The natural history of gross motor development in children with cerebral palsy aged 1 to 15 years

The aim of this study was to explore motor development in children with cerebral palsy (CP) using developmental curves for CP, subtypes, and the five severity levels of the Gross Motor Function Classification System (GMFCS). The Gross Motor Function Measure (GMFM) and the GMFCS were applied to 317 children (145 females, 172 males) with CP, aged between 1 and 15 years. The CP type distribution was spastic diplegia in 157 (49%), spastic hemiplegia in 101 (33%), spastic tetraplegia in 11 (3%), dyskinesia in 38 (12%), and ataxia in 10 (3%). Forty-five physiotherapists were trained in the GMFM and intra- and interrater reliability was tested. The GMFM was measured prospectively every 6 months up to the age of 4 years and once a year thereafter. Developmental curves were constructed for 258 children with spastic CP. About three-quarters of the children at GMFCS Level I reached 90% of the maximum GMFM score at 5 years of age. The performance peaked at 7 years of age. Children at GMFCS Level II reached 90% at a median age of 5 years, which was also the upper limit, reached by about three-quarters at 7 years of age. The majority of children at GMFCS Level III reached 80% of the GMFM by 7 years of age and most of the children at GMFCS Level IV reached 30% at 5 years and remained there. The median score for children at GMFCS Level V was 20%. The intra- and interrater reliability for the GMFM 88 among physiotherapists were Spearman's rank correlation coefficient 0.91 and 0.99 respectively. There were 931 measurements with a median of 2 (1-11) per child. The gross motor development was demonstrated for the five GMFCS levels in children with spastic CP. These kind of curves may be useful for monitoring and predicting motor development, for planning treatment, and for evaluating outcome after interventions.     

Short-term outcome of multilevel surgical intervention in spastic diplegic cerebral palsy compared with the natural history

Outcome in 24 ambulant children with spastic diplegic cerebral palsy, in whom multilevel surgical intervention was recommended following gait analysis, is reviewed. Twelve children had surgical intervention (treatment group; eight males, four females; mean age 9 years 10 months, SD 3 years 4 months) while the other 12 did not (control group; five males, seven females; mean age 10 years 1 month, SD 2 years 11 months). All children had interval three-dimensional gait analyses (mean time between analyses: control group, 14.1 months; treatment group, 17.9 months). At follow-up the control group (mean age 11 years 9 months) showed a significant increase in minimum hip and knee flexion in stance which was not related to age, the interval between analyses, changes in the passive joint range of motion, nor changes in anthropometric measurements. The treatment group (mean age at follow-up 11 years 3 months) showed a significant improvement in minimum knee flexion and in ankle dorsiflexion in stance. Parents of nine children said their child's walking distance had increased following intervention. Of five children using posterior walkers preoperatively, two continued to use them postoperatively; two were using crutches or sticks and the remaining child walked independently. Two children who walked independently preoperatively used sticks postoperatively for community ambulation. The deterioration seen in the kinematics of the control group suggests that previous outcome studies comparing postoperative gait with preoperative gait have underestimated the immediate effects of surgery. It also raises concerns about the long-term effects of surgical intervention.     

The impact of botulinum toxin A and abduction bracing on long-term hip development in children with cerebral palsy

Aim To study the long‐term impact of 3 years of botulinum toxin A (BoNT‐A) injections and abduction bracing on hip development in children with bilateral spastic cerebral palsy (CP). We wanted to know if early treatment improved hip development and reduced the need for surgery. Method A long‐term review of hip morphology and surgery requirements in children who participated in a multicentre, randomized controlled trial. The trial investigated short‐term effects of BoNT‐A injections combined with an abduction brace, compared with usual care, on hip displacement in children with bilateral spastic CP. Results Forty‐six children with bilateral spastic CP (31 males, 15 females; 10 with diplegia, 36 with quadriplegia; mean age at enrolment of 3y 2mo, mean age at most recent clinical review 13y 11mo [range 10y 6mo–16y 8mo]; three children in Gross Motor Function Classification System level II, 11 in level III, 20 in level IV, 12 in level V) were followed for a mean of 10 years 10 months from recruitment to the trial. Mean migration percentage was 15.9% in the BoNT‐A group and 15.2% in the comparison group (t=0.26, p=0.79). Eighty‐nine percent of hips in the treatment group and 91% hips in the comparison group had satisfactory development, using a valid scale (Mann–Whitney U test=867.50, z=?1.59, p=0.11). Forty children had preventive surgery (21 treatment group, 19 comparison group) and 18 children had reconstructive surgery (10 treatment, 8 comparison). Interpretation In children with bilateral spastic CP, early treatment with BoNT‐A and hip abduction bracing does not reduce the need for surgery or improve hip development at skeletal maturity.     

