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Introduction: Current strategies to address shortages of rural doctors focus on developing a pipeline for rural generalist practice. Limited research has explored how doctors' professional journey engenders the skills required to practice rurally. Objective: This paper analyses how rural general practitioners' clinical pathway informs their scope of practice and future retention. Design: Qualitative thematic analysis using semi‐structured telephone interviews. Twenty‐one general practitioners appointed in their local health district of Murrumbidgee and Southern New South Wales, Australia, within the past 10 years. Participants comprised 10 Australian medical graduates (AMG) and 11 international medical graduates (IMG). Findings: AMGs and IMGs contrasted how their pathway into rural practice, and capacity to work rurally, informed their scope of practice. Australian medical graduates' familiarity with rural areas was consolidated through congruous experiences, including at rural clinical schools. Paradoxically, the fluency of their training limited the amount of unsupervised experience and confidence AMGs gained. Together with a focus on work‐life balance, this limited many to providing mainstream general practice, precluding extending their scope of practice. International medical graduates described disseminated experiences, often unsupervised in high‐volume contexts. However, a lack of professional opportunities prevented them from extending their scope of practice. Discussion: IMG and AMG motivation and pathway for working rurally differ. Respective cohorts have concerns regarding requisite skills and knowledge for rural practice, which incorporates opportunity and recognition. Entry points for training should be variable to allow consideration of life stage, prior skill development and extension of scope of practice. Conclusion: Doctors' scope of practice is informed by their pathways into rural practice. Australian medical graduates may not gain adequate competence during expedited training programs to confidently undertake extended clinical activities. International medical graduates, however lacked the opportunities and support, to utilise their expertise in rural practice. Complementarily utilising the expertise and commitment of both AMGs and IMGs may synergistically address workforce shortages. [ABSTRACT FROM AUTHOR]

Background: Acute and late toxicities in patients treated with (chemo)radiotherapy for head and neck cancer (HNC) is common and can negatively impact quality of life and performance. Performance status instruments measure the functional ability to perform daily life activities and are important tools in the oncologic population. Aims: Since Dutch performance status scales for the HNC population are lacking, we conducted this study to translate the Performance Status Scale for Head and Neck Cancer Patients (PSS‐HN) into Dutch (D‐PSS‐HN) and to validate this version. Methods & Procedures: The D‐PSS‐HN was translated into Dutch according to the internationally described cross‐cultural adaptation process. It was administered to HNC patients and together with the Functional Oral Intake Scale completed by a speech and language pathologist at five different time points during the first 5 weeks of (chemo)radiotherapy. Patients were asked each time to complete the Functional Assessment of Cancer Therapy and the Swallowing Quality of Life Questionnaire. Pearson correlation coefficients were used to calculate convergent and discriminant validity and the evolution of D‐PSS‐HN scores was assessed by means of linear mixed models. Outcomes & Results: A total of 35 patients were recruited and > 98% of the clinician‐rated scales were completed. Convergent and discriminant validity were demonstrated, with all correlations rs between 0.467 and 0.819, and between 0.132 and 0.256, respectively. The subscales of the D‐PSS‐HN are sensitive to detect changes through time. Conclusion & Implications: The D‐PSS‐HN is a valid and reliable instrument to assess performance status in patients with HNC treated with (chemo)radiotherapy. It is a useful tool to measure HNC patients' current diet level and functional abilities to perform daily life activities. WHAT THIS PAPER ADDS: What is already known on the subject: Acute and late toxicities in patients treated with (chemo)radiotherapy for HNC are common and can negatively impact quality of life and performance. Performance status instruments measure the functional ability to perform daily life activities and are important tools in the oncologic population. However, Dutch performance status scales for the HNC population are lacking. Therefore, we translated the Performance Status Scale for Head and Neck Cancer Patients (PSS‐HN) into Dutch (D‐PSS‐HN) and validated this version. What this paper adds to existing knowledge: We translated the PSS‐HN and demonstrated its convergent and discriminant validity. The subscales of the D‐PSS‐HN are sensitive to detect changes through time. What are the potential or actual clinical implications of this work?: The D‐PSS‐HN is a useful tool to measure HNC patients' functional abilities to perform daily life activities. The tool can easily be used in clinical settings: since data collection duration is very short, this facilitates clinical (and research‐related) implementation of the scale. Patients' individual needs could be identified by using the D‐PSS‐HN, resulting in more appropriate approaches and (early) referrals if needed. Interdisciplinary communication could be facilitated. [ABSTRACT FROM AUTHOR]

Background: Communication and cognitive impairments are known barriers to shared decision‐making. Most people diagnosed with motor neurone disease (MND) will develop a motor speech impairment over the disease course. Some will develop cognitive, linguistic or behavioural disturbance. Despite this, the impact of communication and cognitive impairment on personal healthcare decision‐making in MND is not well known. Aims: This exploratory, longitudinal study aimed to capture the perspectives of people living with MND (plwMND) and family members on managing their healthcare with, or in anticipation of, a communication impairment. Methods & Procedures: Semi‐structured interviews and functional assessments were conducted with plwMND and family members over one to three time points between December 2017 and January 2020. Participants were recruited from a specialist MND clinic using a maximum variation sampling approach. Interview transcripts were analysed using trajectory data analysis: a matrix‐based approach for thematic analysis of longitudinal data. The study was underpinned by interpretive descriptive methodology. Outcomes & Results: A total of 19 plwMND with a range of MND phenotypes and 15 family members were recruited. Disease progression and participant withdrawal resulted in attrition, however 12 plwMND and seven family members participated at all three time points. Consistent cognitive screening was not feasible, which limited the opportunity to explore the impact of cognitive change. An overarching theme 'Communicating takes effort' was identified and illustrates the efforts required to compensate for, or circumnavigate, impairments to maintain involvement in healthcare. Assistance from family and accommodation from healthcare professionals (HCPs) was needed for ongoing engagement. Where plwMND were dependent on alternative communication devices, this assistance was essential and primarily carried out by family members. Despite these efforts, the quality, quantity and accuracy of communication were sometimes compromised. Participants equated good communication with receiving good healthcare, and some expressed anxiety in the anticipation of being unable to express their needs to healthcare workers. Conclusion & Implications: Communication impairment has a direct impact on healthcare involvement. This study demonstrates the effort required by plwMND and their carers to maintain or maximize ongoing involvement. This effort may not always be visible to HCPs. This information may prompt clinicians to consider the best ways to conduct clinical consultations to accommodate patients' abilities. Compromised communication experiences can be moderated by accommodations and support from HCPs and appropriate adjustments in the health system. Asking patients about their communication preferences and needs, allowing extra time and conducting multidisciplinary sessions are examples of such support. What this paper adds: What is already known on this subject?: Communication and cognitive impairments are known contributors to negative health outcomes and barriers to shared decision‐making generally. The existing literature in decision‐making in MND does not address the specific impact of these impairments on personal healthcare involvement for plwMND and their carers. What this paper adds to existing knowledge?: This paper reports the findings of a research project that interviewed 19 plwMND and 15 carers on one to three occasions over a 26‐month period to obtain their perspectives of the impact of communication on healthcare involvement. Whilst a priori the intention was to look at both communicative and cognitive decline, only the former was achieved. The effort and often 'invisible' activity undertaken to manage or maintain involvement in healthcare is identified. Communication impairment requires support and accommodation, otherwise healthcare involvement can be compromised. Results show participants may associate effective communication with good healthcare. What are the potential or actual clinical implications of the work?: Clinicians may wish to use these insights from plwMND and their carers to guide adjustments to their professional practice to maximize healthcare involvement for their patients. Tailored education for different healthcare groups is needed to improve understanding of MND‐related communication impairments and supportive strategies so that involvement in healthcare is not compromised. [ABSTRACT FROM AUTHOR]

Background: The majority of children with cerebral palsy (CP) experience challenges in functional communication from a young age. A pivotal aspect of functional communication is language comprehension. A variety of classification systems and questionnaires are available to classify and describe functional communication skills in children with CP. A better understanding of the convergent validity of (subsections of) these tools, as well as their relationship with spoken language comprehension, will be valuable in both clinical practice and research. Aims: To investigate the convergent validity of (subsections of) functional communication tools and the relationship with spoken language comprehension in children with CP. Methods & Procedures: Cross‐sectional data on 138 children were subdivided into three developmental stages based on (Dutch) educational phases: ages 18 months–3;11y (n = 59), 4;0–5;11 years (n = 37) and 6;0–8;11 years (n = 42). The following functional communication tools were used to classify and describe functional communication: Communication Function Classification System (CFCS), subscales of the Caregivers Priorities and Child Health Index of Life with Disabilities—Dutch Version (CPCHILD‐DV) and the Focus on Communication Under Six‐34 (FOCUS‐34) questionnaire. Spoken language comprehension was assessed with the Computer‐Based instrument for Low motor Language Testing (C‐BiLLT). Correlations between the functional communication tools, and with the C‐BiLLT, were calculated using Pearson's and Spearman's correlation coefficients. It was hypothesized a priori that correlations of at least 0.60 suggest good convergent validity. Outcomes & Results: At all developmental stages, a significant ordered decreasing tendency of communication outcomes was found across CFCS levels; lower CFCS levels were associated with lower scores on the CPCHILD‐DV and FOCUS‐34, and with a lower level of spoken language comprehension (C‐BiLLT). Correlation coefficients of the functional communication tools exceeded 0.60 at all developmental stages. Correlations between C‐BiLLT raw scores and the functional communication tools varied between 0.351 and 0.591 at developmental stage 18 months–3;11 years, between 0.781 and 0.897 at developmental stage 4;0–5;11 years, and between 0.635 and 0.659 at developmental stage 6;0–8;11 years. Conclusions & Implications: The functional communication tools assessed in this study showed convergent validity at all developmental stages. The CFCS, currently most widely used in paediatric rehabilitation, is adequate in the classification of functional communication. However, for more detailed clinical goal setting and evaluation of change in functional communication, the additional use of FOCUS‐34 or CPCHILD‐DV is recommended. What this paper adds: What is already known on the subject: A range of functional communication tools are available that help describe and classify functional communication in children with CP. These include the CFCS, subsections of CPCHILD‐DV and FOCUS‐34. The CFCS classifies functional communication in daily life with familiar and unfamiliar partners. Specific subsections of the CPCHILD‐DV and FOCUS‐34 include items that pertain to communicative participation. The innovative C‐BiLLT provides a standardized method to assess spoken language comprehension in children with CP and significant motor impairments. What this paper adds to existing knowledge: In the present study, convergent validity was confirmed between CFCS and specific subsections of the CPCHILD‐DV and FOCUS‐34. Correlations between these functional communication tools and the C‐BiLLT were moderate to strong. What are the potential or actual clinical implications of this work?: For clinical and research purposes (for instance, accurate prescription of augmentative and alternative communication—AAC), healthcare and educational professionals together with parents need to know how functional communication tools converge and how functional communication levels relate to the comprehension of spoken language. The CFCS provides a valid classification of functional communication abilities in children with CP. However, to measure change in functional communication and to evaluate treatment outcomes, use of additional functional communication tools such as the CPCHILD‐DV and FOCUS‐34 is recommended. When discrepancies are found between communicative abilities and spoken language comprehension, it is strongly recommended that valid tools are used in a more detailed examination of the child's spoken language comprehension skills and functional communication. [ABSTRACT FROM AUTHOR]

