Pain report and musculoskeletal impairment in young people with severe forms of cerebral palsy: A population-based series.

BACKGROUND: While pain is reportedly more prevalent in more functionally impaired children with cerebral palsy, information is scant in those with poor communication skills. METHODS: Young people (4-27 years) with severe forms of cerebral palsy were recruited from a population-based register. The Child Health Questionnaire (CHQ) provided information on general health and bodily pain; the Paediatric Pain Profile (PPP) was used for participants with limited communication; and the Spinal Alignment and Range of Motion Measure (SAROMM) described musculoskeletal impairment. RESULTS: 123 young people (GMFCS IV=55 and V=68) and their families/carers participated. Fourteen percent of CHQ responses (n=123) reported severe/very severe pain in recent weeks, whilst 7% reported pain every/almost every day. CHQ pain report was significantly higher for young people in GMFCS level V and correlated significantly with both global health and musculoskeletal impairment. High levels of pain were recorded on the PPP for non-communicating children but only a weak correlation between PPP and CHQ scores was detected. CONCLUSION: Managing pain in young people with severe musculoskeletal and cognitive impairment presents a huge challenge to carers and professionals. The PPP may represent a useful adjunct in those young people with severe communication difficulties.

Surgical versus Ponseti approach for the management of CTEV: a comparative study.

BACKGROUND: Results from a comparative study of Ponseti versus surgical management for congenital talipes equino varus (CTEV), using historically managed patients, are presented. No bias existed in terms of management choice or participants recruited. METHODS: Twenty-three surgically treated children (31 club feet; mean age 9.1 y) and 29 treated by the Ponseti technique (42 club feet; mean age 6.5 y) agreed to participate in the study. Twenty-six typically developing children (mean age 7.9 y) were also recruited as a control group. A physical examination and 3-dimensional gait analyses were carried out on all participants, and each child and his/her parent also, independently, completed the Oxford Ankle Foot Questionnaire (OxAFQ). RESULTS: The Ponseti group underwent fewer joint-invasive procedures than the surgical group. Passive range of dorsiflexion and plantarflexion were significantly less in the CTEV groups when compared with the control group (P<0.001), and plantarflexion was also significantly less in the surgical than in the Ponseti group (P<0.05). The bimalleolar axis was found to be significantly less in the CTEV groups than in the control group (P<0.001) and also significantly less in the surgical than in the Ponseti group (P<0.05). The gait deviation index, a gait score based on kinematics, showed a more normal gait pattern in the Ponseti group compared with the surgical group (P<0.001). The CTEV groups did not differ significantly from each other in terms of ankle sagittal and transverse plane kinematics or kinetics, but foot progression angle for the Ponseti group was external, whereas that for the surgical group was internal. The Ponseti group also scored higher than the surgical group in terms of patient satisfaction, with significantly better parent-rated OxAFQ scores in the "emotional" and "school and play" domains. CONCLUSIONS: The adoption of the Ponseti technique has resulted in fewer and less-invasive operations for our CTEV population, with accompanying improvement in the overall gait pattern (gait deviation index) and parent satisfaction (OxAFQ). LEVEL OF EVIDENCE: Level III.

The health of children and young people with cerebral palsy: a longitudinal, population-based study.

BACKGROUND: Cerebral palsy (CP) is a chronic condition about which little is known in relation to the long term stability of and factors influencing health. OBJECTIVES: To describe the health status of 4-17 year olds with ambulant CP, compare with the general population and identify factors predicting change in health over time. DESIGN: A longitudinal, clinical survey. SETTING: A regional hospital-based Gait Analysis Laboratory. PARTICIPANTS: Those aged 4-17 years and able to walk at least 10m independently were identified from a case register of people with CP. A total of 184 subjects took part (38% of all eligibles in the region); 154 (84%) returned for a second assessment on average 2.5 years later. METHODS: The Child Health Questionnaire (Parent-form-50) was completed by 184 parents at time 1, and 156 at time 2. RESULTS: Children and young people with CP have significantly poorer health across a number of domains when compared to children in the general child population. Over time improvements occurred in behaviour (p=0.01), family activities (p<0.001) and physical functioning (p=0.05). Linear regression showed that gross motor function (p<0.001) and cerebral palsy subtype (p<0.05) were associated with changes in physical functioning; age was associated with changes in behaviour (p=0.007) and family activities (p=0.01); and communication ability was significantly associated with changes in family activities (p=0.005). CONCLUSIONS: Children and young people with CP have poorer health than their able bodied peers but relatively stable health over 2.5 years. Where change occurred, it was for the better.

