Relationship between gross motor capacity and daily‐life mobility in children with cerebral palsy

Aim The aim of this study was to examine the relationship between gross motor capacity and daily‐life mobility in children with cerebral palsy (CP) and to explore the moderation of this relationship by the severity of CP. Method Cross‐sectional analysis in a cohort study with a clinic‐based sample of children with CP (n=116; 76 males, 40 females; mean age 6y 3mo, SD 12mo, range 4y 8mo–7y 7mo) was performed. Gross motor capacity was assessed by the Gross Motor Function Measure (GMFM‐66). Daily‐life mobility was assessed using the Pediatric Evaluation of Disability Inventory (PEDI): Functional Skills Scale (FSS mobility) and Caregiver Assistance Scale (CAS mobility). Severity of CP was classified by the Gross Motor Function Classification System (48% level I, 17% level II, 15% level III, 8% level IV, 12% level V), type of motor impairment (85% spastic, 12% dyskinetic, 3% ataxic), and limb distribution (36% unilateral, 49% bilateral spastic). Results Scores on the GMFM‐66 explained 90% and 84% respectively, of the variance of scores on PEDI‐FSS mobility and PEDI‐CAS mobility. Limb distribution moderated the relationship between scores on the GMFM‐66 and the PEDI‐FSS mobility, revealing a weaker relationship in children with unilateral spastic CP (24% explained variance) than in children with bilateral spastic CP (91% explained variance). Interpretation In children aged 4 to 7 years with unilateral spastic CP, dissociation between gross motor capacity and daily‐life mobility can be observed, just as in typically developing peers.     

An Italian Cohort Study Identifies Four New Pathologic Mutations in the ARSA Gene

Metachromatic leukodystrophy is an autosomal recessive neurodegenerative disorder of the myelin metabolism due to the impaired function of the lysosomal enzyme arylsulfatase A. Three major clinical variants of metachromatic leukodystrophy (MLD) have been described: late infantile, juvenile, and late onset. The infantile form, whose clinical onset is usually before the age of 2 years, is the most frequent. The juvenile form manifests itself between 3 and 16 years and the late-onset form manifests at any time after puberty. As of today, more than 150 mutations causing MLD have been identified in the ARSA gene that encodes arylsulfatase A. In this paper, we report our experience with the diagnosis of MLD in seven Italian patients from unrelated families. We found 11 different mutations, four of which have not been previously described: c.1215_1223del9 (p.406_408del), c.601 T>C (p.Tyr201His), c.655 T>A (p.Phe219Ile), and c.87C>A (p.Asp29Glu). Our data show once more that there are still several mutations to be discovered in the ARSA gene and there are rarely repeating ones found in the population. The predictive value of the enzyme activity tests in regard to clinical manifestations is extremely limited.     

Neurophysiological Studies In Metachromatic Leukodistrophy

Metachromatic Leukodystrophy (MLD) is a lysosomal storage disease caused by deficiency of Arylsulfatase A (ASA) with accumulation of cerebroside sulfate in the white matter of central and peripheral nervous system. Late infantile, juvenile and adult forms have been described according to the age of onset and severity. Several studies demonstrated the diagnostic usefulness of neurophysiological tests in leukodystrophies, but their role in the progression of the disease and/or on the therapeutic monitoring is still to be established. Four patients with late juvenile MLD were studied by means of visual, auditory, somatosensory and motor evoked potentials to monitor central nervous system involvement and by means of motor and sensory conduction velocity to monitor peripheral nerve function. All neurophysiological tests demonstrated their ability in detecting abnormalities of the related neurological system. Abnormal findings of multimodal Evoked Potentials and sensory nerve conduction study consisted of disappearance of responses while motor nerve conduction study showed quantitative data which consisted of slowing of conduction velocity. As motor nerve fibers are relatively spared in MLD patients, motor nerve conduction studies would provide a simple method for evaluating the course of the disease and therapeutical efficacy. Unfortunately no neurophysiological tests are presently available for long-term follow-up of CNS damage in MLD.     