Purpose To examine the nature of the interruptions during medication work. Design An exploratory descriptive design was used with 28 nurses during 15 medication rounds in the medical, surgical, and gynecology wards at a 1000‐bed tertiary teaching hospital in Saudi Arabia. Methods: Non‐participant prospective observational and follow‐up interview methods were used to collect data. A standard paper‐based tool and digital stopwatch to count the interruptions were used. Nurses' sources, the secondary tasks undertaken, and the impacts of the interruptions were considered. The data were analyzed via descriptive analysis. Results: A total of 87 medication‐related events occurred. In these, 182 interruptions accounted for 90.0% of the total. Interruptions were more frequent during medication administration and often occurred in the corridors and patients' rooms. Nurses, medical officers, and impediments were frequent sources of interruptions. The secondary tasks attended by the nurses were mostly related with impediment resolutions and patients' needs. System failures appeared to be associated with clinical and procedural errors. Conclusion: The results of this study provide information regarding the course of interruptions during medication work in a Saudi Arabian hospital. Nurses were the main source of interruptions related with the provision of patient care and resolution of impediments. Interruptions tended to be associated with clinical errors and procedural failures due to system failure. Promoting a culture of respect and workplace etiquette in global healthcare settings demands minimizing non‐life‐threatening interruptions during nursing works. Clinical relevance: Strategies used in hospital management systems that affect nursing, pharmacies, human resources, and ancillary service department collaborations should be monitored for effectiveness and to ensure patient safety. Various approaches toward medication management systems, such as the utilization of an Omnicell automatic dispensing machine and cabinets within each patient's room, as well as others, such as wearing a "no‐interruption zone" sign, could be tailored and matched to the context of the practice to reduce interruptions. [ABSTRACT FROM AUTHOR]

Aim: To determine if walking ability and presence of intellectual disability and epilepsy change from childhood to 50 years of age in individuals with cerebral palsy (CP), and if such changes are related to age, sex, or CP subtype.Method: This was a population-based follow-up study of 142 adults born from 1959 to 1978 (82 males, 60 females; mean age 48y 4mo, range 37-58y; 44% unilateral, 35% bilateral, 17% dyskinetic, and 4% ataxic CP) listed in the CP register of western Sweden. We compared childhood data with a follow-up assessment in 2016.Results: At follow-up, walking ability had changed significantly (p<0.001). The proportion of participants walking without aids had decreased from 71% to 62%, and wheelchair ambulation increased from 18% to 25%. Walking ability was related to subtype (p=0.001), but not to age, sex, pain, fatigue, or body mass index. The proportion classified as having intellectual disability had increased from 16% to 22% (p=0.039) and the proportion with epilepsy from 9% to 18% (p=0.015). Of those with childhood epilepsy, 46% were seizure-free without medication.Interpretation: Walking ability and the presence of intellectual disability and epilepsy had changed significantly since childhood. Life-long access to specialized health care is warranted for re-evaluation of impairments, treatment, and assistance. What this paper adds Changes in impairments in individuals with cerebral palsy (CP) over time are related to CP subtype. After 50 years, walking ability in CP may have deteriorated or improved. Intellectual disability in CP may not always be detected in early childhood assessments. Epilepsy in CP may develop after childhood or may be outgrown. [ABSTRACT FROM AUTHOR]

Aim: To evaluate an innovative paediatric neurorehabilitation model in relation to improving quality of neurorehabilitation and reducing length of stay (LOS) for children with acquired brain injury.Method: A process evaluation approach was conducted in line with Medical Research Council evaluation of complex interventions guidance. Analysis was conducted on routinely collected patient data from 2017 to 2018, including LOS and family feedback. Descriptive and inferential statistics were used for quantitative analysis and qualitative data was analysed thematically.Results: Outcomes for 70 children (0-16y, median age 5y, IQR 1-11y, 46 males, 24 females) referred to the service indicated improved function and reduced complexity of need. The mean LOS was 10.6 days compared to baseline mean LOS of 41 days (2011-2012). High satisfaction from the families was recorded; however, ongoing needs and service gaps regarding long-term support were identified.Interpretation: This service model is effective in delivering quality paediatric neurorehabilitation, demonstrating a sustained impact on LOS, and positive patient outcome data and family feedback for this group of patients. What this paper adds Investment in early intensive neurorehabilitation and supported discharge impacts length of stay (LOS) for children with acquired brain injury. Early intensive neurorehabilitation and supported discharge is effective. This is demonstrated by a sustained reduction in LOS, positive patient outcomes, and family feedback. [ABSTRACT FROM AUTHOR]

Violent ideations are increasingly recognized as an important psychological predictor for aggressive and violent behavior. However, little is known about the processes that contribute to violent ideations. This paper examines the extent to which polyvictimization triggers violent ideations in late adolescence and early adulthood, while also adjusting for dispositional and situational factors as well as prior violent ideations. Data came from three waves of the Zurich Project on the Social Development from Childhood into Adulthood (z-proso; n = 1465). Full-information maximum likelihood Tobit models were fitted to regress violent ideations experienced at ages 17 and 20 on multiple victimization experiences in the preceding 12 months while controlling for antecedent developmental risk factors and prior violent ideations. The results showed that violent ideations in late adolescence and early adulthood are influenced by violent thoughts, aggressive behavior, violent media consumption, moral neutralization of violence, and internalizing symptoms measured 2 years earlier. Experiences of polyvictimization significantly contributed to an increase in violent ideations both during late adolescence and in early adulthood. The exposure-response relationship between victimization and violent ideations did not significantly differ by sex. The findings are consistent with the notion that violent ideations are triggered by a retaliation-linked psychological mechanism that entails playing out other directed imaginary aggressive scenarios specifically in response to experiencing intentional harm-doing by others. [ABSTRACT FROM AUTHOR]

Aims: Patients surviving an acute myocardial infarction (AMI) are at risk of developing symptomatic heart failure (HF) or premature death. We hypothesized that sacubitril/valsartan, effective in the treatment of chronic HF, prevents development of HF and reduces cardiovascular death following high-risk AMI compared to a proven angiotensin-converting enzyme (ACE) inhibitor. This paper describes the study design and baseline characteristics of patients enrolled in the Prospective ARNI vs. ACE inhibitor trial to DetermIne Superiority in reducing heart failure Events after Myocardial Infarction (PARADISE-MI) trial.Methods and Results: PARADISE-MI, a multinational (41 countries), double-blind, active-controlled trial, randomized patients within 0.5-7 days of presentation with index AMI to sacubitril/valsartan or ramipril. Transient pulmonary congestion and/or left ventricular ejection fraction (LVEF) ≤40% and at least one additional factor augmenting risk of HF or death (age ≥70 years, estimated glomerular filtration rate <60 mL/min/1.73 m2 , diabetes, prior myocardial infarction, atrial fibrillation, LVEF <30%, Killip class ≥III, ST-elevation myocardial infarction without reperfusion) were required for inclusion. PARADISE-MI was event-driven targeting 708 primary endpoints (cardiovascular death, HF hospitalization or outpatient development of HF). Randomization of 5669 patients occurred 4.3 ± 1.8 days from presentation with index AMI. The mean age was 64 ± 12 years, 24% were women. The majority (76%) qualified with ST-segment elevation myocardial infarction; acute percutaneous coronary intervention was performed in 88% and thrombolysis in 6%. LVEF was 37 ± 9% and 58% were in Killip class ≥II.Conclusions: Baseline therapies in PARADISE-MI reflect advances in contemporary evidence-based care. With enrollment complete PARADISE-MI is poised to determine whether sacubitril/valsartan is more effective than a proven ACE inhibitor in preventing development of HF and cardiovascular death following AMI. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the properties of the Infant Motor Activity Log (IMAL), a caregiver-report for frequency and quality of use of more affected upper extremity in infants with neurological and functional impairments.Method: This was a prospective cohort study of 66 children (34 females, 32 males) aged 6 to 24 months (mean age [SD] 13.7mo [5.3]) with neurological and functional impairments and a confirmed cerebral palsy diagnoses after 2 years, and 51 age-matched typically developing children. The IMAL was administered at baseline and 4 weeks later. Typically developing infants were tested with randomly assigned 'more affected' upper extremity. Psychometric properties were evaluated using Spearman's correlation coefficient, Cronbach's alpha, and Jonckheere-Terpstra tests.Results: In the children with impairments, the IMAL showed internal consistency (alpha≥0.88) for the How Well Scale (HWS) and How Often Scale (HOS). Test-retest reliability was 0.64 (HOS) and 0.70 (HWS), demonstrating stability over time. Correlation with Bayley Scales of Infant and Toddler Development, Third Edition more affected arm raw scores were 0.70 (HOS) and 0.72 (HWS) (p<0.001) demonstrating construct validity. Both scale scores decreased with increasing Gross Motor Function Classification System and Mini-Manual Ability Classification System (p<0.001) levels, supporting discriminative validity. Discrimination between typically developing infants and infants with impairments was high (HWS: area under the receiver operating characteristic curve [AUC] 0.96, 95% confidence interval [CI] 0.94-0.99 and HOS AUC=0.95, CI 0.92-0.99).Interpretation: The IMAL is a valid and reliable discriminative caregiver measure of upper limb performance and may complement measures of capacity in infants with neurological and functional impairments.What This Paper Adds: The Infant Motor Activity Log (IMAL) is a valid and reliable measure of caregiver perception of upper limb function. The IMAL fills a measurement gap for infant motor performance in children with impairments. The IMAL discriminates among motor function levels. [ABSTRACT FROM AUTHOR]

