Causation in cerebral palsy: Parental beliefs and associated emotions.

AIM: To better understand parents' beliefs about causation in cerebral palsy (CP) and the emotions related to those beliefs. METHOD: We surveyed 226 parents of children with CP aged 1 to 18 years, recruited from the Victorian Cerebral Palsy Register, to evaluate their beliefs about the causes of CP, including genetic causes, causes specific to their own child, and their attitudes and emotions in relation to these. RESULTS: Although 92% of participants reported that understanding the causes of their child's CP was important, uncertainty about the cause was expressed by 13%. The most frequently endorsed causal factors, in general and in their own child respectively, were intrapartum hypoxia (81%, 36%) or brain damage (69%, 22%), brain damage during pregnancy (73%, 28%), and preterm birth (66%, 28%). Genetic causes were deemed relevant by 13% of participants and hospital or professional error by 16%. Parents shared related feelings of anger (59%), sadness (80%), guilt (61%), and confusion (53%); parental anger was more likely when their child's CP was attributed to intrapartum events. INTERPRETATION: Substantial parental interest in understanding the causes of CP, together with uncertainty about the causes, parents' causal attributions, and significant emotional sequelae, highlight a strong need for provision of information and support for families of children recently diagnosed with CP. WHAT THIS PAPER ADDS: Understanding the causes of their child's cerebral palsy (CP) was important to parents. Parents most often endorsed intrapartum factors as a cause of CP. Parents reported experiencing strong emotions about the causes of their child's CP.

Distinct manifestations and potential mechanisms of seizures due to cortical versus white matter injury in children.

PURPOSE: To study seizure manifestations and outcomes in children with cortical versus white matter injury, differences potentially explaining variability of epilepsy in children with cerebral palsy. METHODS: In this population-based retrospective cohort study, MRIs of children with cerebral palsy due to ischemia or haemorrhage were classified according to presence or absence of cortical injury. MRI findings were then correlated with history of neonatal seizures, seizures during childhood, epilepsy syndromes, and seizure outcomes. RESULTS: Of 256 children studied, neonatal seizures occurred in 57 and seizures during childhood occurred in 93. Children with neonatal seizures were more likely to develop seizures during childhood, mostly those with cortical injury. Cortical injury was more strongly associated with (1) developing seizures during childhood, (2) more severe epilepsy syndromes (infantile spasms syndrome, focal epilepsy, Lennox-Gastaut syndrome), and (3) less likelihood of reaching > 2 years without seizures at last follow-up, compared to children without cortical injury. Children without cortical injury, mainly those with white matter injury, were less likely to develop neonatal seizures and seizures during childhood, and when they did, epilepsy syndromes were more commonly febrile seizures and self-limited focal epilepsies of childhood, with most achieving > 2 years without seizures at last follow-up. The presence of cortical injury also influenced seizure occurrence, severity, and outcome within the different predominant injury patterns of the MRI Classification System in cerebral palsy, most notably white matter injury. CONCLUSIONS: Epileptogenesis is understood with cortical injury but not well with white matter injury, the latter potentially related to altered postnatal white matter development or myelination leading to apoptosis, abnormal synaptogenesis or altered thalamic connectivity of cortical neurons. These findings, and the potential mechanisms discussed, likely explain the variability of epilepsy in children with cerebral palsy and epilepsy following early-life brain injury in general.

Neonatal well-being and timing of brain injury in persons with cerebral palsy born at term or late preterm.

AIM: To describe the distribution of neuroimaging patterns in a term/late preterm population-based cohort with cerebral palsy (CP), ascertain associations between neuroimaging patterns and neonatal well-being, estimate the proportion with antenatal or perinatal timing of neuropathology, and apply this information to the understanding of common mechanisms of brain injury and causal pathways. METHOD: The cohort for this observational study comprised 1348 persons born between 1999 and 2017 in Victoria, Australia. Using algorithms designed for the study, neonatal well-being and timing of brain injury were tabulated for the whole cohort and across neuroimaging patterns and birth epochs. RESULTS: Clinical and demographic profiles, neonatal well-being, and timing of brain injury differed across neuroimaging patterns. An estimated 57% of the cohort had a complicated neonatal period. Timing of brain injury was antenatal in 57% and perinatal in 41%. A decrease in the relative proportions of perinatal timing of brain injury was observed over a period when the rates of CP in live births at term decreased. INTERPRETATION: This study begins to bridge the knowledge gap about causation in CP, moving towards better description of the main mechanisms of brain injury and their contribution within CP cohorts, and facilitating the ability to monitor changes over time and the success of preventive measures.