Similarities and differences between infantile and early childhood onset vanishing white matter disease

Vanishing white matter disease (VWM) is one of the most prevalent inherited leukoencephalopathies in childhood. Infantile VWM is more severe but less understood than the classic early childhood type. We performed a follow-up study on 14 infantile and 26 childhood patients to delineate the natural history and neuroimaging features of VWM. Infantile and childhood patients shared similarities in the incidence of epileptic seizure (35.7 vs. 38.5%) and episodic aggravation (92.9 vs. 84.6%). Developmental delay before disease onset was more common in infantile patients. Motor disability was earlier and more severe in infantile VWM. In survivors with disease durations of 1–3 years, the Gross Motor Function Classification System (GMFCS) was classified as IV–V in 66.7% of infantile and only 29.4% of childhood patients. Kaplan–Meier survival curve analysis indicated that the 5-year survival rates were 21.6 and 91.3% in infantile and childhood VWM, respectively. In terms of MRI, infantile patients showed more extensive involvement and earlier rarefaction, with more common involvement of subcortical white matter, internal capsule, brain stem and dentate nuclei of the cerebellum. Restricted diffusion was more diffuse or extensive in infantile patients. In addition, four novel mutations were identified. In conclusion, we identified some similarities and differences in the natural history and neuroimaging features between infantile and early childhood VWM.     

Causal relation between spasticity, strength, gross motor function, and functional outcome in children with cerebral palsy: a path analysis

Aim This study examined the causal relation between spasticity, weakness, gross motor function, and functional outcome (expressed as activity limitation) in children with cerebral palsy (CP) and tested models of functional outcome mediated by gross motor function. Method Eighty‐one children (50 males, 31 females) with CP were recruited for this cross‐sectional study. Their mean age was 10 years 4 months (SD 1y 9mo). Strength was assessed using the Manual Muscle Test. Spasticity was assessed by the Modified Ashworth Scale. The Gross Motor Function Measure assessed gross motor function. The Functional Skills domain of the Pediatric Evaluation of Disability Inventory assessed functional outcome. Twenty‐eight children (34.6%) had quadriplegia, 44 children (54.3%) had diplegia, and nine children (11.1%) had hemiplegia. Children were classified using the Gross Motor Function Classification System with 14 (17.3%) in level I, 9 (11.1%) in level II, 13 (16.0%) in level III, 5 (6.2%) in level IV, and 40 (49.4%) in level V. Results The proposed path model showed good fit indices. The direct effects were significant between spasticity and gross motor function (β=?0.339), between strength and gross motor function (β=0.447), and between gross motor function and functional outcome (β=0.708). Spasticity had a significant negative indirect effect (β=?0.240) and strength had a significant positive indirect effect (β=0.317) on functional outcome through effects on gross motor function. Interpretation Activity‐based rather than impairment‐based intervention is more important for reducing activity limitation in children with CP. The study established a base from which researchers can further develop a causal model between motor impairments and functional outcome.     

Epilepsy in hemiplegic cerebral palsy due to perinatal arterial ischaemic stroke

Aim The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS). Method The study group comprised 63 participants (41 males, 22 females) from a population‐based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome. Results Mean age of participants at the time of study was 10 years 6 months (SD 4y 7mo, range 4–20y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty‐four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset. Interpretation Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy.     

Acceptability and potential effectiveness of a foot drop stimulator in children and adolescents with cerebral palsy

Aim Ankle–foot orthoses are the standard of care for foot drop in cerebral palsy (CP), but may overly constrain ankle movement and limit function in those with mild CP. Functional electrical stimulation (FES) may be a less restrictive and more effective alternative, but has rarely been used in CP. The primary objective of this study was to conduct the first trial in CP examining the acceptability and clinical effectiveness of a novel, commercially available device that delivers FES to stimulate ankle dorsiflexion. Method Twenty‐one individuals were enrolled (Gross Motor Function Classification System [GMFCS] levels I and II, mean age 13y 2mo). Gait analyses in FES and non‐FES conditions were performed at two walking speeds over a 4 month period of device use. Measures included ankle kinematics and spatiotemporal variables. Differences between conditions were revealed using repeated measures multivariate analyses of variance. Results Nineteen individuals (nine females, 10 males; mean age 12y 11mo, range 7y 5mo to 19y 11mo; 11 at GMFCS level I, eight at level II) completed the FES intervention, with all but one choosing to continue using FES beyond that phase. Average daily use was 5.6 hours (SD 2.3). Improved dorsiflexion was observed during swing (mean and peak) and at foot–floor contact, with partial preservation of ankle plantarflexion at toe‐off when using the FES at self‐selected and fast walking speeds. Gait speed was unchanged. Interpretation This FES device was well accepted and effective for foot drop in those with mild gait impairments from CP.     