Aim: To investigate the utility of the Insight Inventory (a structured clinical inventory completed by caregivers) for assessment of children with cerebral visual impairment; and to investigate effectiveness of tailored habilitational strategies derived from the responses to the Insight Inventory.Method: Fifty-one eligible children (26 males, 25 females; mean age 9y 5mo, SD 3y, range 5-16y) were recruited from Great Ormond Street Hospital, London. They underwent baseline assessment including neuro-ophthalmological and neuropsychological evaluations, and parent- and child-reported ratings on a questionnaire-based measure of quality of life. Parents also completed the Insight Inventory. On the basis of responses to the Inventory, families received individualized habilitational strategies. Follow-up assessments 6 months later included repeating the Insight Inventory and quality of life questionnaires.Results: Correlations were found between the Insight Inventory and the Wechsler Intelligence Scale for Children, Fourth Edition, the Beery-Buktenica Test of Visual-Motor Integration, and the Benton Facial Recognition Test, suggesting that the Insight Inventory is an effective tool to estimate visual-perceptual difficulties. At 6 months follow-up, caregiver reports indicated significant improvements in the quality of life of children below the age of 12 years.Interpretation: The Insight Inventory is a simple questionnaire which covers practical aspects of cognitive visual function in everyday life. It provides in-depth information about the aspects that children struggle with. It can also guide programmes of individualized habilitation strategies, which may enhance the quality of life of younger children.What This Paper Adds: Questionnaire scores demonstrate biologically plausible correlations with formal neuropsychological tests of visual function. After administration of matched practical habilitational strategies, younger children showed improvement in quality of life and functional vision scores. [ABSTRACT FROM AUTHOR]

Aim: To describe the trajectory of clinical signs in children who developed human immunodeficiency virus encephalopathy (HIVE) after starting early antiretroviral therapy (ART).Method: This was a retrospective case-cohort description of HIVE among Cape Town participants from the Children with HIV Early AntiRetroviral treatment (CHER) trial. Criteria for HIVE diagnosis were at least two of: (1) acquired central motor deficit, (2) impaired brain growth, and (3) failure to attain or loss of developmental milestones in the absence of an alternative aetiology.Results: Of 133 surviving participants who initiated ART at a median age of 9 weeks and who were followed until a median age of 6 years, 20 (12%) developed HIVE at a median age 31 months (interquartile range 19-37). In these, the first neurological deterioration was noticed at a median age of 19 months, when 16 were on ART and nine had undetectable HIV viral load for a median of 12 months. Signs of upper motor neurons were present in 18, of whom 12 resolved and four had persistent spastic diplegia; 19 had motor delay, of whom 14 resolved; 12 had language delay, of whom 11 resolved; and 16 had impaired brain growth, of whom only five recovered. For the 16 participants already on ART at HIVE diagnosis, regimens were not altered in response to diagnosis.Interpretation: HIVE may occur despite early ART initiation and virological suppression and then resolve on unchanged ART, most likely as intrathecal inflammation subsides.What This Paper Adds: Despite suppressive antiretroviral therapy, children can develop human immunodeficiency virus encephalopathy, The most common manifestations are motor deficits and impaired brain growth. Most experience improvement, with many resolving without additional intervention. [ABSTRACT FROM AUTHOR]

Aim: To analyse the development of motor skill and executive function in school-aged children with and without developmental coordination disorder (DCD).Method: Using a longitudinal design, 186 children (86 males, 100 females) aged 6 to 11 years at Time 1 were tested over a 2-year period, 52 of whom were diagnosed with DCD at Time 1 (27 males, 25 females; mean age 8y 5mo, SD 1y 6mo) using DSM-5 criteria. The McCarron Assessment of Neuromuscular Development assessed motor status at Time 1 and at 2-year follow-up (Time 2). Executive function was assessed using a well-validated measure, the Groton Maze Learning Test.Results: The DCD cohort at Time 1 had moderate incidence of executive function deficit (41%). Most importantly, at a group level, children with persisting DCD (across Times 1 and 2) also showed significantly lower levels of executive function than children with typical motor development at both time points. At an individual level, around 26% of children in this group had persisting executive function deficits relative to normal ranges of performance.Interpretation: Children with persisting DCD are at significant risk of executive function issues. The combination of motor and cognitive issues as a potential risk factor in the longer-term development of children is discussed.What This Paper Adds: Around half of children initially diagnosed with developmental coordination disorder (DCD) had the same diagnosis at 2-year follow-up. 41% of children with DCD have impaired executive function. Children with persisting DCD show poorer executive function than those with typical motor development or remitting DCD. [ABSTRACT FROM AUTHOR]

Aim: To examine the efficacy of Hand-Arm Bimanual Intensive Therapy (HABIT) on daily functioning, unimanual dexterity, and bimanual performance of children with bilateral cerebral palsy (CP) compared with customary care.Method: Forty-one children with bilateral CP, aged 4 to 16 years, classified in levels I to III of the Manual Ability Classification System, were randomly assigned to HABIT (90h) (n=21) or to customary care (4.5h) (n=20). Participants' daily functioning (Pediatric Evaluation of Disability Inventory [PEDI], Canadian Occupational Performance Measure [COPM]), unimanual dexterity (Jebsen-Taylor Test of Hand Function, Box and Blocks Test [BBT]), and bimanual performance (Both Hands Assessment) were assessed pre-, post-, and 6 months after the intervention. Linear mixed-effects models were used for inferential analysis.Results: Children participating in HABIT showed greater improvements in daily functioning (COPMperformance : χ12 =9.50, p<0.01; COPMsatisfaction : χ12 =5.07, p<0.05; PEDIfunctional skills : χ12 =6.81, p<0.01; PEDIcaregiver assistance : χ12 =6.23, p<0.05) and in the dexterity of the dominant hand (BBT: χ12 =3.99, p<0.05) compared with children maintaining customary care. Group or time effects did not explain any variance in bimanual performance or in the dexterity of the non-dominant hand.Interpretation: HABIT may be beneficial for children with bilateral CP, with benefits evidenced for daily functioning outcomes.What This Paper Adds: Hand-Arm Bimanual Intensive Therapy (HABIT) improved daily functioning of children with bilateral cerebral palsy (CP). Bimanual performance, measured by the Both Hands Assessment, did not change after HABIT in children with bilateral CP. Children with asymmetric and symmetric hand use exhibited similar improvements after HABIT. [ABSTRACT FROM AUTHOR]

Aim: To describe the cognitive development of children with neurofibromatosis type 1 (NF1) and plexiform neurofibromas, and identify predictors of cognitive development.Method: Participants included 88 children with NF1 and plexiform neurofibromas (50 males, 38 females, aged 6-18y, mean=12y, SD=3y 7mo) on a natural history study at the National Cancer Institute. Neuropsychological assessments (e.g. IQ, academic achievement, attention, and executive functioning) were administered three times over 6 years.Results: Relative to normative peers, the total sample of children with NF1 and plexiform neurofibromas demonstrated significantly lower scores in most cognitive domains and decreasing z-scores over time in math, writing, inhibitory control, and working memory. Children who had parents with (vs without) NF1 were more likely to experience decreased z-scores in performance IQ, reading, writing, attention, and working memory. Higher (vs lower) parental education was related to higher levels of IQ, math, reading, and cognitive flexibility and a slower decrease in math z-scores. Children's sex and the number of NF1 disease-related complications were not related to most cognitive outcomes.Interpretation: Children with NF1 and plexiform neurofibromas are at high risk for cognitive difficulties and declining z-scores in various domains of cognitive functioning over time. The findings highlight the need for a better understanding of the within-group differences in these children and their need for individualized educational plans.What This Paper Adds: Math, writing, inhibitory control, and working memory scores decreased over time. The proportion of children with clinically significant cognitive deficits increased over time. Parental neurofibromatosis type 1 and low education were related to greater cognitive difficulties in children. [ABSTRACT FROM AUTHOR]

Aim: To examine the association between brain magnetic resonance imaging (MRI) characteristics and executive function and bimanual performance in children with unilateral cerebral palsy (CP).Method: Clinical MRI brain scans were classified as: (1) predominant pathological pattern (normal, white matter injury [WMI]; grey matter injury; focal vascular insults [FVI]; malformations; or miscellaneous); and (2) focal lesions (frontal, basal ganglia, and/or thalamus). Assessments included: (1) bimanual performance; (2) unimanual dexterity; and (3) executive function tasks (information processing, attention control, cognitive flexibility, and goal setting) and behavioural ratings (parent).Results: From 131 recruited children, 60 were ineligible for analysis, leaving 71 children (47 males, 24 females) in the final sample (mean age 9y [SD 2y], 6y-12y 8mo). Brain MRIs were WMI (69%) and FVI (31%); and frontal (59%), thalamic (45%), basal ganglia (37%), and basal ganglia plus thalamic (21%). Bimanual performance was lower in FVI versus WMI (p<0.003), and with frontal (p=0.36), basal ganglia (p=0.032), and thalamic/basal ganglia lesions (p=0.013). Other than information processing, executive function tasks were not associated with predominant pattern. Frontal lesions predicted attention control (p=0.049) and cognitive flexibility (p=0.009) but not goal setting, information processing, or behavioural ratings.Interpretation: Clinical brain MRI predicts cognitive and motor outcomes when focal lesions and predominate lesion patterns are considered. What this paper adds Early brain magnetic resonance imaging (MRI) predicts bimanual performance and cognitive outcomes. Brain MRI may identify children requiring targeted interventions. Basal ganglia with/without thalamic lesions predicted bimanual performance. Frontal lesions were associated with attention control and cognitive flexibility. Brain MRI predominant patterns predicted motor, not cognitive outcomes, other than information processing. [ABSTRACT FROM AUTHOR]

