Developmental Medicine and Child Neurology最新文献

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.

Cerebral palsy (CP) is a condition that affects movement and posture. It is the most common lifelong physical disability that begins in childhood. To improve care, services, and research for children with CP, it is important to collect consistent and reliable information about their health, development, and the support they receive.

This scoping review looked at 38 research papers from 11 Arabic-speaking countries (ASCs) to understand what types of information – called common data elements (CDEs) – are being collected about children with CP. First, we gathered and reviewed all the CDEs reported in these studies. Second, we compared the CDEs with the data collected by three major international CP registry networks: the Australian Cerebral Palsy Register (ACPR), the Global Low- and Middle-Income Countries CP Registries (GLM-CPRs), and the Surveillance of Cerebral Palsy in Europe (SCPE). Third, we identified 12 key data elements that were consistently reported across ASC studies and all three international networks.

Most ASC studies included basic information such as the child's age, sex, and birth characteristics. However, fewer studies included information on children's functional abilities, family background, or access to rehabilitation services. This makes it harder to fully understand children's needs and how best to support them. Compared with international registries, the level of agreement was fair with ACPR and GLM-CPRs, and slight with SCPE.

The 12 CDEs consistently reported across all sources include: children's date of birth and sex; mother's age at delivery; place of birth, gestational age, birthweight, and multiple birth status; and clinical information such as MRI findings, postneonatal cause of CP, epilepsy, and functional classifications (GMFCS and MACS).

Dravet syndrome is a severe neurological disorder that causes early-onset epilepsy and developmental delay. In the first years of life, the epilepsy is extremely sensitive to fever. Even minor infections can trigger prolonged seizures that require emergency hospital care. These repeated hospitalizations are stressful for families and may negatively impact children's cognitive development. Despite current medications, many young children with Dravet syndrome continue to experience these frequent fever-related seizures.

This study explored whether intravenous immunoglobulin (IVIG), a treatment made from antibodies, could help reduce fever-related seizures in children with Dravet syndrome. The idea was simple: by strengthening the children's immune system with IVIG, they might have fewer infections, less fever, and therefore fewer prolonged seizures.

This retrospective study looked at 14 children with Dravet syndrome (aged 10 months to 8 years) who received IVIG infusions every 1 to 6 weeks. The researchers compared two 6-month periods: the 6 months before starting IVIG treatment and the 6 months during regular IVIG infusions. When comparing these two periods, there was a significant decrease in the number of hospital admissions for prolonged seizures. However, the treatment had side effects: nearly half of the children experienced fever or seizures during infusions, and several needed a central line (permanent venous access) because veins were difficult to access repeatedly.

Aim: To systematically review and characterize the spectrum of non-epileptic paroxysmal events in individuals with Rett syndrome (RTT).

Method: We conducted a descriptive systematic review of observational evidence. Searches were conducted across the PubMed, Embase, and OVID databases for studies published from January 1962 to September 2024. Eligible studies included case reports, case series, cohort studies, and small clinical trials that described non-epileptic events in individuals with clinically or genetically confirmed RTT. Data were extracted on study design, participant characteristics, and event types. Events were categorized into respiratory, neuromotor, and behavioural domains.

Results: Sixty-two studies met the inclusion criteria, representing a wide age range of individuals with RTT. The most frequently reported paroxysmal non-epileptic events were respiratory disturbances, including breath-holding and hyperventilation, followed by vacant spells, involuntary movements, and behavioural episodes such as agitation and inappropriate laughter. Discrepancies were noted between the diagnostic attribution of clinician-reported and family-reported events. The term 'Rett episodes' was used by a minority of authors.

Interpretation: A wide range of non-epileptic paroxysmal events requiring different treatment approaches are experienced by patients with RTT. Enhancing clinician awareness and developing clearer diagnostic frameworks are key to improving classification accuracy and preventing unnecessary treatment.