The purpose of the present study was to describe in greater anatomical detail the changes in brain structure that occur during maturation between childhood and adolescence. High-resolution MRI, tissue classification, and anatomical segmentation of cortical and subcortical regions were used in a sample of 35 normally developing children and adolescents between 7 and 16 years of age (mean age 11 years; 20 males, 15 females). Each cortical and subcortical measure was examined for age and sex effects on raw volumes and on the measures as proportions of total supratentorial cranial volume. Results indicate age-related increases in total supratentorial cranial volume and raw and proportional increases in total cerebral white matter. Gray-matter volume reductions were only observed once variance in total brain size was proportionally controlled. The change in total cerebral white-matter proportion was significantly greater than the change in total cerebral gray-matter proportion over this age range, suggesting that the relative gray-matter reduction is probably due to significant increases in white matter. Total raw cerebral CSF volume increases were also observed. Within the cerebrum, regional patterns varied depending on the tissue (or CSF) assessed. Only frontal and parietal cortices showed changes in gray matter, white matter, and CSF measures. Once the approximately 7% larger brain volume in males was controlled, only mesial temporal cortex, caudate, thalamus, and basomesial diencephalic structures showed sex effects with the females having greater relative volumes in these regions than the males. Overall, these results are consistent with earlier reports and describe in greater detail the regional pattern of age-related differences in gray and white matter in normally developing children and adolescents.

This study is a comparative analysis and meta-analysis of three randomized clinical trials. Children with spastic diplegia received either ‘selective’ dorsal rhizotomy (SDR) plus physiotherapy (SDR+PT) or PT without SDR (PT-only). Common outcome measures were used for spasticity (Ashworth scale) and function (Gross Motor Function Measure [GMFM]). Baseline and 9- to 12-month outcome data were pooled (n=90). At baseline, 82 children were under 8 years old and 65 had Gross Motor Function Classification System level II or III disability. Pooled Ashworth data analysis confirmed a reduction of spasticity with SDR+PT (mean change score difference –1.2; Wilcoxon p<0.001). Pooled GMFM data revealed greater functional improvement with SDR+PT (difference in change score +4.0, p=0.008). Multivariate analysis in the SDR+PT group revealed a direct relationship between percentage of dorsal root tissue transected and functional improvement. SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.

The aim of this study was to investigate the reliability and sensitivity of the Wartenberg pendulum test for quantification of muscle tone in young children with spastic diplegia undergoing selective dorsal rhizotomy (SDR). Fourteen non-disabled children (mean age of 5.5 years, age range 2.3 to 8.8 years, one female and one male in each year) were tested twice. Twenty children with spastic diplegia (12 males, eight females; mean age of 4.3 years, age range 2.5 to 6.3 years) consecutively selected for SDR, were assessed before and 6 months after SDR. Parameters of the pendulum test: R2, R1, maximal velocity, and swing time were correlated with clinical assessments for spasticity (modified Ashworth scale, quadriceps reflex) and measurements of gross motor function: the Gross Motor Function Classification System and the Gross Motor Function Measure. The Wartenberg pendulum test was found to be an objective and sensitive method for quantifying spasticity in knee extensor muscles in children as young as 2.5 years old. The method was responsive to changes after SDR. The only correlation with clinical measurements of spasticity was between the R2 ratio and the quadriceps reflex. Swing time was the most reliable and sensitive variable; it showed a weak correlation with measurements for gross motor function.