Aim: To determine whether epilepsy surgery improved health-related quality of life (HRQoL) and whether seizure freedom after surgery mediated the improvement in HRQoL.Method: This multicenter cohort study compared HRQoL after epilepsy surgery to pharmacological management in children with drug-resistant epilepsy (DRE). HRQoL was measured using the Quality of Life in Childhood Epilepsy (QOLCE) questionnaire at baseline and 1-year follow-up. The mediator between treatment type and HRQoL was seizure freedom.Results: Two hundred and thirty-seven patients were recruited (surgery group: n=147 [92 males, 45 females]; pharmacological group: n=90 [53 males, 37 females]). Mean age at seizure onset was 6 years (SD 4y 4mo) in the surgical group and 6 years 1 month (SD 4y) in the pharmacological group. The odds ratio of seizure freedom was higher for the surgery versus pharmacological group (β=4.24 [95% confidence interval {CI}: 2.26-7.93], p<0.001). Surgery had no direct effect on total QOLCE score at 1-year (β=0.24 [95% CI -2.04 to 2.51], p=0.839) compared to pharmacological management, but had an indirect effect on total QOLCE that was mediated by seizure freedom (β=0.92 [95% CI 0.19-1.65], p=0.013), adjusting for baseline total QOLCE score. Surgery had a direct effect on improving social function (p=0.043), and an indirect effect on improving physical function (p=0.016), cognition (p=0.042), social function (p=0.012) and behavior (p=0.032), mediated by seizure freedom.Interpretation: Greater seizure freedom achieved through epilepsy surgery mediated the improvement in HRQoL compared to pharmacological management in children with DRE.What This Paper Adds: Seizure freedom is higher after pediatric epilepsy surgery compared to pharmacologically managed epilepsy. Surgery indirectly improves health-related quality of life (HRQoL) mediated by seizure freedom compared to pharmacological management. Surgery has a direct effect on improving social function relative to pharmacological management. Baseline HRQoL was an important predictor of HRQoL after treatment. [ABSTRACT FROM AUTHOR]

Aim: To examine self- and proxy-reported symptoms of depression in children with epilepsy.Method: This was a prospective longitudinal cohort study of children with epilepsy. Participants were treated at six Canadian tertiary-care centers and followed over 28 months with repeated assessments of child self-reported symptoms of depression using the Children's Depression Inventory Short-Form (CDI-S). Trajectories of symptoms of depression were estimated using linear mixed effects (LME) modeling.Results: At baseline, 477 children had complete data (mean age [SD] 11y 5mo [2y 1mo], range 7y 7mo-15y 1mo; 234 females, 243 males). Mean CDI-S T score at baseline was 45.7 (SD=7.5) and at 28 months was 44.9 (SD=8.2), both were within the 'average' range. Results from LME modeling revealed mean raw CDI-S score of 1.897, corrected for age 10 years (corresponding to T scores slightly below the normed mean of 50), with no significant change over three measurements (slope=-0.113, p=0.135), indicating that CDI-S scores were stable over 28 months. Children with high initial CDI-S scores had lower subsequent scores, as demonstrated by the correlation of -0.827 between intercept and slope (p<0.001). Parents reported comparable findings.Interpretation: Self- and proxy-reported symptoms of depression were generally low and stable over an extended follow-up period. Normalization of scores was seen upon repeated assessment, even in children with higher scores of symptoms of depression at one point. These findings speak to the value and importance of repeated assessment over time.What This Paper Adds: In children with epilepsy, self- and proxy-reported symptoms of depression were generally low and stable over 28 months. The trajectory of symptoms of depression was not associated with seizure severity, whether considering the frequency or type of seizures. Parents' reports of symptoms of depression were comparable to the children's self-evaluations. [ABSTRACT FROM AUTHOR]

Aim: To compare the rate of falls between adults with and without cerebral palsy (CP).Method: We used primary care data on 1705 adults with CP and 5115 adults without CP matched for age, sex, and general practice attended. We compared odds of experiencing a fall between adults with and without CP using conditional logistic regression. We compared the rate of falls using a negative binomial model.Results: Participants were 3628 males (53%) and 3192 females (47%) (median age 29y, interquartile range 20-42y) at the start of follow-up. Follow-up was 14 617 person-years for adults with CP and 56 816 person-years for adults without CP. Of adults with CP, 15.3% experienced at least one fall compared to 5.7% of adults without CP. Adults with CP had 3.64 times (95% confidence interval [CI] 2.98-4.45) the odds of experiencing a fall compared to adults without CP. The rate of falls was 30.5 per 1000 person-years and 6.7 per 1000 person-years for adults with and without CP respectively (rate ratio 5.83, 95% CI 4.84-7.02) INTERPRETATION: Adults with CP are more likely to fall, and fall more often, than adults without CP. The causes and consequences of falls in adults with CP need examination.What This Paper Adds: Twenty adults with CP and 5.3 adults without CP experienced at least one fall per 1000 person-years. Adults with CP experienced 30.5 falls per 1000 person-years compared to 6.7 falls per 1000 person-years among adults without CP. Adults with CP had 3.64 times the odds of experiencing a fall compared to adults without CP. Adults with CP experienced 5.83 times more falls than adults without CP. [ABSTRACT FROM AUTHOR]

Aim: To compare language, speech, and voice of children born preterm and at term, and determine relevant predictors of outcome.Method: Three hundred infants (150 males, 150 females; 149 born at <30wks' gestation, 151 term-born) were prospectively recruited at birth from the Royal Women's Hospital. We administered the Preschool Language Scales, Fifth Edition, Diagnostic Evaluation of Articulation and Phonology, Grade Roughness Breathiness Asthenia Strain Scale, and Pediatric Voice Handicap Index at 3 years, and compared groups. We examined hypothesized predictors in children born preterm: gestational age at birth, birthweight, sex, chronic lung disease, high social risk, multilingualism, neurodevelopmental diagnosis, and oromotor feeding.Results: Children born preterm had poorer language than children born at term (coefficient -5.43). Speech and voice were similar between groups (coefficients -0.70 to 1.63). Chronic lung disease predicted voice (coefficient 6.05); male sex (coefficients 4.54-6.18), high social risk (coefficient -6.02 to -9.30), and neurodevelopmental diagnosis (coefficients -16.42 to -20.61) predicted language.Interpretation: Children born before 30 weeks' gestation had poorer language than children born at term. Children born preterm with neurodevelopmental disabilities or high social risk experience poorer language outcomes, and would benefit from enrichment of their language environment.What This Paper Adds: Speech and voice outcomes were similar between children born preterm and at term. Male sex, high social risk, and neurodevelopmental diagnosis predicted language outcomes. [ABSTRACT FROM AUTHOR]

Aim: This study examined children's health care service use, mothers' workforce participation, and mothers' community engagement based on children's risk of developmental delay.Method: We used data from the All Our Families study, a prospective pregnancy cohort. Ages and Stages Questionnaire (ASQ) scores at year 2 indicated risk of developmental delay. To investigate the impact of risk of developmental delay when children were not diagnosed, a sensitivity analysis excluded reports of neurodevelopmental disorder (NDD) diagnosis at year 3. Outcomes were maternal reports of children's health and allied health visits (and estimated costs), and maternal workforce participation and community engagement from year 2 to 3.Results: Among 1314 mother-child dyads, 209 (16%) children were classified as being at risk of developmental delay by the ASQ, and 42 (3%) had a reported diagnosis of NDD. Risk of developmental delay was related to increased use of allied health care services (incidence risk ratio 5.04 [year 3]; 95% confidence interval 2.49-10.2) and health visits (incidence risk ratio 1.33 [year 3]; 95% confidence interval 1.14-1.54). The average expected allied health costs were greater for children at risk versus not at risk of developmental delay. However, when excluding children with reported diagnoses of an NDD from this analysis, increased service use and costs in the remaining at-risk population were not observed. Community engagement and workplace participation among families did not differ on the basis of risk of developmental delay.Interpretation: These results suggest increased health care service use by families of children at risk of developmental delay is driven by those receiving a diagnosis of an NDD in the subsequent year.What This Paper Adds: Early developmental delay risk was related to health care service use and costs. Diagnosis of neurodevelopmental disorder drove increased health care service use and costs. Early developmental delay risk did not relate to parental workforce participation. Early developmental delay risk did not relate to community engagement participation. [ABSTRACT FROM AUTHOR]

Data collected for a genome-wide association study of a primary phenotype are often used for additional genome-wide association analyses of secondary phenotypes. However, when the primary and secondary traits are dependent, naïve analyses of secondary phenotypes may induce spurious associations in non-randomly ascertained samples. Previously, retrospective likelihood-based methods have been proposed to correct for sampling biases arising in secondary trait association analyses. However, most methods have been introduced to handle studies featuring a case-control design based on a binary primary phenotype. As such, these methods are not directly applicable to more complicated study designs such as multiple-trait studies, where the sampling mechanism also depends on the secondary phenotype, or extreme-trait studies, where individuals with extreme primary phenotype values are selected. To accommodate these more complicated sampling mechanisms, only a few prospective likelihood approaches have been proposed. These approaches assume a normal distribution for the secondary phenotype (or the latent secondary phenotype) and a bivariate normal distribution for the primary-secondary phenotype dependence. In this paper, we propose a unified copula-based approach to appropriately detect genetic variant/secondary phenotype association in the presence of selected samples. Primary phenotype is either binary or continuous and the secondary phenotype is continuous although not necessary normal. We use both prospective and retrospective likelihoods to account for the sampling mechanism and use a copula model to allow for potentially different dependence structures between the primary and secondary phenotypes. We demonstrate the effectiveness of our approach through simulation studies and by analyzing data from the Avon Longitudinal Study of Parents and Children cohort. [ABSTRACT FROM AUTHOR]

Aim: To investigate long-term development of hand function after repeated botulinum neurotoxin A (BoNT-A) and occupational therapy at a young age.Method: Twenty children with unilateral spastic cerebral palsy (CP) (14 males, six females; median inclusion age 3y 1mo, range 1y 11mo-4y 3mo) participated in this longitudinal study. Ten children received occupational therapy after a randomized controlled trial and 10 repeated BoNT-A plus occupational therapy during 1-year. The Assisting Hand Assessment (AHA) and active supination, assessed the following 3 years. The assessments were compared with data from a reference group to investigate development over time.Results: The improvement in AHA (7.5 AHA units) after BoNT-A plus occupational therapy was maintained at final follow-up. The occupational therapy group, unchanged after 1-year, improved by 5 AHA units (96% confidence interval [CI] 2-10), thus there was no difference between the groups. Median active supination increased in comparison with the reference group. In the BoNT-A/occupational therapy group, 9 out of 10 (97.85% CI 45 115) children improved in active supination. In the occupational therapy group, 7 out of 10 (97.85% CI -2 to 68) children improved in active supination. No correlation between active supination and AHA was found.Interpretation: Bimanual performance achieved after BoNT-A plus occupational therapy was maintained, while it increased by follow-up in the occupational therapy group, suggesting that combined intervention gave earlier access to bimanual skills. Active supination was unrelated to AHA.What This Paper Adds: Children whose bimanual performance improved after botulinum neurotoxin A and/or occupational therapy, maintained skills or progressed during follow-up. Bimanual performance increased with age, similar to a quality-register reference group. Increased active supination after intervention improved at follow-up but was not related to bimanual performance. [ABSTRACT FROM AUTHOR]