Functional decline in children undergoing selective dorsal rhizotomy after age 10

Aim To compare function and gait in a group of children older than most children who received selective dorsal rhizotomy (SDR) with age‐ and function‐matched peers who received either orthopedic surgery or no surgical intervention. Method A retrospective study examined ambulatory children with diplegic cerebral palsy, aged between 10 years and 20 years and categorized in Gross Motor Function Classification System (GMFCS) levels I or II. Three groups were considered: (1) children who had selective dorsal rhizotomy (n=8; two females, six males; mean age 15y 4mo at SDR, 16y 8mo at follow‐up); (2) children who had orthopedic surgery (n=9; three females, six males; mean age 14y 6mo at SDR, 15y 1mo at follow‐up), and (3) children who had no surgical intervention (n=9; two females, seven males; mean age 15y 6mo at follow‐up). Longitudinal measures of gait analysis (velocity, gait deviation index, and gait variable scores) and gross motor function (GMFCS level, Gross Motor Function Measure scores, and centiles) were examined. Results No significant differences were found between changes in gait comparing rhizotomy with orthopedic surgery; however, the group who received orthopedic surgery demonstrated improved gait compared with the group without surgical intervention. Longitudinal comparisons of gross motor function demonstrated a decrease in the group who received SDR. Between‐group analysis of outcomes also demonstrated worse outcomes of the SDR group compared with the orthopedic surgery group and with the no surgical intervention group. Interpretation Rhizotomy in older children was associated with functional declines compared with similar children who had no surgery and with those who underwent orthopedic surgery. This suggests that age greater than 10 years might be a contraindication for SDR if the goal is to improve motor skills.     

Effects of a supported speed treadmill training exercise program on impairment and function for children with cerebral palsy

Aim To compare the effects of a supported speed treadmill training exercise program (SSTTEP) with exercise on spasticity, strength, motor control, gait spatiotemporal parameters, gross motor skills, and physical function. Method Twenty‐six children (14 males, 12 females; mean age 9y 6mo, SD 2y 2mo) with spastic cerebral palsy (CP; diplegia, n=12; triplegia, n=2; quadriplegia n=12; Gross Motor Function Classification System levels II–IV) were randomly assigned to the SSTTEP or exercise (strengthening) group. After a twice daily, 2‐week induction, children continued the intervention at home 5 days a week for 10 weeks. Data collected at baseline, after 12‐weeks’ intervention, and 4 weeks after the intervention stopped included spasticity, motor control, and strength; gait spatiotemporal parameters; Gross Motor Function Measure (GMFM); and Pediatric Outcomes Data Collection Instrument (PODCI). Results Gait speed, cadence, and PODCI global scores improved, with no difference between groups. No significant changes were seen in spasticity, strength, motor control, GMFM scores, or PODCI transfers and mobility. Post‐hoc testing showed that gains in gait speed and PODCI global scores were maintained in the SSTTEP group after withdrawal of the intervention. Interpretation Although our hypothesis that the SSTTEP group would have better outcomes was not supported, results are encouraging as children in both groups showed changes in function and gait. Only the SSTTEP group maintained gains after withdrawal of intervention.     

Leukodystrophy Presenting as Acute-Onset Polyradiculoneuropathy

BackgroundSulfatides, the most abundant glycosphingolipids, are a major component of myelin. They are degraded by the combined action of sphingolipid activator protein and arylsulfatase A. Deficiency of either of these entities causes metachromatic leukodystrophy (MLD). On the basis of age of onset, this entity is divided into late infantile, juvenile, and adult subtypes. Late infantile form, the commonest subtype, can exhibit peripheral neuropathy as the initial manifestation. The other two forms usually manifest peripheral neuropathy later in the disease course.PatientA 1.5-year-old girl with preexisting isolated motor delay presented with acute-onset ascending flaccid quadriparesis, ptosis, and respiratory failure. Ptosis and respiratory failure responded completely to intravenous immunoglobulin, whereas quadriparesis showed minimal improvement. Nerve biopsy revealed metachromatic granules with demyelination, and serum arylsulfatase A levels were undetectable.ConclusionThe severity and nature of the disease coupled with the response to immunotherapy makes this case unusual. This child may represent either an atypical presentation of MLD with coincidental response to immunotherapy or an episode of immune mediated neuropathy in an individual with already diseased nerves due to MLD.     

