To Örebro University

AIM: To describe the functional limitations and associated impairments of children with cerebral palsy (CP) in rural Uganda, and care-seeking behaviour and access to assistive devices and education.

METHOD: Ninety-seven children with CP (42 females, 55 males; age range 2-17y) were identified in a three-stage population-based screening with subsequent medical examinations and functional assessments. Information on school and access to care was collected using questionnaires. The data were compared with Swedish and Australian cohorts of children with CP. We used the χ² test and linear regression models to analyse differences between groups.

RESULTS: Younger children were more severely impaired than older children. Two-fifths of the children had severe impairments in communication, about half had intellectual disability, and one third had seizures. Of 37 non-walking children, three had wheelchairs and none had walkers. No children had assistive devices for hearing, seeing, or communication. Care-seeking was low relating to lack of knowledge, insufficient finances, and 'lost hope'. One-third of the children attended school. Ugandan children exhibited lower developmental trajectories of mobility and self-care than a Swedish cohort.

INTERPRETATION: The needs for children with CP in rural Uganda are not met, illustrated by low care-seeking, low access to assistive devices, and low school attendance. A lack of rehabilitation and stimulation probably contribute to the poor development of mobility and self-care skills. There is a need to develop and enhance locally available and affordable interventions for children with CP in Uganda.

WHAT THIS PAPER ADDS: Development of mobility and self-care skills is lower in Ugandan than Swedish children with cerebral palsy (CP). Older children in Uganda with CP are less impaired than younger children. Untreated seizures and impairments of communication and intellect are common. Access to health services, assistive devices, and education is low. Caregivers lack knowledge and finances to seek care and often lose hope of their child improving.

AIM: To identify developmental trajectories of hand function in infants aged 3 months to 12 months with unilateral cerebral palsy (CP).

METHOD: Infants at high risk of unilateral CP were recruited from 3 months of age from follow-up programmes and clinics in Sweden, the Netherlands, Italy, and Australia. Measurements on the Hand Assessment for Infants (HAI) were completed until 12 months of age. Group-based trajectory modelling was used to identify subgroups of infants with similar trajectories of development. Multinomial logistic regression determined associations between demographic variables and trajectory membership.

RESULTS: Ninety-seven infants (52 males, 45 females; median gestational age 38wks [interquartile range 30-40wks]) were included. Infants were assessed between two and seven times (mean 4, SD 1.2) with a total of 387 observations. A three-group trajectory model identified a 'low-functioning group' (n=45: 46%), 'moderate-functioning group' (n=30: 31%), and 'high-functioning group' (n=22: 23%). Mean posterior probabilities (0.91-0.96) and odds of correct classification (26.3-33.2) indicated good model fit. Type of brain lesion, sex, side of hemiplegia, country, gestational age, and access to intensive intervention were not associated with group membership.

INTERPRETATION: Three trajectories of hand function development for infants with unilateral CP were identified and indicate some greater distinctions between groups with increasing age. The HAI is a valuable measure, capturing development of hand function of infants with unilateral CP over time.

WHAT THIS PAPER ADDS: Three distinct developmental trajectories of hand function in infants with unilateral cerebral palsy were identified. A low-functioning group made little progress in development of hand function in the first year of life. The degree of impairment on the impaired hand at 6 months of age is highly associated with trajectory membership. Infants with all types of brain lesion were represented across each trajectory group.

BACKGROUND: Early diagnosis of unilateral cerebral palsy is important after asymmetric perinatal brain injury (APBI). Our objective is to estimate the risk of unilateral cerebral palsy (UCP) in infants with APBI during the first months of life using neuroimaging and clinical assessment.

PATIENTS AND METHODS: Prognostic multivariable prediction modeling study including 52 infants (27 males), median gestational age 39.3 weeks with APBI from Sweden (n = 33) and the Netherlands (n = 19).

INCLUSION CRITERIA: (1) neonatal MRI within one month after term equivalent age (TEA), (2) Hand Assessment for Infants (HAI) between 3.5 and 4.5 months of (corrected) age. UCP was diagnosed ≥24 months of age. Firth regression with cross-validation was used to construct and internally validate the model to estimate the risk for UCP based on the predictors corticospinal tract (CST) and basal ganglia/thalamus (BGT) involvement, contralesional HAI Each hand sum score (EaHS), gestational age and sex.

RESULTS: UCP was diagnosed in 18 infants (35%). Infants who developed UCP more often had involvement of the CST and BGT on neonatal MRI and had lower contralesional HAI EaHS compared to those who did not develop UCP. The final model showed excellent accuracy for UCP prediction between 3.5 and 4.5 months (area under the curve, AUC = 0.980; 95% CI 0.95-1.00).

CONCLUSIONS: Combining neonatal MRI, the HAI, gestational age and sex accurately identify the prognostic risk of UCP at 3.5-4.5 months in infants with APBI.

The objective was to evaluate the effects of the Small Step Program on general development in children at risk of cerebral palsy (CP) or other neurodevelopmental disorders. A randomized controlled trial compared Small Step with Standard Care in infants recruited at 4-9 months of corrected age (CA). The 35-week intervention targeted mobility, hand use, and communication during distinct periods. The Peabody Developmental Motor Scales^2ed (PDMS-2) was the primary outcome measure. For statistical analysis, a general linear model used PDMS-2 as the main outcome variable, together with a set of independent variables. Thirty-nine infants were randomized to Small Step (n = 19, age 6.3 months CA (1.62 SD)) or Standard Care (n = 20, age 6.7 months CA (1.96 SD)). Administering PDMS-2 at end of treatment identified no group effect, but an interaction between group and PDMS-2 at baseline was found (p < 0.02). Development was associated with baseline assessments in the Standard Care group, while infants in the Small Step group developed independent of the baseline level, implying that Small Step helped the most affected children to catch up by the end of treatment. This result was sustained at 2 years of age for PDMS-2 and the PEDI mobility scale.