Passive range of motion in a population-based sample of children with spastic cerebral palsy who walk.

While passive range of motion (PROM) is commonly used to inform decisions on therapeutic management, knowledge of PROM of children with spastic cerebral palsy (CP) is limited. A population-based sample of 178 children with spastic CP (110 male; unilateral, n = 94; bilateral, n = 84; age range 4-17 years) and 68 typically developing children (24 male; age range 4-17 years) were recruited to the study. All children were able to walk a minimum of 10 m over a straight flat course, with or without assistive devices. Gross Motor Function Classification System (GMFCS) levels of participants with CP were: Level I = 55, Level II = 88, Level III = 21, and Level IV = 14. Ankle dorsiflexion, knee extension, popliteal angle, hip abduction, hip internal rotation, and hip external rotation were measured using a goniometer. The results indicate that the children with CP had significantly reduced PROM compared to the children with typical development. Children with CP demonstrated reduced length in the hamstrings, hip adductor, iliopsoas and gastrocnemius-soleus musculature, and contracture at the knee joint. Among children with CP, there were significant reductions in range with increasing functional limitation (higher GMFCS level) and variations based on unilateral or bilateral involvement. This was particularly the case for the hamstrings and hip adductor musculature, where PROM varied considerably across GMFCS Levels I to IV.

Recruitment bias and characteristics of participants with severe cerebral palsy in a cross-sectional survey.

AIM: This article is a report of recruitment bias in a sample of 5-25-year-old patients with severe cerebral palsy. BACKGROUND: The way in which study participants are recruited into research can be a source of bias. METHOD: A cross-sectional survey of 5-25-year-old patients with severe cerebral palsy using standardized questionnaires with parents/carers was undertaken in 2007/2008. A case register was used as the sampling frame, and 260 families were approached: 178/260 (68%) responded and 82/260 families never replied (non-respondents). Among responders: 127/178 (71%) opted in to the study, but only 123/127 were assessed, and 82/178 were opted out (or refused). Multivariable logistic regression giving odds ratios was used to study the association between participant characteristics and study outcomes (responders vs. non-responders; opting in vs. opting out; assessed vs. eligible, but not assessed). RESULTS: Responders (compared with non-responders) were significantly more likely to have a family member with cerebral palsy who was male and resident in more affluent areas. Families who opted in (compared with those opting out and refusing) were more likely to have a family member with cerebral palsy and intellectual impairment and to reside in certain geographical areas. Families who were actually assessed (compared with all eligible, but not assessed) were more likely to have a family member with cerebral palsy and intellectual impairment. CONCLUSION: Several sources of bias were identified during recruitment for this study. This has implications for the interpretation and conclusions of surveys of people with disabilities and complex needs.

Age-related changes in energy efficiency of gait, activity, and participation in children with cerebral palsy.

AIM: The aim of this study was to use a prospective longitudinal study to describe age-related trends in energy efficiency during gait, activity, and participation in ambulatory children with cerebral palsy (CP). METHOD: Gross Motor Function Measure (GMFM), Paediatric Evaluation of Disability Inventory (PEDI), and Lifestyle Assessment Questionnaire-Cerebral Palsy (LAQ-CP) scores, and energy efficiency (oxygen cost) during gait were assessed in representative sample of 184 children (112 male; 72 female; mean age 10y 9mo; range 4-16y) with CP. Ninety-four children had unilateral spastic CP, 84 bilateral spastic CP, and six had other forms of CP. Fifty-seven were classified as Gross Motor Function Classification System (GMFCS) level I, 91 as level II, 22 as level III, and 14 as level IV). Assessments were carried out on two occasions (visit 1 and visit 2) separated by an interval of 2 years and 7 months. A total of 157 participants returned for reassessment. RESULTS: Significant improvements in mean raw scores for GMFM, PEDI, and LAQ-CP were recorded; however, mean raw oxygen cost deteriorated over time. Age-related trends revealed gait to be most inefficient at the age of 12 years, but GMFM scores continued to improve until the age of 13 years, and two PEDI subscales to age 14 years, before deteriorating (p<0.05). Baseline score was consistently the single greatest predictor of visit 2 score. Substantial agreement in GMFCS ratings over time was achieved (κ(lw) =0.74-0.76). INTERPRETATION: These findings have implications in terms of optimal provision and delivery of services for young people with CP to maximize physical capabilities and maintain functional skills into adulthood.