Aim: To characterize fatigue in children with hemiparesis with perinatal stroke and explore associations with measures of motor performance and corticospinal excitability.Method: Forty-five children (16 females, 29 males), aged 6 to 18 years (mean [SD] 12y [4]), with magnetic resonance imaging-confirmed perinatal stroke participated. Associations between fatigue (Pediatric Quality of Life Inventory Version 3.0 cerebral palsy module fatigue subscale), motor performance (Assisting Hand Assessment [AHA], Box and Blocks Test, grip strength), and excitability of corticospinal projections to both hands were examined using ranked tests of correlation, robust regression, and the Mann-Whitney U test.Results: Nearly half of the participants (n=21) reported experiencing fatigue in the previous month. Function in the less affected hand (Box and Blocks Test, grip strength) was correlated with fatigue scores. Participants with preserved ipsilateral projections to the more affected hand had less fatigue, and scores correlated with the excitability of these projections. Fatigue scores were not associated with age, sex, or AHA score.Interpretation: Fatigue is common in children with hemiparesis with perinatal stroke and is associated with motor performance and the presence and excitability of ipsilateral corticospinal projections from the contralesional hemisphere to the more affected hand.What This Paper Adds: Fatigue is common in children with hemiparesis with perinatal stroke. Fatigue was associated with motor performance and strength in the less affected, but not the more affected, hand. Fatigue was associated with the presence and excitability of ipsilateral corticospinal projections from the contralesional hemisphere. [ABSTRACT FROM AUTHOR]

Aim: To determine development curves of communication and social interaction from childhood into adulthood for individuals with cerebral palsy (CP).Method: This Pediatric Rehabilitation Research in the Netherlands (PERRIN)-DECADE study longitudinally assessed 421 individuals with CP, aged from 1 to 20 years at baseline, after 13 years (n=121 at follow-up). Communication and social interactions were assessed using the Vineland Adaptive Behavior Scales. We estimated the average maximum performance limit (level) and age at which 90% of the limit was reached (age90 ) using nonlinear mixed-effects modeling.Results: One-hundred individuals without intellectual disability were aged 21 to 34 years at follow-up (39 females, 61 males) (mean age [SD] 28y 5mo [3y 11mo]). Limits of individuals without intellectual disability, regardless of Gross Motor Function Classification System (GMFCS) level, approached the maximum score and were significantly higher than those of individuals with intellectual disability. Ages90 ranged between 3 and 4 years for receptive communication, 6 and 7 years for expressive communication and interrelationships, 12 and 16 years for written communication, 13 and 16 years for play and leisure, and 14 and 16 years for coping. Twenty-one individuals with intellectual disability were between 21 and 27 years at follow-up (8 females, 13 males) (mean age [SD] 24y 7mo [1y 8mo]). Individuals with intellectual disability in GMFCS level V showed the least favourable development, but variation between individuals with intellectual disability was large.Interpretation: Individuals with CP and without intellectual disability show developmental curves of communication and social interactions similar to typically developing individuals, regardless of their level of motor function. Those with intellectual disability reach lower performance levels and vary largely in individual development.What This Paper Adds: Communication and social interactions in individuals with cerebral palsy without intellectual disability develop similarly to typically developing individuals. Communication and social interactions of individuals with intellectual disability develop less favourably and show large variation. [ABSTRACT FROM AUTHOR]

Aim: This study aims to identify characteristics at 2 years of age that differ between children with confirmed cerebral palsy (CP) and a non-CP diagnosis by 5 years of age.Method: This was a retrospective cohort analysis. A CP diagnosis may be considered a 'probable' diagnosis at 2 years, which is often 'confirmed' at 4 or 5 years, particularly in the context of CP registries. A total of 1683 children with a diagnosis of CP or probable CP at 2 years of age were identified from the Canadian Cerebral Palsy Registry, of whom 48 received a non-CP diagnosis at 5 years ('non-confirmed CP'). Perinatal adversity, preterm birth status, Gross Motor Function Classification System (GMFCS) level, presence of comorbidities, magnetic resonance imaging (MRI) findings, and initial CP motor type were compared between the two groups by univariate and logistic regression analyses.Results: χ2 analysis and multivariate analysis both confirmed that children with a non-CP diagnosis by 5 years of age were more likely to have a normal MRI (χ2 odds ratio [OR]=7.8, 95% confidence interval [CI]=3.8-16.1; OR=5.4, 95% CI=2.4-12.5), ataxic-hypotonic (χ2 OR=10.1, 95% CI=4.9-21.2; OR=6.1, 95% CI=2.2-16.2) or dyskinetic CP (χ2 OR=2.7, 95% CI=1.2-5.9; OR=2.9, 95% CI=1.0-7.6), born at term (χ2 OR=3.7, 95% CI=1.7-8.0; OR=3.6, 95% CI=1.0-12.1), and lack perinatal adversity (χ2 OR=4.1, 95% CI=1.6-10.7; OR=3.4, 95% CI=1.0-11.7).Interpretation: Normal MRI, ataxic-hypotonic or dyskinetic CP, lack of perinatal adversity, and term birth are associated with a higher odds of non-CP diagnosis by 5 years of age, thus potentially enhancing diagnostic work-up.What This Paper Adds: Normal magnetic resonance imaging (MRI) at 2 years was associated with a non-cerebral palsy (CP) diagnosis by 5 years. Diagnosis of ataxic-hypotonic or dyskinetic CP motor subtype at 2 years was associated with a non-CP diagnosis by 5 years. Perinatal adversity and preterm birth were rarer with a non-CP diagnosis by 5 years. [ABSTRACT FROM AUTHOR]

Aim: This paper aimed to analyse the association between small for size at birth, stunting, recovery from stunting and pubertal development in a rural Bangladeshi cohort.Methods: The participants were 994 girls and 987 boys whose mothers participated in the Maternal and Infant Nutrition Interventions in Matlab trial. The birth cohort was followed from birth to puberty 2001-2017. Pubertal development according to Tanner was self-assessed. Age at menarche was determined and in boys, consecutive height measurements were used to ascertain whether pubertal growth spurt had started. The exposures and outcomes were modelled by Cox's proportional hazards analyses and logistic regression.Results: There was no difference in age at menarche between girls that were small or appropriate for gestational age at birth. Boys born small for gestational age entered their pubertal growth spurt later than those with appropriate weight. Children who were stunted had later pubertal development, age at menarche and onset of growth spurt than non-stunted children. Children who recovered from infant or early childhood stunting had similar pubertal development as non-stunted children.Conclusion: Infant and childhood stunting was associated with a later pubertal development. Recovery from stunting was not associated with earlier puberty in comparison with non-stunted children. [ABSTRACT FROM AUTHOR]

In many applications of linear mixed-effects models to longitudinal and multilevel data especially from medical studies, it is of interest to test for the need of random effects in the model. It is known that classical tests such as the likelihood ratio, Wald, and score tests are not suitable for testing random effects because they suffer from testing on the boundary of the parameter space. Instead, permutation and bootstrap tests as well as Bayesian tests, which do not rely on the asymptotic distributions, avoid issues with the boundary of the parameter space. In this paper, we first develop a permutation test based on the likelihood ratio test statistic, which can be easily used for testing multiple random effects and any subset of them in linear mixed-effects models. The proposed permutation test would be an extension to two existing permutation tests. We then aim to compare permutation tests and Bayesian tests for random effects to find out which test is more powerful under which situation. Nothing is known about this in the literature, although this is an important practical problem due to the usefulness of both methods in tackling the challenges with testing random effects. For this, we consider a Bayesian test developed using Bayes factors, where we also propose a new alternative computation for this Bayesian test to avoid some computational issue it encounters in testing multiple random effects. Extensive simulations and a real data analysis are used for evaluation of the proposed permutation test and its comparison with the Bayesian test. We find that both tests perform well, albeit the permutation test with the likelihood ratio statistic tends to provide a relatively higher power when testing multiple random effects. [ABSTRACT FROM AUTHOR]

Standard methods for two-sample tests such as the t-test and Wilcoxon rank sum test may lead to incorrect type I errors when applied to longitudinal or clustered data. Recent alternatives of two-sample tests for clustered data often require certain assumptions on the correlation structure and/or noninformative cluster size. In this paper, based on a novel pseudolikelihood for correlated data, we propose a score test without knowledge of the correlation structure or assuming data missingness at random. The proposed score test can capture differences in the mean and variance between two groups simultaneously. We use projection theory to derive the limiting distribution of the test statistic, in which the covariance matrix can be empirically estimated. We conduct simulation studies to evaluate the proposed test and compare it with existing methods. To illustrate the usefulness proposed test, we use it to compare self-reported weight loss data in a friends' referral group, with the data from the Internet self-joining group. [ABSTRACT FROM AUTHOR]

Aim: To determine the mortality rate, immediate cause of death (CoD), and predictors of death in children with cerebral palsy (CP) in rural Bangladesh.Method: We carried out a prospective population-based surveillance study of children with CP aged 0 to 18 years registered with the Bangladesh Cerebral Palsy Register (BCPR) between January 2015 and December 2016, with subsequent follow-up until December 2017. Verbal autopsy was applied to assign immediate CoD. Crude mortality rates, hazard ratios of death, and survival probabilities were estimated.Results: Twenty-nine of the 678 children in the BCPR died during the study period, resulting in a crude mortality rate of 19.5 per 1000 person-years of observation (total follow-up duration 1486.8 person-years; mean 2y [standard deviation 6mo]). The leading immediate CoD was meningitis (n=9) and pneumonia (n=8). Survival probability and hazard ratio of death was significantly associated with age, Gross Motor Functional Classification System level, and associated impairments. Severe underweight and/or severe stunting was significantly overrepresented among deceased children than others in the cohort (p<0.05) when compared with the World Health Organization reference population.Interpretation: The majority of deaths were due to potentially preventable causes. The life expectancy of these children could have been extended by ensuring primary healthcare and nutritional supplementation.What This Paper Adds: Mortality rate in children with cerebral palsy (CP) in rural Bangladesh is 19.5 per 1000 person-years. The majority of children with CP died from potentially preventable and treatable conditions. Motor severity, associated impairments, and malnutrition make children with CP vulnerable to premature death in rural Bangladesh. [ABSTRACT FROM AUTHOR]

