Cerebral palsy in a total population of 4–11 year olds in southern Sweden. Prevalence and distribution according to different CP classification systems

<p>Abstract</p> <p>Background</p> <p>The aim of this study was to investigate the prevalence of cerebral palsy (CP) as well as to characterize the CP population, its participation in a secondary prevention programme (CPUP) and to validate the CPUP database.</p> <p>Methods</p> <p>The study population was born 1990–1997 and resident in Skåne/Blekinge on Jan 1^st2002. Multiple sources were used. Irrespective of earlier diagnoses, neuropaediatrician and other professional medical records were evaluated for all children at the child habilitation units. The CPUP database and diagnosis registers at hospital departments were searched for children with CP or psychomotor retardation, whose records were then evaluated. To enhance early prevention, CP/probable CP was searched for also in children below four years of age born 1998–2001.</p> <p>Results</p> <p>The prevalence of CP was 2.4/1,000 (95% CI 2.1–2.6) in children 4–11 years of age born in Sweden, excluding post-neonatally acquired CP. Children born abroad had a higher prevalence of CP with more severe functional limitations. In the total population, the prevalence of CP was 2.7/1,000 (95% CI 2.4–3.0) and 48% were GMFCS-level I (the mildest limitation of gross motor function).</p> <p>One third of the children with CP, who were born or had moved into the area after a previous study in 1998, were not in the CPUP database. The subtype classification in the CPUP database was adjusted in the case of every fifth child aged 4–7 years not previously reviewed.</p> <p>Conclusion</p> <p>The prevalence of CP and the subtype distribution did not differ from that reported in other studies, although the proportion of mild CP tended to be higher.</p> <p>The availability of a second opinion about the classification of CP/CP subtypes is necessary in order to keep a CP register valid, as well as an active search for undiagnosed CP among children with other impairments.</p

Assessment of data quality in a multi-centre cross-sectional study of participation and quality of life of children with cerebral palsy

BACKGROUND: SPARCLE is a cross-sectional survey in nine European regions, examining the relationship of the environment of children with cerebral palsy to their participation and quality of life. The objective of this report is to assess data quality, in particular heterogeneity between regions, family and item non-response and potential for bias. METHODS: 1,174 children aged 8–12 years were selected from eight population-based registers of children with cerebral palsy; one further centre recruited 75 children from multiple sources. Families were visited by trained researchers who administered psychometric questionnaires. Logistic regression was used to assess factors related to family non-response and self-completion of questionnaires by children. RESULTS: 431/1,174 (37%) families identified from registers did not respond: 146 (12%) were not traced; of the 1,028 traced families, 250 (24%) declined to participate and 35 (3%) were not approached. Families whose disabled children could walk unaided were more likely to decline to participate. 818 children entered the study of which 500 (61%) self-reported their quality of life; children with low IQ, seizures or inability to walk were less likely to self-report. There was substantial heterogeneity between regions in response rates and socio-demographic characteristics of families but not in age or gender of children. Item non-response was 2% for children and ranged from 0.4% to 5% for questionnaires completed by parents. CONCLUSION: While the proportion of untraced families was higher than in similar surveys, the refusal rate was comparable. To reduce bias, all analyses should allow for region, walking ability, age and socio-demographic characteristics. The 75 children in the region without a population based register are unlikely to introduce bias

Long-term outcomes five years after selective dorsal rhizotomy

<p>Abstract</p> <p>Background</p> <p>Selective dorsal rhizotomy (SDR) is a well accepted neurosurgical procedure performed for the relief of spasticity interfering with motor function in children with spastic cerebral palsy (CP). The goal is to improve function, but long-term outcome studies are rare. The aims of this study were to evaluate long-term functional outcomes, safety and side effects during five postoperative years in all children with diplegia undergoing SDR combined with physiotherapy.</p> <p>Methods</p> <p>This study group consisted of 35 children, consecutively operated, with spastic diplegia, of which 26 were Gross Motor Function Classification System (GMFCS) levels III–V. Mean age was 4.5 years (range 2.5–6.6). They were all assessed by the same multidisciplinary team at pre- and at 6, 12, 18 months, 3 and 5 years postoperatively. Clinical and demographic data, complications and number of rootlets cut were prospectively registered. Deep tendon reflexes and muscle tone were examined, the latter graded with the modified Ashworth scale. Passive range of motion (PROM) was measured with a goniometer. Motor function was classified according to the GMFCS and measured with the Gross Motor Function Measure (GMFM-88) and derived into GMFM-66. Parent's opinions about the children's performance of skills and activities and the amount of caregiver assistance were measured with Pediatric Evaluation Disability Inventory (PEDI).</p> <p>Results</p> <p>The mean proportion of rootlets cut in S2-L2 was 40%. Muscle tone was immediately reduced in adductors, hamstrings and dorsiflexors (p < 0.001) with no recurrence of spasticity over the 5 years. For GMFCS-subgroups I–II, III and IV–V significant improvements during the five years were seen in PROM for hip abduction, popliteal angle and ankle dorsiflexion (p = 0.001), capacity of gross motor function (GMFM) (p = 0.001), performance of functional skills and independence in self-care and mobility (PEDI) (p = 0.001).</p> <p>Conclusion</p> <p>SDR is a safe and effective method for reducing spasticity permanently without major negative side effects. In combination with physiotherapy, in a group of carefully selected and systematically followed young children with spastic diplegia, it provides lasting functional benefits over a period of at least five years postoperatively.</p

