Developmental Medicine and Child Neurology最新文献

Clinicians and families regularly enter into a process of shared decision-making. Seldom, if ever, however, is the critical question of time usage, or, more specifically, time-related burdens, accounted for when establishing goals and outcome measures. Time-related burdens are not included, for instance, as an outcome measure in cerebral palsy research-something which may have profound effects about which we are unaware. By contrast, in the field of oncology, time-related burdens, or, more technically, what has been termed time toxicity, has been increasingly studied. Building on that work, we seek to apply the concept of time toxicity to people with disabilities who interface with healthcare at great frequency.

Aim: To investigate the prevalence of eating and drinking difficulties as classified with the Eating and Drinking Ability Classification System (EDACS) in a large population-based cohort of children with cerebral palsy (CP) at all levels of motor function, and how EDACS classifications relate to undernutrition.

Method: This was a cross-sectional study based on data from the Swedish CP follow-up programme of children aged 19 years and younger. EDACS ratings were compared to z-scores for weight for age and height for age, calculated using standard references. Regression models were used to estimate how height for age was affected by EDACS levels when controlling for gross motor function and CP subtype.

Results: We included 2280 children with CP (945 females, 1335 males), median age 10 years 2 months. Almost a third (30.6%) had safety concerns during mealtimes (EDACS levels III-V). Most children (57.5%) could eat and drink independently, 20.2% required assistance, and 22.4% were totally dependent. One in five had undernutrition (19.9%) and there was an association between EDACS level and nutrition (p < 0.001). Height for age decreased from EDACS level II and also when controlling for gross motor function and CP subtype.

Interpretation: Almost one in three children with CP have dysphagia. Growth is affected in children with CP already from EDACS level II.

Aim: To assess the interrater reliability, concurrent validity, and responsiveness of the Children's Eating and Drinking Activity Scale (CEDAS).

Method: Interrater reliability was assessed using an online questionnaire completed by speech and language therapists working in hospital and community settings. Participants (n = 39) applied CEDAS to 12 vignettes. Interrater reliability was calculated using Krippendorff's alpha (α). Additionally, CEDAS was applied retrospectively to consecutive speech and language therapy referrals at a single hospital (n = 85, median age 11 years 2 months, range 1 week-17 years 1 month). Responsiveness was assessed by comparing median CEDAS scores at referral, after initial speech and language therapy assessment, and at discharge. The Functional Status Scale (FSS) feeding domain was used to evaluate concurrent validity.

Results: Interrater reliability was excellent (Krippendorff's α = 0.927). CEDAS was responsive to change, with expected score increases seen between referral and discharge (Z = -4.37, p < 0.001), and initial assessment and discharge (Z = -3.23, p < 0.001). Strong concurrent validity was established with the FSS (r_s = -0.77 to -0.99, p < 0.003).

Interpretation: CEDAS demonstrates excellent interrater reliability across clinical settings. It is a responsive tool with established construct validity when used in an acute hospital setting.

Aim: To describe individuals with cerebral palsy (CP) followed at the SARAH Network of Rehabilitation Hospitals in Brazil.

Method: This was a multicentre, cross-sectional, descriptive study. Individuals diagnosed analysis (n = 17 771).

Results: The mean age at the first visit was 3 years 5 months (SD = 3 years 1 month), with 55.3% participants being male. At birth, 52.0% had normal weight and 50.3% were born at term. Perinatal risk factors were the most prevalent: preterm birth (48.4%); low birthweight (48.0%); and hypoxic-ischaemic encephalopathy (40.1%). Spastic CP (75.5%), bilateral involvement (75.9%), and Gross Motor Function Classification System levels III to V (57.8%) were predominant.

Interpretation: Demographic and clinical data are comparable with previous reports from low- and middle-income countries (LMICs). Despite clinical advances in prenatal and perinatal care, most individuals with CP had severely affected motor function. As the prevalence of CP is higher in LMICs, and considering the direct influence of psychosocial factors on children with CP, it is essential to support multicentre and national registries in LMICs to adapt family-centred rehabilitation planning guidelines.

After treatment with new disease-modifying therapies, cognitive and neurodevelopmental aspects have been observed in individuals with spinal muscular atrophy (SMA). Emerging evidence suggests that children with SMA type 1 may experience cognitive, language, and behavioural delays, with reported rates of neurodevelopmental difficulties ranging from 9% to 60%. However, the evidence is inconclusive because of diagnostic inconsistencies and challenges in defining and categorizing cognitive, behavioural, and broader neurodevelopmental disorders. Some of these challenges are related to the difficulties in assessing children with severe motor, communication, or fatigue-related impairments. Despite neurodevelopmental and cognitive aspects being relatively frequent in SMA type 1, the lack of systematic assessment methodologies and the presence of motor-related testing biases hinder efforts to distinguish true neurodevelopmental disorders from the secondary effects of motor limitations, thus highlighting the unmet need of dedicated tools.

Aim: To systematically review the factors that predict changes in upper-extremity motor function in response to constraint-induced movement therapy (CIMT) or bimanual training in children with cerebral palsy (CP).

Method: The PubMed, Cochrane, Embase, Scopus, CINAHL, and PEDro databases were searched. Studies were included if predictor variables were measured at baseline and were linked to an upper-extremity motor function outcome after intervention. Studies investigating only neurophysiological biomarkers were excluded. Two independent reviewers conducted study selection, risk of bias assessment, data extraction, and synthesis.

Results: The electronic search yielded 4317 articles, of which 23 were included (14 of 23 were randomized controlled trials). The most frequently studied factors were baseline motor outcomes (19 studies), age (19 studies), behavior (six studies), sex (six studies), and affected side (six studies), with inconsistent evidence to support an association with changes in upper-extremity motor function after CIMT or bimanual training. Cognitive deficits, somatosensory deficits, and mixed effects of predictive factors were rarely studied.

Interpretation: Based on current evidence, it is inconclusive whether children with CP benefit from CIMT or bimanual training, regardless of their baseline upper-extremity motor function, age, sex, affected side, or behavior.

Aim: To explore motor impairments linked to developmental coordination disorder (DCD) in children with childhood apraxia of speech (CAS).

Method: This prospective cohort study included 105 children (79 male, aged 5-15 years) with CAS. Assessments included the Developmental Coordination Disorder Questionnaire, Revised, the visuomotor precision subtest of the Neuropsychological Assessment, Second Edition, the motor subtest of the Vineland Adaptive Behaviour Scales, Third Edition (Vineland-3), and Full-scale IQ (FSIQ) scores. Data analysis used multiple imputation for missing data, along with descriptive statistics, repeated-measures analysis of variance, multiple binary logistic regression, and receiver operating characteristic (ROC) analyses.

Results: Eighty-two per cent of participants showed motor impairments; 63% screened positive for DCD risk; and 71% had visuomotor precision impairments. Fine-motor scores were lower than gross-motor and coordination scores. The logistic regression model accurately predicted DCD risk at 73%, identifying the Vineland-3 (odds ratio [OR] = 7.17) and visuomotor precision subtest (OR = 4.90) as significant predictors, whereas the FSIQ was not. The Vineland-3 showed a high false negative rate (46%) for DCD risk, and the ROC analysis suggested a higher cut-off point for improved prediction accuracy.

Interpretation: This study highlights a high prevalence of DCD risk and visuomotor precision impairments among children with CAS, independent of FSIQ. The Vineland-3 motor subtest is not recommended for DCD assessment, whereas screening for DCD is essential for targeted interventions in CAS.