Aim: To describe the baseline developmental profile and influence of clinical and demographic factors on the developmental skills of infants diagnosed with infantile spasms.Method: Ninety-five infants (55 males, 40 females) newly diagnosed with infantile spasms were recruited for a cross-sectional, longitudinal study. All infants underwent Bayley Scales of Infant and Toddler Development assessments in the cognitive, receptive communication, expressive communication, and fine and gross motor developmental domains; they also underwent visual, auditory, and social behaviour assessments. Infants were categorized as 'early' (<6mo) or 'late' (≥6mo) presenters; if presented within 28 days, this was considered as 'early presentation', whereas a delay greater than 28 days was considered as a 'delay in presentation'. Antenatal, perinatal, and postnatal risk factors were identified.Results: Over 90% of infants showed impairment in all domains, with the majority having severe delay; 99% showed cognitive impairment. Delayed presentation was significantly associated with receptive communication delay (odds ratio [OR]=5.35; 95% confidence interval [CI]=1.05-27.32). Onset at 6 months or less influenced auditory (OR=2.8; 95% CI=1.16-6.8) and visual (OR=3.03; 95% CI=1.22-7.57) behaviours. Neonatal infections impacted both receptive (OR=1.12; 95% CI=1.04-1.2) and expressive communication (OR=1.08; 95% CI=1.02-1.14) delay. Neonatal seizures significantly influenced visual, auditory, and social impairments. Expressive communication and gross motor development shared common perinatal risk factors.Interpretation: Adverse developmental status at presentation, associated with delayed presentation and neonatal risk factors should alert clinicians to the surveillance of at-risk infants and seek out timely interventions.What This Paper Adds: Ninety per cent of infants showed impaired cognitive, communication, and motor skills at presentation. Visual, auditory, and social behaviour impairments were significantly associated with perinatal risks. Visual, auditory, and social behaviour impairments were significantly associated with neonatal seizures. [ABSTRACT FROM AUTHOR]

Aim: To describe the prevalence of cerebral palsy (CP), subtype distribution, motor and intellectual impairment, and epilepsy in adults with CP compared with children with CP.Method: CP subtype and impairment data from the population-based CP register of western Sweden and population data from Statistics Sweden were used to compare surviving adults (n=581; 244 females, 337 males) born between 1959 and 1978, with the same cohort as children (n=723; 307 females, 416 males), and with the most recent cohort, born from 2007 to 2010 (n=205; 84 females, 121 males).Results: Prevalence of CP in adults born between 1959 and 1978 was 1.14 per 1000. The occurrence of impairments differed between CP subtypes. Motor and intellectual impairment were closely related, regardless of subtype. Subtype distribution among survivors differed significantly from the original cohorts (p=0.002), and the most recent cohort (p<0.01), tetraplegia and dyskinetic CP being less common in survivors. Severe motor impairment, intellectual disability, and epilepsy were less common among survivors than in the original cohorts (p=0.004, p=0.002, p=0.037) and the most recent cohort (p=0.004, p=0.008, p<0.01).Interpretation: Data on prevalence, subtype distribution, and impairments in children with CP are not applicable to adults with CP. Population-based studies of adults with CP are needed.What This Paper Adds: Cerebral palsy (CP) subtypes are differently distributed in adults compared to children. The prevalence of impairments in adults with CP is related to CP subtype. Spastic tetraplegia and dyskinetic CP are less common in adults than children. Severe motor impairment, intellectual disability, and epilepsy are less common in adults. [ABSTRACT FROM AUTHOR]

Aim: To test the hypothesis that improvements in gait and function following individualized interdisciplinary interventions consisting of physical therapy, orthotics, spasticity management, and orthopaedic surgery using instrumented gait analysis are superior to 'usual care' in children with cerebral palsy (CP).Method: This was a prospective, single-blind, parallel-group, randomized controlled trial investigating the effectiveness of interventions based on the use of gait analysis. Primary outcome was gait (Gait Deviation Index) and secondary outcomes were walking and patient-reported outcome measures of function, disability, and health-related quality of life. Follow-ups were done at 26 weeks (questionnaires) and at the primary end point of 52 weeks (all outcomes).Results: Sixty participants with CP (39 males, 21 females, mean age 6y 10mo, standard deviation 1y 3mo, range 5y-9y 1mo) in Gross Motor Function Classification System levels I or II, were randomized to interventions with or without gait analysis. No significant or clinically relevant between-group differences in change scores of the primary or secondary outcomes were found. The recommended categories of interventions were dominated by non-surgical interventions and were applied in 36% to 86% of the participants.Interpretation: Interventions using gait analysis were not superior to 'usual care' on gait, walking, or patient-reported outcomes in a sample of relatively young and independently walking children with CP not expected to need surgery.What This Paper Adds: Gait analysis in children with cerebral palsy in Gross Motor Function Classification System levels I or II recommends interdisciplinary interventions. Compliance to interventions recommended after gait analysis was low. No statistically significant advantages were identified for the intervention group versus the control group. [ABSTRACT FROM AUTHOR]

Aim: To conduct a longitudinal follow-up of the development of global cognitive abilities and adaptive skills in individuals with congenital and childhood forms of myotonic dystrophy type 1 (DM1).Method: Fifty-one participants (29 males, 22 females, mean age 19y 5mo, SD 4y 11mo, range 10y 10mo-28y 11mo) were divided into severe congenital (n=16), mild congenital (n=17), and childhood DM1 (n=18) subgroups. The average time between the first and second assessments was 7 years 8 months. Adaptive skills were evaluated using the Vineland Adaptive Behavior Scales and global cognitive functioning using Wechsler scales.Results: There was no statistically significant decline in cognitive abilities and adaptive behaviour. A tendency of decline regarding the level of intellectual disability was found in the congenital DM1 groups but not in the childhood group. In the congenital DM1 groups, the gap in relation to typically developing peers in cognitive and adaptive functioning increased. Predictors of change over time in adaptive skills were age and current level of intellectual disability: individuals with severe intellectual disability and younger individuals deteriorated the most. However, when raw scores were compared, no actual regression in adaptive functioning was found.Interpretation: The participants had not lost any important adaptive skills. Greater cognitive and adaptive development was found in the childhood group than in the congenital groups.What This Paper Adds: There is no absolute decline in cognitive and adaptive abilities in individuals with congenital and childhood myotonic dystrophy type 1. Pace of development is slow in comparison with normative data. The childhood group tended to show greater cognitive and adaptive development than the congenital groups. [ABSTRACT FROM AUTHOR]

The many adverse effects of child maltreatment make the scientific investigation of this phenomenon a matter of vital importance. Although the relationship between maltreatment and problematic emotion reactivity and regulation has been studied, the strength and specificity of these associations are not yet clear. We examine the magnitude of the maltreatment-child-emotion reactivity/regulation link. Studies with substantiated maltreatment involving children aged up to 18 were included, along with a smaller number of longitudinal studies (58 papers reviewed, encompassing more than 11,900 children). In comparison to nonmaltreated children, maltreated children experience more negative emotions, behave in a manner indicative of more negative emotion, and display emotion dysregulation. We outline several theoretical implications of our results. [ABSTRACT FROM AUTHOR]

Aim: To assess the predictive validity of the Functional Evaluation of Eating Difficulties Scale (FEEDS) on long-term eating developmental outcomes in infants with neurodevelopmental disorders.Method: In total, 144 infants (69 females, 75 males) aged 0 to 12 months (mean [SD] 5.34mo [3.42]) with neurodevelopmental disorders and requiring enteral nutrition support, hospitalized between January 2004 and December 2017, were included. The FEEDS was administered at the onset of hospitalization. Follow-up evaluations of feeding modalities occurred at discharge and at 6 months, 12 months, and 24 months after discharge. FEEDS score was tested as a predictor of infants' feeding modality (percutaneous endoscopic gastrostomy, nasogastric tube, mixed, oral feeding) and time to autonomous oral feeding. Percentages of false-positive and negative cases were checked.Results: Lower FEEDS scores significantly predicted infants' feeding modality (0.40≤R2 ≤0.61). A 1-point increase in FEEDS score was associated with increased risk (6%-14%; p<0.05) of being non-autonomous feeders at the different follow-up points in infants who had a FEEDS score above the clinical cut-off.Interpretation: The FEEDS appears to be a clinically valid assessment to predict the presence of eating difficulties in infants with neurodevelopmental disabilities.What This Paper Adds: Functional Evaluation of Eating Difficulties Scale (FEEDS) significantly predicted eating difficulties in infants with neurodevelopmental disabilities. Lower FEEDS score is significantly associated with autonomous feeding at the 24-month follow-up. FEEDS cut-off identified infants at low-risk and high-risk for eating disorder. [ABSTRACT FROM AUTHOR]

Aim: To examine the stability over time of the Manual Ability Classification System (MACS) levels in children with cerebral palsy (CP) aged 18 to 60 months.Method: This was a prospective longitudinal population-based study of 252 Australian children (160 males [63%] 92 females [37%]; mean age [SD] 41.7mo [14], range 17.2mo-69.2mo) with CP. Children were classified at 18 months (n=70), 24 months (n=131), 30 months (n=173), 36 months (n=209), 48 months (n=226), and 60 months (n=221) of age. Stability of the MACS was examined using the proportion of specific positive agreement and transition proportions, which are measures of agreement.Results: There were 1030 unique observations, with each of the 252 participants seen between two and six occasions (median=4). Average specific positive agreement over the study period was 76% for MACS level I, 67% for level II, 50% for level III, 51% for level IV, and 83% for level V. MACS levels I and V have the highest degree of stability, while levels III and IV have the lowest. We show how this may be explained by the proportion of children in each MACS level.Interpretation: Using measures of agreement rather than measures of reliability provides accurate information when measuring stability over time of an ordinal classification system. The relative stability of MACS levels can be explained by the proportion of children in each level.What This Paper Adds: Children classified in Manual Ability Classification System (MACS) levels III and IV change levels at next assessment about 50% of the time. Children should be assessed with the MACS regularly, particularly those in levels III and IV. Stability within ordinal classification level categories can be predicted using a measurement error model. Transition proportions or specific agreement is recommended for reporting stability of ordinal classification systems. [ABSTRACT FROM AUTHOR]

