR (30% of variance explained by 90-day TIMP). The best TIMP score to maximize specificity and correctly identify 84% of the infants above versus below the 10th PR at 6 months was a cut-off point of 1 SD below the mean. The same cut-off point correctly identified 88% of the infants at 12 months. A cut-off of –0.5 SD, however, maximized sensitivity at 92%. A negative test result, i.e. score above –0.5 SD at 3 months, carried only a 2% probability of a poor 12-month outcome. We conclude that TIMP scores significantly predict AIMS PR 6 to 12 months later, but the TIMP at 3 months of age has the greatest degree of validity for predicting motor performance on the AIMS at 12 months and can be used clinically to identify infants likely to benefit from intervention.

We report four previously healthy female children, aged between 3 and 8 years, who presented with encephalopathy and an extrapyramidal movement disorder (chorea n=4, rigidity n=2, oculogyric crisis n=2). In addition, an acute behavioural disturbance occurred in two patients and mutism in two others. Seizures heralded the onset of the illness in three patients. Acute MRI was either normal or initially normal with later generalized cerebral atrophy. All infective (including streptococcus), biochemical, and metabolic investigations were normal, although all four patients had oligoclonal bands in the (CSF) but not the serum, indicating intrathecal immunoglobulin synthesis. All four children made an apparently full recovery within four months of the onset.We suggest that these patients represent an immune-mediated movement disorder and encephalopathy syndrome.

Stuttering, or stammering as it is also referred to, is a developmental speech disorder, which usually appears in children between the ages of 3 and 8 years. More often than not it remits before puberty, but it can persist into adult life. Stuttering is characterized by involuntary syllable repetitions, syllable prolongations, or interruptions (blocks) in the smooth flow of speech. There may sometimes be difficulty in differentiating infants who definitely stutter from those who show the dysfluency often seen in infancy as a normal stage of speech development. However, the two are distinct. The overall frequency of dysfluency, the proportion and duration of dysfluency types, and the associated behaviours not directly related to speech such as eye, head, and body movements, can help to distinguish between the two. There has certainly been controversy over the exact definition of stuttering when selecting participants for research studies. Wingate criticized Yairi and coworkers for not being strict enough in their selection of affected children and for including those who were classified as having stutter-like dysfluencies, especially whole-word repetitions. However, Yairi and colleagues refuted these claims, stating that although syllable repetitions are the most common component of stuttering, whole-word repetitions do occur in stuttering and therefore this is not a criteria for exclusion. In fact, whole-word repetitions seem to be more common when the onset of stuttering is early in life.