Developmental outcomes for survivors of placental laser photocoagulation for the management of twin-to-twin transfusion syndrome.

BACKGROUND: Fetoscopic laser coagulation of placental anastomoses reverses the pathological process in twin-to-twin transfusion syndrome, thereby increasing survival, but there are a paucity of studies addressing long-term neurodevelopmental outcome of survivors. This study aimed to ascertain the presence of neurodevelopmental disabilities in child survivors of monochorionic pregnancies managed by placental laser photocoagulation in the Australian state of Victoria. METHODS: All pregnancies undergoing placental laser photocoagulation with the Victorian Fetal Therapy Service between 2006-2017 were included. Information on each surviving child, including demographics, perinatal course, and developmental progress was collected from parents, and consent was sought to complete the Child Behaviour Checklist. Interviewers evaluated whether this information was consistent with a diagnosis of any of 14 neurodevelopmental conditions. A three-tiered outcome measure was allocated for each child: (1) unimpaired or developmentally normal, (2) mild or moderate neurological impairment, or (3) severe neurological impairment. Clinical predictors for adverse outcome were identified. RESULTS: Of 116 pregnancies (113 twin, 3 triplet), 96 (83%) resulted in 1 + surviving fetuses. 57/113 (50%) twin pregnancies resulted in 2 survivors, 36 (32%) in 1 survivor, and 20 (18%) in no survivors. Of the 235 fetuses, 154 (65.5%) survived to follow-up. Survival increased from 59% in 2006-2008 to 73% in 2015-2017. 90/154 (58%) survivors were followed up at a mean age of 7.5 [SD 3.0] years. Based on parental interview and Child Behaviour Checklist data, 28/90 (31%) participants were assessed as having neurodevelopmental impairment, 27 of mild-moderate severity and 1 severe. Speech/language disorders, attention deficit (hyperactivity) disorders, and fine motor impairment were most common. Neonatal length of stay conferred the highest risk of impairment. CONCLUSION: Substantial variation exists between fetal therapy services in the type and length of neonatal follow-up following fetoscopic laser coagulation, contributing to a lack of data on long-term outcomes. The findings from this study support increasingly urgent calls to undertake systematic and sustained follow-up of fetoscopic laser coagulation survivors until school age. Information from this study may assist parents in their decision-making when offered fetal surgery. Importantly, it highlights a group for targeted surveillance and early intervention.

Paroxysmal Nonepileptic Events in Children With Epilepsy and Cerebral Palsy.

Objective: To determine the frequency of paroxysmal nonepileptic events in children with cerebral palsy due to brain injury who have epilepsy and to describe the factors associated with paroxysmal nonepileptic events. Methods: Retrospective, population-based study of children from the Victorian CP Register born 1999-2006. Neuroimaging, medical records, electroencephalograms (EEG), and EEG requests were analyzed. Results: Of the included 256 children, 87 had epilepsy. EEGs (with video correlation) were available for 82 of 87. Eighteen (18/82, 22%) had epileptic events captured on EEG. Twenty-one (21/82, 26%) had paroxysmal nonepileptic events captured on EEG. The majority (13/18, 77%) of children with epileptic events also had paroxysmal nonepileptic events captured. Ten parents and carers continued to report events as epileptic despite there being no ictal EEG correlate for specific events on multiple EEGs. There were no clear associations to identify which children would have ongoing paroxysmal nonepileptic events reported. Conclusions: Paroxysmal nonepileptic events were captured on EEG in one-fourth of children from this cerebral palsy cohort with epilepsy and available EEG. Half the parents and carers reported previously identified paroxysmal nonepileptic events as epileptic on subsequent EEGs, highlighting the need for clearer counseling so that parents better understand seizure semiology in children with EEG-proven paroxysmal nonepileptic events.

Survival of individuals with cerebral palsy in Victoria, Australia: A longitudinal cohort study spanning four decades.