To quantify parameters of rhythmic suckle feeding in healthy term infants and to assess developmental changes during the first month of life, we recorded pharyngeal and nipple pressure in 16 infants at 1 to 4 days of age and again at 1 month. Over the first month of life in term infants, sucks and swallows become more rapid and increasingly organized into runs. Suck rate increased from 55/minute in the immediate postnatal period to 70/minute by the end of the first month (p<0.001). The percentage of sucks in runs of [ges ]3 increased from 72.7% (SD 12.8) to 87.9% (SD 9.1; p=0.001). Average length of suck runs also increased over the first month. Swallow rate increased slightly by the end of the first month, from about 46 to 50/minute (p=0.019), as did percentage of swallows in runs (76.8%, SD 14.9 versus 54.6%, SD 19.2; p=0.002). Efficiency of feeding, as measured by volume of nutrient per suck (0.17, SD 0.08 versus 0.30, SD 0.11cc/suck; p=0.008) and per swallow (0.23, SD 0.11 versus 0.44, SD 0.19 cc/swallow; p=0.002), almost doubled over the first month. The rhythmic stability of swallow–swallow, suck–suck, and suck–swallow dyadic interval, quantified using the coefficient of variation of the interval, was similar at the two age points, indicating that rhythmic stability of suck and swallow, individually and interactively, appears to be established by term. Percentage of sucks and swallows in 1:1 ratios (dyads), decreased from 78.8% (SD 20.1) shortly after birth to 57.5% (SD 25.8) at 1 month of age (p=0.002), demonstrating that the predominant 1:1 ratio of suck to swallow is more variable at 1 month, with the addition of ratios of 2:1, 3:1, and so on, and suggesting that infants gain the ability to adjust feeding patterns to improve efficiency. Knowledge of normal development in term infants provides a gold standard against which rhythmic patterns in preterm and other high-risk infants can be measured, and may allow earlier identification of infants at risk of neurodevelopmental delay and feeding disorders.

Using prospectively collected data from 13971 births enrolled in a large population-based cohort study (Avon Longitudinal Study of Parents and Children: ALSPAC), the prevalence of feeding difficulties at 4 weeks and 6 months of age in 33 children subsequently diagnosed with cerebral palsy (CP) were investigated. It was also assessed whether early feeding difficulties could be predictive of functional and growth outcomes at age 8 years. Weak sucking at 4 weeks of age was reported in 11 of 23 children with CP and in 2206 of 12299 (18%) of the remaining population (p<0.0001), and great difficulties feeding at 6 months of age was reported in two of 21 (10%) of the group with CP and in 373 of 10941 (3.3%) of control participants (p=0.017). Feeding difficulties at 4 weeks of age were associated with the pattern of functional impairment at age 4 years (p=0.009) and at 8 years (p=0.068), being clinically underweight (p=0.01), and having speech and swallowing difficulties (p=0.005) at 8 years of age. Early, persistent, and severe feeding difficulties are a marker for subsequent poor growth, feeding, and developmental outcomes and can identify children with CP who will benefit from gastrostomy feeding.

Twenty children and young adults (age range 5 to 33 years, 12 females and eight males) with genetically confirmed 22q11 deletion syndrome (CATCH 22: Cardiac anomaly, Anomalous face, Thymus hypoplasia/aplasia, Cleft palate, and Hypocalcaemia), recruited from a large ongoing study, were given comprehensive assessments with a view to determining the pattern of neuropsychiatric and neuropsychological deficits thought to be part of the syndrome in many cases. IQ ranged between 46 and 100 with a mean score of 70. Half the group had an IQ <70. In 13 individuals, attention-deficit–hyperactivity disorder (ADHD), mainly inattentive or combined type in most cases, and/or autism spectrum problems were diagnosed. Many participants, even among those who had an IQ within the normal range and had neither ADHD nor autistic spectrum problems, showed a characteristic and pronounced behavioural profile with low mental energy, initiation difficulties, deficits in sustained attention, and social interaction (often augmented by limited facial expression and communication and speech problems).

Eighteen children with hemiplegia, mean age 8 years 5 months, underwent gait analysis and musculoskeletal modelling using specially designed software. The maximum lengths of the hamstrings were determined for each child walking in and out of an ankle–foot orthosis (AFO). The muscles were deemed to be short if shorter than the normal average –1SD. In bare feet 8 participants had short medial hamstrings with a higher proportion of these in the less involved individuals. All participants showed an increase in maximum hamstring length when wearing an AFO. In all but one child this was sufficient to restore hamstring length to within normal limits. These finding suggest that hamstring pathology in hemiplegic gait is usually secondary to more distal lower limb pathology.