Peripheral neuropathy in metachromatic leucodystrophy. A study of 40 cases from south India

BACKGROUND: There is a paucity of literature from India on metachromatic leucodystrophy (MLD), a rare metabolic disorder of childhood resulting from aryl sulfatase A (ASA) deficiency.Patients/ METHODS: Case records of histopathologically verified cases of MLD, evaluated over a period of 12 years at the National Institute of Mental Health and Neurosciences, Bangalore, India, were reviewed. RESULTS: The late infantile group (36) manifested with regression of milestones (all), delayed mile stones (14), gait abnormalities (14), and seizures (11). Despite spasticity (29), there was hypo/areflexia in 25 patients. Optic atrophy (six) was rare. Consanguinity was noted in 25 children and four had a history of similar illness in siblings. Behavioural problems dominated in the juvenile group (four), but associated cognitive decline and hyporeflexia provided a clue to the diagnosis. Low serum ASA (seven of 20), raised cerebrospinal fluid protein (five of 12), and urinary metachromatic granules (two of 32) were infrequent. Electrophysiological evidence of severe demyelinating and length dependent sensory motor neuropathy was observed in all, even in the presence of hyper-reflexia. In addition to metachromatic dysmyelinating neuropathy in all patients, sural nerve biopsy in 20 patients revealed orthochromatic deposits within perivascular macrophages, particularly among those patients with normal ASA values (11 of 14), suggesting the accumulation of other glycosphingolipids. CONCLUSIONS: This study produced some noteworthy observations: the high degree of consanguinity associated with MLD in India, the existence of MLD with normal serum concentrations of ASA, the deposition of orthochromatic lipids, and electrophysiological evidence of a partial conduction block.     

Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia

Aim To investigate the association between magnetic resonance imaging (MRI) patterns and motor function, epileptic episodes, and IQ or developmental quotient in patients born at term with spastic diplegia. Method Eighty‐six patients born at term with cerebral palsy (CP) and spastic diplegia (54 males, 32 females; median age 20y, range 7–42y) among 829 patients with CP underwent brain MRI between 1990 and 2008. The MRI and clinical findings were analysed retrospectively. Intellectual disability was classified according to the Enjoji developmental test or the Wechsler Intelligence Scale for Children (3rd edition). Results The median ages at diagnosis of CP, assignment of Gross Motor Function Classification System (GMFCS) level, cognitive assessment, and MRI were 2 years (range 5mo–8y), 6 years (2y 8mo–19y), 6 years (1y 4mo–19y), and 7 years (10mo–30y) respectively. MRI included normal findings (41.9%), periventricular leukomalacia, hypomyelination, and porencephaly/periventricular venous infarction. The frequency of patients in GMFCS levels III to V and intellectual disability did not differ between those with normal and abnormal MRI findings. Patients with normal MRI findings had significantly fewer epileptic episodes than those with abnormal ones (p=0.001). Interpretation Varied MRI findings, as well as the presence of severe motor dysfunction and intellectual disability (despite normal MRI), suggest that patients born at term with spastic diplegia had heterogeneous and unidentified pathophysiology.     

Relation between physical fitness and gross motor capacity in children and adolescents with cerebral palsy

Aim  To examine the relation between physical fitness and gross motor capacity in children with cerebral palsy (CP) who were classified in Gross Motor Function Classification System levels I or II.
Method  In total, 68 children with CP (mean age 12y 1mo, SD 2y 8mo; 44 males, 24 females; 45 classified as having spastic unilateral CP, 23 as having spastic bilateral CP) participated in this study. All participants performed a maximal aerobic exercise test (10m Shuttle Run Test), a short-term muscle power test (Muscle Power Sprint Test), an agility test (10×5m sprint test), and a functional muscle strength test (30s repetition maximum) within 2 weeks. Gross motor capacity was concurrently assessed using dimensions D (standing) and E (walking, running, and jumping) of the 88-item version of the Gross Motor Function Measure (GMFM).
Results  No relation between aerobic capacity, body mass index, and dimensions D and E of the GMFM was found. The correlations between short-term muscle power, agility, functional muscle strength, and dimensions D and E of the GMFM were moderate to high ( r ∼0.6–0.7).
Interpretation  The relations found between short-term muscle power, agility, functional muscle strength, and gross motor capacity indicate the importance of these components of physical fitness, and may direct specific interventions to maximize gross motor capacity in children and adolescents with CP.     