AIM: To provide an updated description of the rates, trends, and predictors of mortality of individuals with cerebral palsy (CP), born in the Australian state of Victoria between 1970 and 2012. METHOD: Data were extracted for 4807 individuals (2091 females; 2716 males). The probability of survival to 30th June 2017 was calculated using the Kaplan-Meier method. Mortality rates were calculated per 1000 person-years using age strata and compared with population mortality rates to produce mortality ratios. Cox proportional hazards regression was used to calculate hazard ratios for selected demographic and clinical characteristics and to estimate the effect of birth epoch on 15-year survival. RESULTS: There were 666 recorded deaths. Compared to the general population, mortality was higher for all persons with CP and highest for children aged 1 to 15 years (45-62 times). We observed 35% improvement in the probability of survival to 15 years for births in the 2000s relative to the 1970s (hazard ratio 0.65, 95% confidence interval [CI] 0.49, 0.86), but only 4% improvement for the subgroup with complex CP (hazard ratio 0.96, 95% CI 0.69, 1.33). INTERPRETATION: The observed improvements in survival for those born in the 2000s is likely related predominantly to a proportional reduction in complex CP within the cohort. WHAT THIS PAPER ADDS: Length of survival improved for Australians with cerebral palsy (CP) born this millennium. Improved survival was mainly because of a proportional reduction in complex CP. A small improvement in length of survival was seen for children with complex CP.

Ten-year follow-up study found that motor-free intelligence quotient declined in children with mild-to-moderate cerebral palsy.

AIM: This 10-year follow-up study examined cognitive change in a cohort of children with cerebral palsy from preschool to adolescence at the group and individual levels. METHODS: The Wechsler Preschool and Primary Scale of Intelligence was administered to 80 children with cerebral palsy (mean = 4 years 6 months, standard deviation = 7 months) at baseline (Time 1). At 10-year follow-up (Time 2), 28 adolescents (mean = 14 years 6 months, standard deviation = 9 months) returned for assessment with the Wechsler Intelligence Scale for Children. Motor-free intelligence quotient (IQ) scores were calculated and paired-samples t-tests and the Reliable Change Index (RCI) were used to investigate change in IQ over time. RESULTS: At the group level, nonverbal IQ scores declined significantly. At the individual level, RCI indicated nine and 11 children showed a clinically significant decline in Full Scale IQ (FSIQ) and nonverbal IQ scores, respectively. Decline in FSIQ was related to a history of seizures whereas decline in nonverbal IQ was associated with higher initial IQ. CONCLUSION: Cognitive abilities in children with cerebral palsy evolve over time and selective deficits may not be observable until a later age, highlighting the importance of repeated cognitive assessment throughout childhood and adolescence.

Assessing IQ in adolescents with mild to moderate cerebral palsy using the WISC-V.

Objective: To examine the influence of subtests that require fine motor responses on measures of intellectual ability, and compare three approaches to minimizing motor demands while assessing cognitive abilities in adolescents with cerebral palsy (CP) to the traditional method of the Wechsler Intelligence Scale for Children - Fifth edition (WISC-V). Method: Seventy adolescents with CP (M = 14 years 6 months, SD = 10 months) who were able to provide either a verbal or point response were assessed using the WISC-V administered via Q-interactive. The pencil-to-paper version of Coding was also administered. Performance on Block Design and pencil-to-paper Coding was compared to Visual Puzzles and Coding on Q-interactive, respectively. Full Scale IQ (FSIQ) scores derived according to the Traditional method of the WISC-V were compared to alternative estimates of FSIQ derived according to the Q-interactive, Nonmotor, and Motor-free methods, which minimized motor demands. Results: An additional 7-12% of participants were able to respond to Visual puzzles and Coding on Q-interactive compared to Block Design and pencil-to-paper Coding, respectively, and performance was marginally but significantly better. For 54 adolescents (Gross Motor Function Classification System (GMFCS) Level I-III) who were able to obtain FSIQ scores, the Traditional method underestimated FSIQ by 3-6 points compared to the alternative methods and the difference was most pronounced for those with more severe CP as measured by the GMFCS. Conclusion: Adolescents with CP are at an inherent disadvantage when cognitive ability is assessed using the Traditional method of the WISC-V. Findings suggest clinicians should employ the Nonmotor or Motor-free methods when assessing IQ in adolescents with CP.

Second trimester maternal serum biomarkers and the risk of cerebral palsy.