AIM: To create normative reference values for unilateral and bilateral use of the hands, using the Hand Assessment for Infants (HAI), a newly developed criterion-referenced assessment measuring hand use in infants aged 3 months to 12 months at risk of cerebral palsy (CP).

METHOD: In total, 489 HAI assessments of typically developing infants (243 females, 246 males), aged 3 months to 10 months (mean 6mo 14d [SD 2mo 5d]), were collected in Italy and Sweden. Normative growth curves based on mean and SDs were created, as well as skill acquisition curves for each test item. Correlation to age and differences between groups based on sex and nationality, as well as differences between the right and the left hand, were investigated.

RESULTS: The growth curves showed a steady increase in mean value and a decrease in SD over age. There were no differences between groups based on sex or nationality. There was a negligible mean difference (0.1 raw score) between the right and left hands.

INTERPRETATION: HAI normative reference values are now available, which can assist in identifying deviating hand use for each month of age, as well as a side difference between hands in infants at risk of CP.

WHAT THIS PAPER ADDS: A Hand Assessment for Infants (HAI) result greater than 2SD below the mean indicates atypical hand use. Skill acquisition curves describe the age at which typically developing infants master the HAI items. Most typically developing infants do not demonstrate asymmetry in hand use.

The aim of this study was to evaluate whether short-term positive effects on bimanual function after surgery of the paretic arm in cerebral palsy are maintained long term. Assisting Hand Assessment (AHA) and active range of motion was tested before surgery and at 7 month and 9-year follow-up (n=18). AHA improved significantly from 50 to 52 U at 7 months, but was not different from before surgery at the 9-year follow-up, 49 U. Surgery of wrist and elbow flexors significantly improved active extension. Improvement in wrist and elbow extension was maintained at the 9-year follow-up, but usefulness of the hand measured with AHA had returned to the same level as before surgery.

Purpose: To elucidate the association between developmental stage of human figure drawing(HFD) and fine motor control, visual perception, and further investigate its potential to be used for screening developmental delay.

Methods: Participants were 301 children at 5½ years of age, 176 born preterm and 125 at term, whose HFDs were categorized into six developmental stages. Motor-Free-Visual-Perception Test, Movement-ABC, Performance Intelligence Quotient (PIQ: Wechsler Scale), and the Visual-Motor Integration test were used. Fine motor functions were explored using ImageJ.

Results: Age-expected HFDs were drawn by 87% of the children, while 13%, mostly preterm boys, drew immature ones. Stages of HFD were related to both PIQ and Movement-ABC. Visuomotor control and visual perception significantly explained the HFD. The sensitivity and specificity of HFD as a screening tool was moderate to good. Conclusions: HFD is influenced by visual perception and visuomotor control and can be used for screening developmental delay at preschool age.

AIMS: To explore the validity of change scores of the Children's Hand-use Experience Questionnaire (CHEQ).

METHODS: Analysis of the CHEQ included 44 children (15 girls) between 6-16 years (median 9.0; IQR 8-11) with unilateral cerebral palsy, with baseline and post- (two-week intensive) intervention assessments using the Goal Attainment Scale (GAS) as external anchor for change. Hypotheses on the magnitude of expected change were formulated and correlation coefficients and effect sizes calculated. Receiver operating curve analysis was performed and the area under the curve (AUC) calculated to investigate the ability of CHEQ to discriminate between improvement and non-improvement according to GAS.

RESULTS: All hypotheses about the magnitude of change were confirmed supporting longitudinal validity of CHEQ scales to measure change in the perception of bimanual performance. AUCs for the Grasp efficacy and the Time utilization were slightly below, and for the Feeling bothered slightly above the threshold. The latter one accurately discriminating between children that improved and did not improve according to the GAS.

CONCLUSIONS: Evidence was found that CHEQ scales capture change in bimanual performance but with limited accuracy for two out of three scales. The validity of CHEQ change scores needs to be further explored in a wider population.

BACKGROUND: There is large variation in treatment responses in children with cerebral palsy. Experimental and clinical results suggest that dopamine neurotransmission and brain-derived neurotrophic factor (BDNF) signalling are involved in motor learning and plasticity, which are key factors in modern habilitation success. We examined whether naturally occurring variations in dopamine and BDNF genes influenced the treatment outcomes.

METHODS: Thirty-three children (18-60months of age) with spastic unilateral cerebral palsy were enrolled in the study. Each child had participated in a training programme consisting of active training of the involved hand for 2h every day during a 2-month training period. The training outcome was measured using Assisting Hand Assessment before and after the training period. Saliva was collected for genotyping of COMT, DAT, DRD1, DRD2, DRD3, and BDNF. Regression analyses were used to examine associations between genetic variation and training outcome.

FINDINGS: There was a statistically significant association between variation in dopamine genes and treatment outcome. Children with a high polygenic dopamine gene score including polymorphisms of five dopamine genes (COMT, DAT, DRD1, DRD2, and DRD3), and reflecting higher endogenous dopaminergic neurotransmission, had the greatest functional outcome gains after intervention.

INTERPRETATION: Naturally occurring genetic variation in the dopamine system can influence treatment outcomes in children with cerebral palsy. A polygenic dopamine score might be valid for treatment outcome prediction and for designing individually tailored interventions for children with cerebral palsy.