Aim: This cross-sectional investigation evaluated whether recurrent botulinum neurotoxin A (BoNT-A) interventions to the medial gastrocnemius have an influence on muscle morphology, beyond Gross Motor Function Classification System (GMFCS) level.Method: A cohort of typically developing children (n=67; 43 males, 24 females; median age 9y 11mo [range 7y 10mo-11y 6mo]), a cohort of children with spastic cerebral palsy (CP) naive to BoNT-A interventions (No-BoNT-A; n=19; 10 males, nine females; median age 9y 3mo [range 8y 5mo-10y 10mo]) and a cohort of children with spastic CP with a minimum of three recurrent BoNT-A interventions to the medial gastrocnemius (BoNT-A; n=19; 13 males, six females; median age 9y 8mo [range 7y 3mo-10y 7mo]) were recruited. Three-dimensional freehand ultrasound was used to estimate medial gastrocnemius volume normalized to body mass and echo-intensity.Results: Normalized medial gastrocnemius volume and echo-intensity significantly differed between the two spastic CP cohorts (p≤0.05), with the BoNT-A cohort having larger alterations. Associations between normalized medial gastrocnemius volume and echo-intensity were highest in the No-BoNT-A cohort, followed by the BoNT-A cohort. Multiple regression analyses revealed that both GMFCS level and BoNT-A intervention history were significantly associated with smaller normalized medial gastrocnemius volume and higher echo-intensity.Interpretation: Recurrent BoNT-A interventions may induce alterations to medial gastrocnemius volume and echo-intensity beyond the natural history of the spastic CP pathology.What This Paper Adds: In spastic cerebral palsy, medial gastrocnemius volumes are smaller and echo-intensities higher compared with typical development. Alterations after botulinum neurotoxin A intervention (BoNT-A) are larger than in no BoNT-A intervention. Gross Motor Function Classification System level and BoNT-A history significantly associate with medial gastrocnemius and echo-intensity alterations. [ABSTRACT FROM AUTHOR]

Aim: To examine: (1) relationships between brain structure, and concurrently assessed neurological and behavioural functioning, in infants born preterm at term-equivalent age (TEA; approximately 38-44wks); and (2) whether brain structure-function relationships differ between infants born very (24-29wks) and moderate-late (32-36wks) preterm.Method: A total of 257 infants (91 very preterm, 166 moderate-late preterm; 120 males, 137 females) had structural magnetic resonance imaging (MRI) and neurological and behavioural assessments (Prechtl's general movements assessment, Neonatal Intensive Care Unit Network Neurobehavioral Scale [NNNS] and Hammersmith Neonatal Neurological Examination [HNNE]). Two hundred and sixty-three infants (90 very preterm, 173 moderate-late preterm; 131 males, 132 females) had diffusion MRI and assessments. Associations were investigated between assessment scores and global brain volumes using linear regressions, regional brain volumes using Voxel-Based Morphometry, and white matter microstructure using Tract-Based Spatial Statistics.Results: Suboptimal scores on some assessments were associated with lower fractional anisotropy and/or higher axial, radial, and mean diffusivities in some tracts: NNNS attention and reflexes, and HNNE total score and tone, were associated with the corpus callosum and optic radiation; NNNS quality of movement with the corona radiata; HNNE abnormal signs with several major tracts. Brain structure-function associations generally did not differ between the very and moderate-late preterm groups.Interpretation: White matter microstructural alterations may be associated with suboptimal neurological and behavioural performance in some domains at TEA in infants born preterm. Brain structure-function relationships are similar for infants born very preterm and moderate-late preterm.What This Paper Adds: Brain volume is not related to neurological/behavioural function in infants born preterm at term. White matter microstructure is related to some neurological/behavioural domains at term. Brain-behaviour relationships are generally similar for infants born very preterm and moderate-late preterm. [ABSTRACT FROM AUTHOR]

Background: Longitudinal cohort studies gather large amounts of data over time, often without direct benefit to participants. A positive experience may encourage retention in the study, and participants may benefit in unanticipated ways. Objective: To explore first‐time mothers' experiences of taking part in a longitudinal cohort study and completing self‐administered surveys during pregnancy and at 3, 6, 9 and 12 months' postpartum. Design: Content analysis of comments written by participants in the Maternal health And Maternal Morbidity in Ireland study's five self‐completion surveys, a multisite cohort study exploring women's health and health problems during and after pregnancy. This paper focuses on what women wrote about taking part in the research. Ethical approval was granted by the site hospitals and university. Setting and participants: A total of 2174 women were recruited from two maternity hospitals in Ireland between 2012 and 2015. Findings: A total of 1000 comments were made in the five surveys. Antenatally, barriers related to surveys being long and questions being intimate. Postpartum, barriers related to being busy with life as first‐time mothers. Benefits gained included gaining access to information, taking time to reflect, stopping to think and being prompted to seek help. Survey questions alone were described as valuable sources of information. Discussion and conclusions: Findings suggest that survey research can "give back" to women by being a source of information and a trigger to seek professional help, even while asking sensitive questions. Understanding this can help researchers construct surveys to maximize benefits, real and potential, for participants. [ABSTRACT FROM AUTHOR]

Aim: To examine the longitudinal trends of cardiometabolic diseases in a large sample of adults with cerebral palsy (CP).Method: The Optum Clinformatics Data Mart is a de-identified nationwide claims database of beneficiaries from a single private payer. Beneficiaries were included if they had an International Classification of Diseases, Ninth Revision, Clinical Modification code for a diagnosis of CP. Adults with at least 3 years of continuous enrollment on a single plan between 2002 and 2009 were included in the final analyses (n=2659). We examined the longitudinal trends of incident diabetes mellitus, hypercholesterolemia, hypertension, cardiac dysrhythmias, and atherosclerosis, stratified by age categories: 18 to 39 years, 40 to 59 years, and 60 years and over. Kaplan-Meier product-limit survival curves were compared across age categories for each of the cardiometabolic outcomes, and a Cox proportional hazards regression was run to determine adjusted hazard ratios.Results: The cumulative incidence of each of the cardiometabolic diseases ranged from 6.0% for atherosclerosis to 34.4% for hypercholesterolemia at 3 years and over. Risk-adjusted Cox proportional hazard models revealed that age was a robust predictor of survival for each outcome, with higher hazard ratio ranges in middle age (hazard ratio 1.41-2.72) and older adults (hazard ratio 2.20-5.93) compared with young adults.Interpretation: Adults with CP have high rates of cardiometabolic diseases; and disease-free survival shortens significantly with higher ages.What This Paper Adds: Adults with cerebral palsy have high rates of cardiometabolic diseases. Disease-free survival of all cardiometabolic diseases shortens significantly with higher ages. The highest rates were for hypercholesterolemia and hypertension. [ABSTRACT FROM AUTHOR]

Aim: Neurodevelopmental outcomes in children with congenital cerebellar malformations (CCMs) remain poorly defined. We aimed to assess whether specific neuroimaging features in CCM patients correlate with neurodevelopmental outcomes.Method: Hospital records and neuroimaging of 67 children with CCMs were systematically reviewed. Logistic regression analyses were used to assess associations between specific imaging features and neurodevelopmental outcomes.Results: CCM categories were distributed as follows: 28 percent isolated vermis hypoplasia (n=19), 28 percent global cerebellar hypoplasia (n=19), 15 percent Dandy-Walker malformation (n=10), 13 percent pontocerebellar hypoplasia (PCH, n=9), 9 percent molar tooth malformation (n=6), 3 percent rhombencephalosynapsis (n=2), and 3 percent unilateral cerebellar malformation (n=2). Overall, 85 percent (55/65) of the cohort had global developmental delay (GDD). Intellectual disability was present in 61 percent (27/43) and autism spectrum disorder (ASD) in 12 percent (6/52). Adjusting for supratentorial malformations and presence of genetic findings, severe GDD was associated with cerebellar hypoplasia (p=0.049) and PCH (p=0.030), whereas children with vermis hypoplasia were less likely to have severe GDD (p=0.003). Presence of supratentorial abnormalities was not significantly associated with worse neurodevelopmental outcome but was associated with epilepsy.Interpretation: Children with CCMs have high prevalence of neurodevelopmental deficits. Specific features on imaging can aid prognostication and establish early intervention strategies.What This Paper Adds: Atypical long-term neurodevelopmental outcome is very common in patients with congenital cerebellar malformations (CCMs). Involvement of the brainstem and cerebellar hemispheres predicts more severe neurodevelopmental disability. Most patients with vermis hypoplasia have language delay but are verbal. Supratentorial abnormalities are not significantly associated with worse neurodevelopmental outcome but are associated with epilepsy. Comorbidities are common in CCMs, especially ophthalmological issues in cerebellar hypoplasia and sensorineural hearing loss in pontocerebellar hypoplasia. [ABSTRACT FROM AUTHOR]

Aim: To investigate trends in birth prevalence of cerebral palsy (CP) overall and by gestational age, and examine the distribution of motor type, spastic topography, and severity using Australian CP Register data from 1995 to 2009.Method: Prenatal and perinatal CP data were collated from state/territory CP registers. Birth prevalence estimates per 1000 live births and per 1000 neonatal survivors (NNS) were calculated in five epochs. Data from three state registers with population-level ascertainment were used to investigate birth prevalence trends by gestational age using Poisson regression. Distribution of motor type, spastic topography, and moderate to severe disability (IQ≤50 and/or Gross Motor Function Classification System levels III-V) were evaluated within birthweight categories.Results: Birth prevalence of CP varied across population-level states but within each state declined significantly over time (p<0.05). Birth prevalence per 1000 neonatal survivors declined amongst children born before 28 weeks (South Australia, Victoria p<0.001) and those born at or after 37 weeks (Victoria p<0.001, Western Australia p<0.002). Across Australia the percentage of children with bilateral spastic CP declined amongst those born less than 1000g. The percentage of children with moderate to severe disability decreased (48%-34%, p<0.001).Interpretation: Birth prevalence of CP declined. Encouragingly, the percentage of children with CP whose disability was moderate to severe also decreased.What This Paper Adds: Birth prevalence of cerebral palsy (CP) differed but declined across Australian states (1995-2009). Australian CP birth prevalence declined significantly amongst children born before 28 weeks and those born at or after 37 weeks. The percentage of children with moderate to severe disability decreased. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to investigate the longitudinal development of passive range of motion (ROM) in the upper limbs in a population-based sample of children with cerebral palsy (CP), and to investigate which children are more likely to develop contractures related to functional level, CP subtype, and age.Method: Registry data of annual passive ROM measurements of the upper limbs from 771 children with CP (417 males, 354 females; mean age 11y 8mo, [SD 5mo] range 1-18y) were analysed. Mixed models were used to investigate at what age decreased passive ROM occurs. Odds ratios were calculated to compare risks and logistic regression analysis was used to predict contracture development.Results: Thirty-four per cent of the children had developed contractures. Among these children, decreased passive ROM was significant at a mean age of 4 years for wrist extension and 7 years for shoulder flexion, elbow extension, and supination. Children at Manual Ability Classification System (MACS) level V had a 17-times greater risk of contractures than children at MACS level I.Interpretation: One-third of the children in the total population developed upper-limb contractures while passive ROM decreased with age. MACS level was the strongest predictor of contracture development.What This Paper Adds: In a population-based sample of 771 children with cerebral palsy, 34% developed an upper-limb contracture. Contracture development started at preschool age. The first affected movements were wrist extension and supination. Passive range of motion decreased with age. High Manual Ability Classification System level was the most important predictor of contractures. [ABSTRACT FROM AUTHOR]