Test-retest reliability of a 1-min walk test in children with bilateral spastic cerebral palsy (BSCP).

The test-retest reliability of a 1-min walk test at a child's maximum walking speed was assessed in children with bilateral spastic cerebral palsy (BSCP). Twelve male and five female children (age range 3-18 years, mean age 12 years 8 months) participated in the study. Children were classified as GMFCS level I (n=5), level II (n=8) and level III (n=4). Results showed that for walk tests performed on different days, distances varied by no more than 13.1m (for 95% of participants) and that a practice walk was vital for reducing systematic bias. The intraclass correlation coefficient was 0.94. A 1-min walk test is a reliable method of assessing function in children with CP but care must be taken when interpreting changes in individual patient data.

The validity of an existing gait classification system when applied to a representative population of children with hemiplegia.

This study describes sagittal plane gait patterns in a representative sample of children with hemiplegic cerebral palsy (CP). Ninety-four children were prospectively recruited to the study (age range 5-18 yrs, mean age 10 yrs 7 mo) and valid kinematic data was captured for 91 participants. Data was also captured for 49 children (age range 5-18 yrs, mean age 10 yrs) with no physical impairment. One representative gait cycle from each child was exported to an Excel template and run through an algorithm that facilitated the description of gait, using logical arguments derived from Winters' Classification. Children with hemiplegic CP, regardless of past surgery history, were allocated to the following gait types: Groups IV (n=9; 10%), III (n=7: 8%), II (n=5: 5%), I (n=32: 35%) and not classified (n=38: 42%). For children with no lower limb surgical history (n=61), gait types were: Groups IV (n=7: 12%), III (n=2: 3%), II (n=2: 3%), I (n=20: 33%) and not classified (n=30: 49%). The gait data taken from children with no physical impairment were not classified. The ability of the Winters' classification system to distinguish between children with higher levels of hemiplegic involvement and children with no physical impairment was demonstrated: the majority of children with hemiplegic CP present with relatively minor gait deviations. A more complete definition of sagittal plane ankle joint kinematics may account for those children that were not classified.

Energy efficiency in gait, activity, participation, and health status in children with cerebral palsy.

The aim of the study was to establish if a relationship exists between the energy efficiency of gait, and measures of activity limitation, participation restriction, and health status in a representative sample of children with cerebral palsy (CP). Secondary aims were to investigate potential differences between clinical subtypes and gross motor classification, and to explore other relationships between the measures under investigation. A longitudinal study of a representative sample of 184 children with ambulant CP was conducted (112 males, 72 females; 94 had unilateral spastic C P, 84 had bilateral spastic C P, and six had non-spastic forms; age range 4-17 y; Gross Motor Function Classification System Level I, n=57; Level II, n=91; Level III, n=22; and Level IV, n=14); energy efficiency (oxygen cost) during gait, activity limitation, participation restriction, and health status were recorded. Energy efficiency during gait was shown to correlate significantly with activity limitations; no relationship between energy efficiency during gait was found with either participation restriction or health status. With the exception of psychosocial health, all other measures showed significant differences by clinical subtype and gross motor classification. The energy efficiency of walking is not reflective of participation restriction or health status. Thus, therapies leading to improved energy efficiency may not necessarily lead to improved participation or general health.

Lifestyle limitations of children and young people with severe cerebral palsy: a population study protocol.

AIM: This paper is a presentation of a study protocol to establish the prevalence of orthopaedic problems (hip dislocation, pelvic obliquity, spinal deformity and contractures) and their impact on pain, function, participation and health in a population of children and young people with severe cerebral palsy. BACKGROUND: Cerebral palsy is the commonest cause of motor impairment in childhood and is associated with life-long disability. An estimated 30% of people with cerebral palsy have severe forms and are non-ambulant. Although the underlying neurological damage is not amenable to correction, many health services are dedicated to providing therapeutic and adaptive support to help people with the condition reach their potential. METHOD: A cross-sectional survey of children and young people, aged 4-25 years with severe, non-ambulant cerebral palsy as defined using the Gross Motor Function Classification System (Levels IV and V). Study participants will be identified from a pre-existing, geographically defined case register and recruited via a healthcare professional known to them. Two assessments will be undertaken: one involving parents/carers at home and using questionnaires; the other involving the child/young person ideally in one of three settings and including X-rays if clinically indicated. DISCUSSION: This study will contribute to our knowledge of the history and epidemiology of orthopaedic problems in children and young people with cerebral palsy and how these problems accumulate and impact on participation, health and well-being. The study will also identify unmet need and make recommendations for good practice in relation to the orthopaedic care and management for people with severe cerebral palsy.