Development and validation of item sets to improve efficiency of administration of the 66‐item Gross Motor Function Measure in children with cerebral palsy

Aim To develop an algorithmic approach to identify item sets of the 66‐item version of the Gross Motor Function Measure (GMFM‐66) to be administered to individual children, and to examine the validity of the algorithm for obtaining a GMFM‐66 score. Method An algorithmic approach was used to identify item sets of the GMFM‐66 (GMFM‐66‐IS) using data from 95 males and 79 females with cerebral palsy (CP; mean age 14y 7mo, SD 1y 8mo, range 12y 7mo to 17y 8mo). The GMFM‐66‐IS scores were then validated using combined data from three Dutch studies involving 134 males and 92 females with CP (mean age 7y, SD 4y 6mo, range 1y 4mo to 13y 8mo), representing all levels of the Gross Motor Function Classification System. Results The final algorithm contains three decision items from the GMFM‐66 that determine which one of four item sets to administer. The GMFM‐66‐IS has excellent agreement with the full GMFM‐66 both at a single assessment (intraclass correlation coefficient [ICC]=0.994, 95% confidence intervals [CI] 0.993–0.996) and across repeat assessments (ICC=0.92, 95% CI 0.89–0.95). Interpretation The GMFM‐66‐IS is a promising alternative to the full GMFM‐66. Users should be consistent in their choice of measure (GMFM‐66 or GMFM‐66‐IS) on repeat testing and clearly identify which method was used.     

Correspondence of classifications between parents of children with cerebral palsy aged 2 to 6 years and therapists using the Gross Motor Function Classification System

Aim The aim of this study was to determine the agreement and reliability of parent report using a lay version of the Gross Motor Function Classification System (GMFCS) among children with cerebral palsy in the two youngest age bands. Method Data were obtained from the Canadian section of the Movement and Participation in Life Activities of Young Children study database. One hundred and thirty‐two parents of two groups of children participated: children aged 2 to 4 years (35 males, 26 females; mean age 3y 2mo; SD 5mo) and children aged 4 to 6 years (39 males, 32 females; mean age 4y 11mo; SD 6mo) at the final data collection point. Therapists classified motor function using the GMFCS and parents used the GMFCS Family Report Questionnaire, with parents and therapists being masked to the others’ responses. Agreement between respondents was determined using precise agreement and Cohen’s unweighted kappa statistic. Reliability between respondents was determined using the intraclass correlation coefficient (ICC). Results Overall, precise agreement was 77%, chance‐corrected agreement was κ=0.70 (95% confidence interval [CI] 0.61–0.79), and reliability was ICC=0.95 (95% CI 0.93–0.96). Interpretation These values indicate substantial agreement and reliability between parents of children aged 2 to 6 years and therapists. Some parents had a tendency to rate their children as more functionally limited than did therapists, leading us to question whose the true criterion standard’ rating should be.     

Alterations of brain metabolites in metachromatic leukodystrophy as detected by localized proton magnetic resonance spectroscopy in vivo

The brain morphology and chemistry of seven children with late infantile (4/7) and juvenile (3/7) forms of metachromatic leukodystrophy (MLD) were investigated by magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy (MRS). Patients who were examined at least 6 months after the onset of symptoms (6/7) had severe leukodystrophic changes on MRI. Proton MRS revealed a marked reduction of the neuronal markerN-acetylaspartate in white and grey matter and elevated lactate in demyelinated areas. In contrast to other leukodystrophies MLD patients showed a generalized increase of brainmyo-inositol (2- to 3-fold in white matter), indicating a specific role in the pathophysiology of demyelination in MLD.     

Correlates of decline in gross motor capacity in adolescents with cerebral palsy in Gross Motor Function Classification System levels III to V: an exploratory study