Aim: To determine the long-term course of difficulty in participation of individuals with cerebral palsy (CP) without intellectual disability between 16 years and 34 years of age.Method: One hundred and fifty-one individuals with CP aged 16 to 20 years were included (63% male, 37% female; Gross Motor Function Classification System [GMFCS] levels I-IV; without intellectual disability). The Assessment of Life Habits questionnaire 3.0 general short form was used up to three times biennially and at 13-year follow-up (13-year follow-up: n=98). Scores (range 0-10) reflect difficulty and assistance in participation in housing, education and employment, interpersonal relationships, recreation, community life, and responsibilities. Multilevel models were used to determine the course of difficulty in participation by GMFCS level.Results: Despite high average participation levels, 41% to 95% of adolescents and young adults with CP experienced difficulty. Difficulty in participation in housing and interpersonal relationships increased from age 16 years onwards and in most other life areas in the mid- and late 20s. In adolescents in GMFCS levels III and IV, participation in recreation and community life improved up to age 23 years.Interpretation: Individuals with CP experience increasing difficulties in participation in their mid- and late 20s. Clinicians should systematically check for participation difficulties in young adults with CP and offer timely personalized treatment.What This Paper Adds: Many individuals with cerebral palsy (CP) aged 16 to 34 years experience difficulty in participation. Difficulty in participation increases in the mid- and late 20s for individuals with CP. Participation in recreation/community life improves before age 23 years for those in Gross Motor Function Classification System levels III and IV. [ABSTRACT FROM AUTHOR]

Aim: To compare the efficacy of the main methodologies in attaining sleep and electroencephalography (EEG) abnormalities in children with a view to producing recommendations on best practice.Method: Fifty-one UK centres participated. Methods for sleep induction (sleep deprivation, melatonin, and combined sleep deprivation/melatonin) were compared. Data pertaining to demographics, achievement of stage II sleep, and recording characteristics (duration of study, presence of epileptiform activity in awake/sleep states) were prospectively collected for consecutive patients in November and December 2013.Results: Five hundred and sixty-five patients were included. Age range was 1 years to 17 years (mean 7y 10mo), 27.7 per cent had an underlying neurobehavioural condition. Stage II sleep was achieved in 69 per cent of sleep deprived studies, 77 per cent of melatonin studies, and 90 per cent of combined intervention studies (p<0.001, χ2 ). In children who slept, there was no difference between the three interventions in eliciting epileptiform discharges. In children who did not sleep, epileptiform abnormalities were seen more often than after sleep deprivation alone (p=0.02, χ2 ). Seizures were rare.Interpretation: Combined sleep deprivation/melatonin is more effective than either method alone in achieving sleep. The occurrence of epileptiform activity during sleep is broadly similar across the three groups. We recommend the combined intervention to induce sleep for paediatric EEG.What This Paper Adds: Combined sleep deprivation/melatonin is more effective in achieving sleep than either sleep deprivation or melatonin alone. Sleep latency is shorter with combined sleep deprivation/melatonin. When children do sleep, there is no difference in the occurrence of epileptiform abnormalities between different induction methods. Seizures are rare in sleep electroencephalography recordings. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to track alterations in muscle volume for 6 months in children with cerebral palsy (CP) after the first exposure to botulinum neurotoxin A (BoNT-A), a commonly used focal spasticity treatment.Method: Eleven ambulant children (eight males, three females) with spastic CP, mean age 8 years 10 months (SD 3y 1mo) participated. Participants received injections to the affected gastrocnemius. The muscle volume of the gastrocnemius, soleus, tibialis anterior, and hamstrings was measured using magnetic resonance imaging. Muscle volume was normalized to bone length, and changes analysed relative to baseline. Assessments were conducted 1 week before, and 4 weeks, 13 weeks, and 25 weeks after BoNT-A treatment.Results: All children demonstrated positive clinical and functional gains. Muscle volume of the injected gastrocnemius was found to be significantly reduced at 4 weeks (-5.9%), 13 weeks (-9.4%), and 25 weeks (-6.8%). Significant increases in normalized soleus muscle volume were identified at each follow-up, while hamstrings showed significant increase at 4 weeks only.Interpretation: Absolute and normalized muscle volume of the injected muscle reduces after first BoNT-A exposure, and does not return to baseline volume by 25 weeks. Hypertrophy is seen in the soleus up to 25 weeks; the volume of the plantar flexor compartment is stable.What This Paper Adds: Muscle atrophy after first botulinum neurotoxin A (BoNT-A) exposure in children with cerebral palsy is noted. Mild BoNT-A-induced muscle atrophy is still apparent 6 months after BoNT-A exposure. Hypertrophy is evident in soleus after gastrocnemius BoNT-A exposure. Total plantarflexor volume is unchanged. [ABSTRACT FROM AUTHOR]

Aim: To investigate the responsiveness of the ACTIVLIM-CP questionnaire after two evidence-based interventions for children with cerebral palsy (CP).Method: Seventy-five children with CP either participated in an intensive motor-skill learning intervention (hand-arm bimanual intensive therapy including lower extremities [HABIT-ILE], n=47) or received botulinum neurotoxin-A (BoNT-A) injection(s) into lower extremities combined with conventional physical therapy (n=28). All children were assessed three times: at baseline (T0 ; before HABIT-ILE/the day of BoNT-A injection), at T1 (last day of HABIT-ILE/6wks after BoNT-A injection), and at follow-up (T2 ; 3-4mo after the beginning of intervention). Parents completed ACTIVLIM-CP and three other activity questionnaires. Responsiveness was analysed using group (based on intervention), subgroup (based on gross motor function level), and individual approaches.Results: For the HABIT-ILE group, significant improvements in ACTIVLIM-CP were observed for the T0 -T1 period (p<0.001) but not for the T1 -T2 period. No significant changes were found in the BoNT-A group during assessments (p=0.84). In the subgroup analysis for the HABIT-ILE group (T0 -T1 ), greater changes were demonstrated for children in Gross Motor Function Classification System levels III and IV (p<0.001, effect size=1.36). The individual approach was congruent with the group approach.Interpretation: ACTIVLIM-CP demonstrated high responsiveness after HABIT-ILE, showing that this scale may be used to investigate global activity performance in clinical trials focusing on improving daily life activities.What This Paper Adds: Good responsiveness of ACTIVLIM-CP questionnaire during intensive motor-skill learning intervention. Higher responsiveness for children in Gross Motor Function Classification System (GMFCS) levels III and IV versus I and II after intensive intervention. ACTIVLIM-CP is useful to identify children improving their performance after botulinum neurotoxin-A injection. [ABSTRACT FROM AUTHOR]

Aim: Evidence is accumulating that motor and cognitive development are interrelated. This study investigates associations between motor development in infancy and cognitive function at 4 years of age.Method: This study is part of the Groningen prospective cohort study on the development of children born after assisted reproductive techniques such as in vitro fertilization. The study group consisted of 223 children (119 males, 104 females) born to subfertile couples (median gestational age 39.6wks, range 30-43wks). Motor behaviour was assessed with the Infant Motor Profile (IMP) at 4 months, 10 months, and 18 months. IQ was evaluated at 4 years with the Kaufman Assessment Battery for Children, Second Edition. Latent class growth modelling was used to analyse relations between IMP and IQ scores.Results: Infants with low total IMP scores had an IQ that was 8.9 points lower at 4 years than infants with typical IMP scores (95% confidence interval 3.6-14.1). Also, low scores in the domains of variation and performance were associated with a lower IQ at 4 years of age, by 6.1 points and 13.2 points respectively.Interpretation: Motor development in relatively low-risk infants is associated with cognition at 4 years of age. In particular, low motor variation and performance are associated with a lower IQ at 4 years of age.What This Paper Adds: Motor development in infancy is directly related to cognitive function at 4 years of age. This relationship is most pronounced for infant motor variation and performance. The size of the differences in IQ is clinically relevant. [ABSTRACT FROM AUTHOR]

Aim: The International Paediatric Multiple Sclerosis Study Group (IPMSSG) has proposed criteria for acute disseminated encephalomyelitis (ADEM) not evaluated in clinical practice. Our objective was to assess epidemiological implications of the IPMSSG criteria for ADEM in a cohort study using prospectively collected data.Method: We identified all diagnosed cases of ADEM in Denmark between 2008 and 2015 from the Danish National Patient Register by International Classification of Diseases 10 codes assigned to acute demyelinating episodes, and we reviewed all medical records to validate ADEM.Results: We found 52 children up to the age of 18 years with a verified clinical diagnosis of ADEM (incidence rate 0.54/100 000 person-years; all had abnormal brain magnetic resonance imaging). Only 18 (35%) fulfilled the IPMSSG criteria regarding encephalopathy and polyfocal neurological deficits. Among all 52 children with ADEM, 33 per cent had clinical sequelae after a median follow-up of 4 years 6 months (range: 10mo-8y 3mo). Surprisingly, none progressed to multiphasic ADEM or multiple sclerosis, but median age at end of follow-up was only 10 years 9 months (range: 2y-24y 3mo).Interpretation: Among 52 children with ADEM, none converted to multiphasic ADEM or multiple sclerosis (median follow-up: 4y 6mo; range: 10mo-8y 3mo). Applying the IPMSSG criteria to all children with a diagnosis of ADEM leaves 65 per cent of the cases without a diagnosis and lowers the incidence rate of paediatric ADEM.What This Paper Adds: The incidence of paediatric acute disseminated encephalomyelitis (ADEM) was 0.54 per 100 000 person-years in children younger than 18 years. Only 35 per cent of children with ADEM fulfilled the International Paediatric Study Group consensus criteria. ADEM in clinical practice was primarily based on magnetic resonance imaging findings. Paediatric neurologists diagnosed ADEM in the absence of encephalopathy. None of the children with ADEM progressed to multiple sclerosis/multiphasic ADEM during follow-up. [ABSTRACT FROM AUTHOR]