Developmental Medicine and Child Neurology最新文献

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.

Aim: This study aimed to identify determinants associated with how individuals with cerebral palsy (CP) access hospital-based health services in Victoria, Australia.

Method: This was a retrospective observational study interrogating linked administrative health data sets. Data describing service type, access frequency, and individual predisposing and enabling factors were identified for individuals with CP (2019-2023). Factors associated with service use were examined using negative binomial regression models.

Results: Data describing 284 individuals (mean age 40 years 7 months) were identified. Health service encounters by participants ranged from 1 to 163 times. Living in supported accommodation increased likelihood of accessing any health service (incidence rate ratios [IRR] 1.51; 95% confidence interval [CI], 1.04-2.19) including emergency care (IRR 1.90; 95% CI 1.35-2.68). Whilst younger adults were less likely to access any health service compared to children (IRR 0.67; 95% CI 0.46-0.99), being older increased likelihood of inpatient admission (IRR 2.39; 95% CI 1.65-3.46).

Interpretation: Living in supported accommodation increased the likelihood of any health service access. Older adults were more likely to access inpatient care, and younger adults were less likely to access any health service. Greater knowledge of how individual predisposing and enabling factors influence service use in geographical contexts, disability complexity, and life course is needed for optimal health outcomes.

Gastroenterological disorders and hepatic (liver) diseases can affect the health and quality of life of adults with cerebral palsy (CP), but not much is known about how often certain gastroenterological disorders (gastroesophageal reflux disease [GERD], constipation, dysphagia [swallowing problems], fecal incontinence, dental/oral cavity disorders, colorectal cancer) or hepatic diseases are present. It is also not known whether some treatments are more effective than others in these adults.

Six databases were searched for research studies published since 1990 that reported information on specific gastroenterological disorders or hepatic diseases in adults with CP and met other criteria. Study information was extracted and summarized, and confidence in results was rated based on factors like the number of people included and how the study was done (methods).

In total 32 studies (30 different samples) were found describing one or more specific gastroenterological disorders or hepatic diseases. Most (25) studies described prevalence of disorders in adults with CP: GERD (prevalence 3%–42%), constipation (4%–67%), dysphagia (6%–77%), fecal incontinence (6%–29%), dental/oral cavity disorders (25%–53%), and aggregated hepatic diseases (1%–6%). No studies described colorectal cancer prevalence. GERD, dysphagia, and hepatic disease prevalence was higher in adults with CP than those without. Fecal incontinence prevalence was greater in persons with worse gross motor function. Four studies describing treatments for oral/dental health or dysphagia were found, but certainty of evidence for the intervention effectiveness was low to very low.