Interobserver agreement of the Gross Motor Function Classification System in an ambulant population of children with cerebral palsy.

Gross Motor Function Classification System (GMFCS) level was reported by three independent assessors in a population of children with cerebral palsy (CP) aged between 4 and 18 years (n=184; 112 males, 72 females; mean age 10y 10mo [SD 3y 7mo]). A software algorithm also provided a computed GMFCS level from a regional CP registry. Participants had clinical diagnoses of unilateral (n=94) and bilateral (n=84) spastic CP, ataxia (n=4), dyskinesia (n=1), and hypotonia (n=1), and could walk independently with or without the use of an aid (GMFCS Levels I-IV). Research physiotherapist (n=184) and parent/guardian data (n=178) were collected in a research environment. Data from the child's community physiotherapist (n=143) were obtained by postal questionnaire. Results, using the kappa statistic with linear weighting (kappa(1w)), showed good agreement between the parent/guardian and research physiotherapist (kappa(1w)=0.75) with more moderate levels of agreement between the clinical physiotherapist and researcher (kappa(1w)=0.64) and the clinical physiotherapist and parent/guardian (kappa(1w)=0.57). Agreement was consistently better for older children (>2y). This study has shown that agreement with parent report increases with therapists'experience of the GMFCS and knowledge of the child at the time of grading. Substantial agreement between a computed GMFCS and an experienced therapist (kappa(1w)=0.74) also demonstrates the potential for extrapolation of GMFCS rating from an existing CP registry, providing the latter has sufficient data on locomotor ability.

Oxygen cost versus a 1-minute walk test in a population of children with bilateral spastic cerebral palsy.

Difficulties with direct measurement of oxygen (O2) consumption have led to the use of simpler proxy measures to estimate energy efficiency in children with cerebral palsy. This study aimed to investigate the relationship between a fast 1-minute walk test and O2 cost in children with bilateral spastic cerebral palsy. Eighty-four children (31 girls, 53 boys; mean age, 10 years 11 months; Gross Motor Function Classification System level I n = 8, level II n = 41, level III n = 21, level IV n = 14) were eligible to participate in the study. Seventy-seven children completed the walk test and 47 children completed the O2 consumption protocol. Results showed significant differences between Gross Motor Function Classification System levels for both tests (P < 0.001) and a significant moderate quadratic relationship between the distance covered during the 1-minute fast-walk test and the net O2 cost (adjusted r = 0.477; P < 0.001). The 1-minute fast-walk test may provide useful information on the efficiency of gait in children with bilateral spastic cerebral palsy in the absence of more sophisticated equipment; however, further work is required to address its reliability and responsiveness.

Recruitment bias in a population-based study of children with cerebral palsy.

OBJECTIVE: The purpose of this analysis was to assess recruitment bias in a population-based study of locomotor ability in children with cerebral palsy. METHOD: A population-based case register was used as a sampling frame and was considered a highly ascertained record of children with the condition. A twin track approach to recruitment for the Locomotor Study was adopted through: (1) a specialist orthopedic service and (2) a community pediatric network on behalf of the case register. The subjects included children with cerebral palsy aged 4 to 16 years in 2003, able to walk > or = 10 m, and a resident in Northern Ireland, as well as their parents. RESULTS: The Orthopaedic Service identified clinically distinct children with cerebral palsy in terms of type, severity, age, and geographic residence. More families responded to an invitation, and more were ultimately recruited into the study via the Orthopaedic Service compared with a case register using community pediatric contacts. Overall, 37.8% of the eligible cerebral palsy population participated in the Locomotor Study, but there was no evidence of any systematic biases in demographic or key clinical characteristics when compared with nonparticipants. One follow-up reminder led to an increase in recruitment of 10%. CONCLUSIONS: Care must be taken in the recruitment of children with cerebral palsy through clinic-based populations, although these routes may prove more successful in follow-up. Provided they are comprehensive, case registers have a valuable contribution to make to clinical research by providing a sampling frame including information on baseline characteristics of an affected population.