AIMS: To investigate whether second trimester maternal serum screening (2TMSS) biomarkers are associated with cerebral palsy (CP) and identify CP characteristics associated with abnormal biomarker levels. METHOD: In this retrospective case-control data linkage study, we linked mothers of 129 singleton CP cases from a population register to their 2TMSS records and selected 10 singleton pregnancy controls per case (n = 1290). We compared mean and abnormal levels of alpha-fetoprotein (AFP), beta subunit of human chorionic gonadotrophin (ß-hCG), unconjugated estriol (uE3), and inhibin between cases and controls and within CP subgroups. RESULTS: Compared to control pregnancies, CP pregnancies had higher mean levels of AFP (1.10 vs. 1.01 multiple of the population median [MoM], p = 0.01) and inhibin (1.10 vs. 0.98 MoM, p ≤ 0.01). CP pregnancies were 2.5 times more likely to be associated with high levels of AFP (OR 2.52 [95% confidence interval [CI] 1.30, 4.65]; p < 0.01) and 2.6 times for inhibin (OR 2.63 [95% CI 1.37, 4.77]; p < 0.01), and 6.8 times when AFP and inhibin were both elevated (OR 6.75 [95% CI 2.41, 18.94]; p < 0.01). In CP cases, high AFP and high inhibin levels were associated with preterm birth and low birthweight. INTERPRETATION: Abnormal second-trimester biomarker levels suggest abnormal placentation plays a role in the causal pathway of some CP cases.

WISC-V motor-free cognitive profile and predictive factors in adolescents with cerebral palsy.

BACKGROUND: The most commonly used intelligence tests - the Wechsler Scales - do not provide standardised procedures for assessing children with motor impairment, and as a result, may underestimate the intelligence quotient (IQ) of young people with CP. AIMS: To characterise a motor-free cognitive profile of adolescents with CP using the Wechsler Intelligence Scale for Children - Fifth edition (WISC-V) and explore the influence of clinical factors on cognitive abilities. METHODS AND PROCEDURE: The WISC-V was used to assess cognitive abilities in 70 adolescents (M = 14 years 6 months, SD = 10 months). Sixty-six adolescents (Gross Motor Function Classification System (GMFCS) Level I, n = 26 ; II, n = 23; III, n = 15; IV, n = 1; V, n = 1) obtained either a Motor-free IQ or index score using the motor-free method. OUTCOMES AND RESULTS: MFIQ and index scores fell below the normative data and rates of borderline and impaired cognitive abilities were significantly higher in the CP group. Scores showed an uneven cognitive profile with a relative strength in verbal abilities. Severity of motor impairment and small for gestational age (SGA) were associated with lower IQ scores. A history of seizures was related to lower verbal abilities. CONCLUSIONS AND IMPLICATIONS: Cognitive abilities of adolescents with CP are significantly below expectation compared to normative data. Severity of motor impairment, SGA, and seizures need to be recognised by health professionals as risk factors for cognitive impairment. A substantial proportion of adolescents showed borderline cognitive abilities, constituting a group with CP which are relatively neglected in the literature.

First-trimester maternal serum biomarkers and the risk of cerebral palsy.

AIM: To investigate whether combined first-trimester screening (cFTS) biomarkers are associated with cerebral palsy (CP) and to identify CP characteristics associated with abnormal biomarker levels. METHOD: In this retrospective case-control data linkage study, we matched mothers of 435 singletons with CP from a population register to their cFTS records and selected 10 singleton pregnancy controls per case. We compared mean and abnormal levels (expressed as multiples of the median [MoMs]) of pregnancy-associated plasma protein-A (PAPP-A), beta subunit of human chorionic gonadotrophin (ß-hCG), and nuchal translucency between cases and controls and between CP subgroups. RESULTS: Compared with control pregnancies, CP pregnancies had lower mean levels of PAPP-A (0.95 vs 1.01 MoM, p=0.02) and ß-hCG (0.93 vs 0.99 MoM, p=0.02). Biomarker levels in CP pregnancies were 1.8 times more likely to be associated with abnormally low levels of PAPP-A (p<0.01), 1.4 times for ß-hCG (p=0.12), and 2.6 times for low PAPP-A and ß-hCG together (p=0.04). In cases with CP, an abnormally low PAPP-A level was associated with moderate preterm birth, low Apgar scores, and Gross Motor Function Classification System level V. Low ß-hCG was associated with very low birthweight. INTERPRETATION: Low first-trimester biomarker levels suggest a role for early pregnancy factors in some causal pathways to CP. WHAT THIS PAPER ADDS: Low first-trimester levels of biomarkers in maternal serum are associated with later cerebral palsy (CP). Early pregnancy factors have potential importance in causal pathways to CP. Causal pathways involving preterm birth, term neonatal encephalopathy, and genetic syndromes may be implicated.

Blood transfusion following major orthopaedic surgery in cerebral palsy: a retrospective analysis.