Aim To explore associations between clinical variables and decline in motor capacity in adolescents with cerebral palsy (CP). Method Participants included 76 males and 59 females, whose mean age at the beginning of the study was 14 years 6 months (SD 2.4, range 11.6–17.9); 51 at Gross Motor Function Classification System (GMFCS) level III, 47 at level IV, and 37 at level V. Ninety‐six participants had tetraplegia, 32 had diplegia, and one had hemiplegia. Types of motor disorder were spastic n=98; mixed, n=11; dystonic, n=9; hypotonic, n=7; and ataxic n=3 (seven participants were not classified). Reliable raters collected data annually for 4 years on anthropometric characteristics, the Spinal Alignment and Range of Motion Measure, as well as the Gross Motor Function Measure, 66 items (GMFM‐66); participants or their parents reported on health status (using the Health Utilities Questionnaire), pain, and exercise participation (using measures developed for this study). The predicted drop in GMFM‐66 scores after childhood was calculated using data on the same children from an earlier study. Correlations were calculated between the drop in GMFM‐66 scores and the average and change scores of the clinical variables (the alpha level for statistical significance of this exploratory study was 0.10). Results The drop in GMFM‐66 score was significantly correlated with limitations in range of motion (r=0.42) and spinal alignment (r=0.28), and pain (r=0.16). Increases in triceps skinfold (r=?0.19), mid‐arm circumference (r=?0.23), and the ratio of mid‐arm circumference to knee height (r=?0.23) were associated with less decline. Interpretation Preventing range‐of‐motion limitations and pain experiences and optimizing nutrition might contribute to less decline in the gross motor capacity of adolescents with CP. Further investigation is required to clarify the role other factors that contribute to maintained function over time.     

Level of motivation in mastering challenging tasks in children with cerebral palsy

Aim The aim of this study was to describe and identify factors associated with motivation in children with cerebral palsy (CP). Method Children with CP were recruited for this cross‐sectional study. Children were assessed using the Leiter Intelligence Test, the Gross Motor Function Measure, and the Vineland Adaptive Behavior Scale. Parents completed the Dimensions of Mastery Questionnaire (DMQ) and questionnaires on demographics, child behaviour, and family functioning. Results The parents of 74 children (46 males, 28 females; mean age 9y 2mo, SD 2y 1mo, range 5y 10mo–12y 11mo) completed the DMQ. Just over half of the children (39/74) were classified at Gross Motor Function Classification System (GMFCS) level I, with 13 classified at GMFCS level II, one at level III, six at level IV, and 14 at level V; one child was not classified. The most common diagnoses were spastic hemiplegia and quadriplegia (23 each), followed by diplegia (14). The highest motivation scores were obtained for the dimensions of mastery pleasure and social persistence and the lowest for persistence with motor or cognitive tasks. Age and sex were not predictive of scores on the DMQ. Higher IQ (r=0.41), better motor ability (r=0.43), and fewer limitations in self‐care, communication, and socialization (r=0.44–0.53) were positively associated with motivation total score. A negative impact of the child’s disability on the family was associated with lower motivation (r=?0.44). Positive social behaviours were positively correlated with motivation (r=0.38–0.66), whereas hyperactivity and peer problems were negatively associated. Interpretation High motivation was associated with fewer activity limitations and behavioural problems and reduced family burden. Low motivation may adversely influence a child’s functional potential and the effectiveness of interventions. Strategies focusing on the child, peers, adults, or activities are proposed to enhance the children’s motivation to engage in more challenging activities.     

Oromotor dysfunction and communication impairments in children with cerebral palsy: a register study

Aim To report the prevalence, clinical associations, and trends over time of oromotor dysfunction and communication impairments in children with cerebral palsy (CP). Method Multiple sources of ascertainment were used and children followed up with a standardized assessment including motor speech problems, swallowing/chewing difficulties, excessive drooling, and communication impairments at age 5 years. Results A total of 1357 children born between 1980 and 2001 were studied (781 males, 576 females; median age 5y 11mo, interquartile range 3–9y; unilateral spastic CP, n=447; bilateral spastic CP, n=496; other, n=112; Gross Motor Function Classification System [GMFCS] level: I, 181; II, 563; III, 123; IV, 82; IV, 276). Of those with ‘early‐onset’ CP (n=1268), 36% had motor speech problems, 21% had swallowing/chewing difficulties, 22% had excessive drooling, and 42% had communication impairments (excluding articulation defects). All impairments were significantly related to poorer gross motor function and intellectual impairment. In addition, motor speech problems were related to clinical subtype; swallowing/chewing problems and communication impairments to early mortality; and communication impairments to the presence of seizures. Of those with CP in GMFCS levels IV to V, a significant proportion showed a decline in the rate of motor speech impairment (p=0.008) and excessive drooling (p=0.009) over time. Interpretation These impairments are common in children with CP and are associated with poorer gross motor function and intellectual impairment.