Prevalence and natural history of progressive supranuclear palsy

We surveyed neurologists and chronic care facilities in and near two New Jersey counties with a combined population of 799,022, regarding cases of progressive supranuclear palsy. All suspected cases were examined personally, using rigid criteria. The prevalence ratio was 1.39/100,000. A total of 50 New Jersey cases yielded median intervals to onset of requiring gait assistance, 3.1 years; visual symptoms, 3.9 years; dysarthria, 3.4 years; dysphagia, 4.4 years; requiring wheelchair, 8.2 years; and death, 9.7 years.     

A classification system for hip disease in cerebral palsy

In population-based studies, hip displacement affects approximately one-third of children with cerebral palsy (CP). Given the extreme range of clinical phenotypes in the CP spectrum, it is unsurprising that hip development varies from normality, to dislocation and degenerative arthritis. Numerous radiological indices are available to measure hip displacement in children with CP; however, there is no grading system for assessing hip status in broad categorical terms. This makes it difficult to audit the incidence of hip displacement, determine the relationship between hip displacement and CP subtypes, assess the outcome of intervention studies, and to communicate hip status between health care professionals. We developed a categorical, radiographic classification of hip morphology based on qualitative indices and measurement of the key continuous variable, the migration percentage of Reimers. One hundred and thirty-four radiographs were reviewed of 52 female and 82 male adolescents with CP who were at, or close to, skeletal maturity (mean age 16y 1mo [SD 1y 4mo] range 14y to 19y 1mo). Twenty-nine were classified at Gross Motor Function Classification System level I, 25 at level II, 27 at level III, 24 at level IV, and 29 at level V. A classification system was developed to encapsulate the full spectrum of hip morphology in CP, with and without intervention.     

The effect of botulinum toxin type A injections on the natural history of equinus foot deformity in paediatric cerebral palsy patients

Neuromuscular blockade with botulinum toxin type A (BTX-A) injections was employed to manage equinus foot deformity in the lower extremities of paediatric cerebral palsy patients. The patients were followed to evaluate the effect of the blockade on lower extremity function and to determine the effect of treatment on the need for tendo-Achilles lengthening (TAL) surgery. The average length of follow-up was 3.4 years. Of the 48 patients, 17 (35%) responded to treatment witbin 6 months of initiation of therapy, as determined by a Physician Rating Scale of gait. Overall, 25 (52%) of the 48 patients underwent TAL surgery during the follow-up period, including eight patients who responded to the BTX-A treatment. The average age of the patients at the time of surgery was 7 years. This age compares favourably with the age at surgery reported in the recent literature. There were no significant differences by responder status, age, or gender in those patients who did and did not require TAL surgery. No adverse events relating to BTX-A were reported. These results indicate that patients who respond to BTX-A treatment have improved physical functioning and gait, and are able to sustain these results long term.     

Correct positioning for hip radiographs allows reliable measurement of hip displacement in cerebral palsy

Aim The pelvic radiograph in children with cerebral palsy (CP) can inform the degree of hip displacement by calculation of the migration percentage. However, concerns have arisen about the reliability of this measurement. The present study examined the reliability of radiographic assessment of displacement and the importance of positioning and reporting experience. Method Two pelvic radiographs, taken at least an hour apart, were performed in 20 children (total 40 hips) in the standard position by a trained paediatric radiographer. Children (13 males, seven females) were aged 30 months to 10 years with severe bilateral spastic CP in Gross Motor Function Classification System levels IV (n=10) and V (n=10). The migration percentage of each hip was measured on two occasions 3 months apart by two experienced radiologists independently. Comparisons of migration percentage were made in three ways by (1) the same observer at the same time, (2) the same observer 3 months apart, and (3) different observers 3 months apart. Results Migration percentage (mean [SD]) was (1) 3.2% (3.5), (2) 3.3% (3.2), and (3) 3.7% (3.8) respectively. Interpretation Reliable measures of migration percentage can be obtained with correct positioning and if reported by suitably experienced radiologists, making this a valid surveillance method. Clinical decisions can be made taking into account an expected error in hip displacement measurements.     