Postural control deficits have been suggested to be a major component of gait disorders in cerebral palsy (CP). Standing balance was investigated in 23 ambulatory children and adolescents with spastic diplegic CP, ages 5 to 18 years, and compared with values of 92 children without disability, ages 5 to 18 years, while they stood on a force plate with eyes open or eyes closed. The measurements included center of pressure calculations of path length per second, average radial displacement, mean frequency of sway, and Brownian random motion measures of the short-term diffusion coefficient, and the long-term scaling exponent. In the majority of children with CP (14 of 23) all standing balance values were normal. However, approximately one-third of the children with CP (eight of 23) had abnormal values in at least two of the six center of pressure measures. Thus, mean values for path length, average radial displacement, and diffusion coefficient were higher for participants with CP compared with control individuals with eyes open and closed (p<0.05). Mean values for frequency of sway and the long-term scaling exponent were lower for participants with CP compared with control participants (p<0.05). Increased average radial displacement was the most common (nine of 23) postural control deficit. There was no increase in abnormal values with eyes closed compared with eyes open for participants with CP, indicating that most participants with CP had normal dependence on visual feedback to maintain balance. Identification of those children with impaired standing balance can delineate factors that contribute to the patient's gait disorder and help to guide treatment.

An infant is reported who presented with a de novo 21;21 translocation trisomy 21 and an atypical phenotype for Down syndrome (DS). Findings included microcephaly, small stature, downslanting palpebral fissures, absent Brushfield spots, moderate micrognathia, left ptosis, left torticollis, severe developmental delay, seizures, and hypertonia. Further clinical evaluation using both the diagnostic criteria for DS and the Jackson checklist of 25 signs was inconsistent with the diagnosis for DS. Blood karyotype revealed: 46,XX,+21,dic(21;21)(p11.2;p11.2). Fluorescence in situ hybridization (FISH) analysis confirmed the trisomy 21 translocation. Both parents had normal karyotypes. Chromosome and FISH analyses were performed on skin fibroblasts. These studies revealed mosaicism for a translocation trisomy 21 cell line as well as a second cell line consisting of one normal chromosome 21 and a ring chromosome 21 derived from translocation 21q21q which appeared to have a deletion of the critical region for DS involving the distal portion of the the long arm of chromosome 21. The chromosome findings illustrate an atypical phenotype in the spectrum of mosaic DS and suggest possible mechanisms for the variability of the phenotype. It also emphasizes the importance of evaluating other tissues for mosaicism when presented with atypical clinical findings.

Cerebral palsy (CP) is a collection of disorders characterized by an insult to the developing brain that produces a physical disability as the primary or distinguishing feature. The spastic form of CP is most common and in those patients, additional clinical signs may include muscle shortening, diminished selective control, and weakness. The recognition of weakness as a component of CP has been longstanding as evidenced by the names given to this disorder and its subtypes. ‘Cerebral palsy’ means weakness originating from the brain, and the use of the suffixes ‘plegia’ or ‘paresis’ also indicate that weakness is a prominent feature. More than 50 years ago, Phelps contended that resisted exercise ‘to develop strength or skill in a weakened muscle or an impaired muscle group’ was an integral part of treatment in CP. (p 59) Since that time, physical educators have also advocated strengthening. Yet for years, conventional clinical wisdom in physical therapy argued against the use of strength testing and training in children with CP and, indeed, in all persons with CNS disorders. The rationale for this exclusion appears to be multifaceted. First, therapists were discouraged by the relatively meager functional responses to strength training in patients with spasticity compared with those with polio. Clinicians also feared that strong near maximal effort would exacerbate spasticity and muscle tightness in those who were already ‘stiffer’ than normal. Many also attested that impaired selective control in CP essentially prohibited performance of strengthening activities. Consequently, this approach was discarded in favor of a more direct focus on the brain. Only recently has strength testing and training experienced a resurgence in habilitation and rehabilitation programs for this population and for other spastic motor disorders. However, hesitation and even resistance to their incorporation are still encountered despite the lack of evidence to suggest that strengthening is detrimental in the presence of spasticity and accumulating evidence to support this type of exercise. The purpose of this annotation is to summarize existing research on strength testing and training, primarily focusing on CP and address the following clinical questions: (1) Can strength be measured reliably and in a valid way in cerebral palsy? (2) Is weakness a significant impairment in CP? (3) Is strength training effective in increasing force production and improving motor function and disability in CP? (4) Is strength training safe in the presence of spasticity and for children and adolescents, regardless of health status, who have an immature musculoskeletal system?