Electrical stimulation in cerebral palsy: a randomized controlled trial.

A randomized placebo-controlled trial was carried out to investigate the efficacy of neuromuscular electrical stimulation (NMES) and threshold electrical stimulation (TES) in strengthening the quadriceps muscles of both legs in children with cerebral palsy (CP). Sixty children (38 males, 22 females; mean age 11y [SD 3y 6mo]; age range 5-16y) were randomized to one of the following groups: NMES (n=18), TES (n=20), or placebo (n=22). Clinical presentations were diplegia (n=55), quadriplegia (n=1), dystonia (n=1), ataxia (n=1), and non-classifiable CP (n=2). Thirty-four children walked unaided, 17 used posterior walkers, six used crutches, and the remaining three used sticks for mobility. Peak torque of the left and right quadriceps muscles, gross motor function, and impact of disability were assessed at baseline and end of treatment (16wks), and at a 6-week follow-up visit. No statistically significant difference was demonstrated between NMES or TES versus placebo for strength or function. Statistically significant differences were observed between NMES and TES versus placebo for impact of disability at the end of treatment, but only between TES and placebo at the 6-week follow-up. In conclusion, further evidence is required to show whether NMES and/or TES may be useful as an adjunct to therapy in ambulatory children with diplegia who find resistive strengthening programmes difficult.

Validity of a 1 minute walk test for children with cerebral palsy.

The concurrent validity of a 1 minute walk test at a child's maximum walking speed was assessed in children with bilateral spastic cerebral palsy (BSCP). The distance covered during the 1 minute walk test was compared with the children's gross motor function as assessed by the Gross Motor Function Measure (GMFM). Twenty-four male and 10 female children with CP (mean age 11y, range 4 to 16y) participated in the study. Gross Motor Function Classification System (GMFCS) levels were; level I (n=3), level II (n=17), level III (n=10), and level IV (n=4). Participants had clinical diagnoses of symmetrical diplegia (n=19), asymmetrical diplegia (n=14), and quadriplegia (n=1). Results showed a significant correlation between GMFM score and the distance covered during the 1 minute walk (r=0.92; p<0.001). There was also a significant decrease in the distance walked with increasing GMFCS level (p<0.001). We concluded that the 1 minute walk test is a valid measure for assessing functional ability in children with ambulatory BSCP. Its cost-effectiveness and user friendliness make it a potentially useful tool in the clinical setting. Further study needs to address its reliability and ability to detect change over time.

Estimating mechanical cost in subjects with myelomeningocele.

An attempt was made to validate a system of calculating mechanical cost using 3-D motion analysis by using Oxygen cost measurements in 26 subjects with myelomeningocele. The three models investigated were (i) vertical excursion of the centre of mass (model 1), (ii) external work done by the centre of mass (CM) (model 2) and (iii) a full body model allowing energy transfers between segments within limbs (model 3). Oxygen cost, and models 1 and 3 all demonstrated significant differences between S1 and L4/L5 level involvement (P=0.001, 0.002 and 0.01, respectively). No significant differences were observed between subjects who had L4 and L5 level involvement. While further analysis showed a moderate correlation between Oxygen cost and the vertical excursion of CM model (R2=0.60), a lack of correlation for the more sophisticated models 2 and 3 questions their use as a method of assessing the energy cost of locomotion in this population.

The lack of hypoalgesic efficacy of H-wave therapy on experimental ischaemic pain.

The hypoalgesic effect of H-wave therapy (HWT) at various frequencies (2-60 Hz) was assessed using a standardised form of the submaximal effort tourniquet technique (SETT). Healthy human volunteers (n = 112; 56 men, 56 women) were required to attend on two occasions; on the first, baseline pain scores were obtained and on the second, 48 h later, subjects were randomly assigned to control, placebo or 1 of 5 H-wave treatment conditions. In the treatment groups 2 self-adhesive electrodes were attached to the ipsilateral Erb's point and just lateral to the spinous processes of C6/C7. H-wave stimulation commenced 10 min prior to pain induction and continued throughout the 12-min duration of the technique. In the placebo group electrodes were attached as normal but leads were connected to a dummy power output. Analysis of variance of the differences in visual analogue scale and McGill Pain Questionnaire scores showed no significant differences in the treatment groups compared to placebo or controls. These results do not provide convincing evidence for any hypoalgesic effects of HWT at the frequency parameters stated on the experimental model of pain used.