Validity and reliability of the Muscle Function Scale, aimed to assess the lateral flexors of the neck in infants

Infants with congenital muscular torticollis (CMT) often have an imbalance in muscle function in the lateral flexors of the neck and the need for a valid and reliable assessment tool to determine muscle function in these muscles is essential. The lateral up righting response is used to examine and to strengthen the Sternocleidomastoid muscle. A muscle function scale (MFS) has been refined and used for several years in a clinic for infants with CMT. The MFS describes an infant’s muscle function in the lateral flexors of the neck through ordered categorical scores. The aim of this study was to find out if the Muscle Function Scale (MFS) is valid and reliable. A panel of experts examined validity, Kappa statistic and intraclass correlation coefficient was calculated for inter-rater and intra-rater reliability. Conclusion: The MFS is found to be a valid tool to measure the muscle function of the lateral flexors of the neck in infants with CMT. The inter-rater and intra-rater reliability is high for both novice and experienced physiotherapists (kappa >0.9, ICC >0.9)

Stretching Treatment for Infants With Congenital Muscular Torticollis: Physiotherapist or Parents? A Randomized Pilot Study

Objective To investigate the time needed to achieve a good result in the range of motion (ROM) in the neck for infants with congenital muscular torticollis (CMT). Design Comparison of stretching treatments performed by physical therapists and parents. Participants Twenty infants (10 female and 10 male) with CMT. Method The infants were randomly assigned to 1 of 2 groups. Stretching treatment was continued until a good ROM was obtained in both rotation (≥90°) and lateral flexion (no side difference). The main outcome measurement was treatment time. The infants were evaluated for ROM in rotation and lateral flexion, muscle function in the lateral flexor muscles of the neck, plagiocephaly, and head tilt. Results The time needed to achieve a good result according to the ROM in the neck was significantly shorter (P < .001) in the physical therapist group than in the parent group. Symmetrical head posture was achieved sooner (P = .03) in the physical therapist group. Conclusion Infants with CMT gained good ROM and symmetric head posture approximately 2 months sooner when treated by an experienced physical therapist; however, interpretation of the results of this small study should be guarded. Further studies are needed to confirm these results

Are infants with torticollis at risk of a delay in early motor milestones compared with a control group of healthy infants?

Recently it is claimed that infants with congenital muscular torticollis (CMT) are at risk of having a delay in achieving early motor milestones. The aim of this study was to investigate if infants with CMT are at risk of at risk of having a delay in achieving early motor milestones and to compare them with a control group of healthy infants. A second aim was to investigate if the time spent in a prone position and plagiocephaly, had any influence on the motor development. Eighty-two infants with CMT (35 female and 47 male) were compared with 40 healthy infants (18 female and 22 male). Motor development was assessed with the Alberta Infant Motor Scale (AIMS). Multiple regression showed that infants in the CMT group had a significantly lower score at AIMS compared with the control group at two (P=0.03) and six months of age (P=0.05). Infants who spent ≥ three times daily in a prone position when awake, had significantly higher scores at AIMS than infants who spent less time in prone at two (P=0.001), six (

The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability

The Manual Ability Classification System (MACS) has been developed to classify how children with cerebral palsy (CP) use their hands when handling objects in daily activities. The classification is designed to reflect the child's typical manual performance, not the child's maximal capacity. It classifies the collaborative use of both hands together. Validation was based on the experience within an expert group, a review of the literature, and thorough analysis of children across a spectrum of function. Discussions continued until consensus was reached, first about the constructs, then about the content of the five levels. Parents and therapists were interviewed about the content and the description of levels. Reliability was tested between pairs of therapists for 168 children (70 females, 98 males; with hemiplegia [n=52], diplegia [n=70], tetraplegia [n=19], ataxia [n=6], dyskinesia [n=19], and unspecified CP [n=2]) between 4 and 18 years and between 25 parents and their children's therapists. The results demonstrated that MACS has good validity and reliability. The intraclass correlation coefficient between therapists was 0.97 (95% confidence interval 0.96-0.98), and between parents and therapist was 0.96 (0.89-0.98), indicating excellent agreement

Association between participation in life situations of children with cerebral palsy and their physical, social, and attitudinal environment:A cross-sectional multicenter European study

OBJECTIVE: To evaluate how participation of children with cerebral palsy (CP) varied with their environment. DESIGN: Home visits to children. Administration of Assessment of Life Habits and European Child Environment Questionnaires. Structural equation modeling of putative associations between specific domains of participation and environment, while allowing for severity of child’s impairments and pain. SETTING: European regions with population-based registries of children with CP. PARTICIPANTS: Children (n=1174) aged 8 to 12 years were randomly selected from 8 population-based registries of children with CP in 6 European countries. Of these, 743 (63%) agreed to participate; 1 further region recruited 75 children from multiple sources. Thus, there were 818 children in the study. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Participation in life situations. RESULTS: For the hypothesized associations, the models confirmed that higher participation was associated with better availability of environmental items. Higher participation in daily activities—mealtimes, health hygiene, personal care, and home life—was significantly associated with a better physical environment at home (P<.01). Mobility was associated with transport and physical environment in the community. Participation in social roles (responsibilities, relationships, recreation) was associated with attitudes of classmates and social support at home. School participation was associated with attitudes of teachers and therapists. Environment explained between 14% and 52% of the variation in participation. CONCLUSIONS: The findings confirmed the social model of disability. The physical, social, and attitudinal environment of disabled children influences their participation in everyday activities and social roles