Surgical treatment for hip pain in the adult cerebral palsy patient

Hip subluxation or dislocation in the cerebral palsy population is an acquired condition that can result in pain and limitation of function. The incidence is reported to be from 18 to 59%. Awareness of the factors that cause the problem are essential in order to prevent this condition. Early treatment consists of appropriate muscle lengthening or releases, varus rotation hip osteotomies and in some cases pelvic osteotomies to provide acetabular coverage for the femoral head. For painful hip subluxation or dislocation with arthrosis in the adolescent or adult salvage procedures such as hip arthrodesis, valgus osteotomy, proximal femoral resection, or total hip arthroplasty have all been done to relieve pain. The author recounts his experience of the surgical management of the hip in the individual with cerebral palsy.     

Hip dysplasia in bilateral cerebral palsy: incidence and natural history in children aged 18 months to 5 years

Children with bilateral cerebral palsy (CP) born during 1989 to 1992 (n=346) to a geographically defined population were ascertained and followed up to age 5 years. The aims of the study were to monitor hip development by serial X-rays; to record gross locomotor development, aspects of physical management, and any hip-related orthotics or surgery; to learn more of the natural history of hip development in this condition; and to provide guidelines for a surveillance protocol for those clinically managing children with bilateral CP. Children were X-rayed at 18, 24, 30, 48, and 60 months. X-rays were taken in a standardized position and measured to record migration percentage, acetabular index, Sharp's angle, and Smith's diaphyseal ratios. Relationships between the measures were investigated and a natural history of the children's hip development suggested. At as early as 18 months, migration percentages were significantly greater than in the normally developing population, although no measured CP factor could be identified to be the cause of this early change. Two possible factors affecting early hip development are described, neither of which is clinically apparent: the first separates the bilateral CP population as a whole from the normal population; but there is an additional presdisposing factor affecting some children only which in the presence of the motor disorder, has a significant influence on early hip development and subsequent dysplasia. The study confirms that, when measured correctly, migration percentage is the best guide to hip surveillance and the need for treatment. It is suggested that all children with bilateral CP should be X-rayed in a standardized position at 30 months (corrected for gestational age).     

The development of anticipatory action planning in children with unilateral cerebral palsy

Background Previous studies suggest that compromised bimanual performance experienced by children with unilateral cerebral palsy (CP) is not only due to difficulties in action execution but may also be a result of impaired anticipatory action planning.Aims The effect of age and side of hemiplegia were examined and the relationship between anticipatory action planning, unimanual capacity and bimanual performance was explored.Methods and procedures Using a multi-centre, prospective, cross-sectional observational design, anticipatory action planning was analyzed in 104 children with unilateral cerebral palsy, aged 6–12 years, using the sword task.Outcomes and results Anticipatory action planning did not improve with age in children with unilateral CP, aged between 6–12 years. No differences were found between children with left or right hemiplegia. Finally, anticipatory action planning was not related to unimanual capacity or bimanual performance.Conclusion and implications This study demonstrates anticipatory action planning, measured using the sword task, does not improve with age in children with unilateral CP and is not related to bimanual performance or laterality. Future studies of anticipatory action planning in children with unilateral CP should consider using measures that require effective anticipatory action planning for successful task completion rather than end state comfort.     

The origins of cerebral palsy

PURPOSE OF REVIEW: Cerebral palsy is the most common and visible motor disability of childhood. Its aetiology remains a topic of hot debate between those who see it as a reflection of medical mismanagement of an avoidable complication and those who see its origins in the development of the fetal brain affected at many points along a causal pathway to damage. This review outlines the themes of research publications over the year 2004/2005. RECENT FINDINGS: The review looks at recent findings relating to epidemiology, infection and inflammation, prematurity, multiple pregnancy, thrombophilias, genetics, placenta, neuroimaging and rescue therapies in cerebral palsy. SUMMARY: Papers this year have helped clarify risk groups and identify some areas (e.g. the management of thrombophilias and the potential of induced hypothermia) with the potential to be rapidly introduced into clinical practice. In this enigmatic and multifactorial condition, however, progress remains slow. New tools such as magnetic resonance imaging are providing valuable insights into the lesions that result in cerebral palsy but the pathways to injury remain unclear. The future of cerebral palsy research lies in understanding the complex interactions of multiple factors on the road to cerebral palsy or in looking for final common pathways such as inflammation which may be amenable to manipulation.