BACKGROUND: Progressive musculoskeletal pathology is ubiquitous among children with cerebral palsy (CP). Corrective surgery places them at risk of major blood loss and red blood cell (RBC) transfusion. Significant variability exists in uptake of perioperative patient blood management (PBM) strategies. This study aimed to examine factors contributing to RBC transfusion and assist in future development of care pathways. METHODS: A retrospective review at a tertiary paediatric hospital was undertaken to identify patients with CP undergoing either primary spinal fusion or single event, multilevel surgery (SEMLS) between 2010 and 2015. Solely soft tissue procedures were excluded. Data collected included demographics, Gross Motor Function Classification System level, surgical details, perioperative PBM and transfusion rates. Univariable analysis was performed to assess contributing factors to RBC transfusion. RESULTS: A total of 36 spinal fusion and 98 SEMLS patients were included. Preoperatively, 12% were anaemic, but only 19% had a ferritin checked. Overall, 49 patients (37%) received RBC transfusions. Intraoperative usage of tranexamic acid and cell salvage was 89% and 81%, respectively, for the spine cohort, and 22% and 3% for the SEMLS cohort. Successively higher Gross Motor Function Classification System levels, sodium valproate usage, longer surgical times, spinal fusion, pelvis instrumentation and more osteotomies were associated with RBC transfusion. CONCLUSION: More than one-third of CP patients who underwent major orthopaedic surgery received RBC transfusion. As expected, the more severely affected patients undergoing longer procedures were at highest risk. Significant improvements can be made in PBM to help optimize patients for surgery and minimize the need for transfusion.

Growth Trajectories in Genetic Subtypes of Prader-Willi Syndrome.

Prader-Willi syndrome (PWS) is a rare disorder caused by the loss of expression of genes on the paternal copy of chromosome 15q11-13. The main molecular subtypes of PWS are the deletion of 15q11-13 and non-deletion, and differences in neurobehavioral phenotype are recognized between the subtypes. This study aimed to investigate growth trajectories in PWS and associations between PWS subtype (deletion vs. non-deletion) and height, weight and body mass index (BMI). Growth data were available for 125 individuals with PWS (63 males, 62 females), of which 72 (57.6%) had the deletion subtype. There was a median of 28 observations per individual (range 2-85), producing 3565 data points distributed from birth to 18 years of age. Linear mixed models with cubic splines, subject-specific random effects and an autoregressive correlation structure were used to model the longitudinal growth data whilst accounting for the nature of repeated measures. Height was similar for males in both PWS subtypes, with non-deletion females being shorter than deletion females for older ages. Weight and BMI were estimated to be higher in the deletion subtype compared to the non-deletion subtype, with the size of difference increasing with advancing age for weight. These results suggest that individuals with deletion PWS are more prone to obesity.

Anxiety in children and adolescents with cerebral palsy.

AIMS: To describe the prevalence of, and factors associated with, anxiety in 6-18-year-old children with cerebral palsy (CP) and determine how often clinicians screen for and manage anxiety in this group. METHODS: Using a population CP register as the sampling pool, 569 families were approached by email, and 172 (mean age of children 12 years 7 months [SD 3 years 5 months]; 96 males) participated. Parents and, where able, children completed the Screen for Child Anxiety Related Emotional Disorders (SCARED). Parents also completed the Strengths and Difficulties Questionnaire. Children's medical records were searched for previous anxiety diagnoses and treatments. RESULTS: Clinically significant anxiety was identified in 38% of children on parent reports and 46% on child reports. Girls were twice as likely to have anxiety (p = 0.02). Parent- and child-reported scores were strongly correlated (r = 0.853). Fewer parents of children with intellectual and communication impairments completed the survey. Based on the SCARED parent reports, anxiety was not identified by a clinician in 16 children (43%) with clinically significant anxiety. CONCLUSION: Anxiety symptoms are prominent among children with CP, indicating a need for routine screening. Available screening tools are unsuitable for children with more severe limitations in cognition and communication; further research is needed to address this gap.

Anticholinergic medications for reducing drooling in children with developmental disability.

AIM: To determine: the effectiveness of three anticholinergic medications in reducing drooling in children with developmental disabilities (such as cerebral palsy, intellectual disability, and autism spectrum disorder), the frequency and nature of side effects, and their impact on treatment discontinuation. METHOD: After prescription of benzhexol hydrochloride, glycopyrrolate, or scopolamine patches at a tertiary saliva control clinic, all carers of 110 consecutive, eligible patients were recruited over a 5-year period. They provided data for 52 weeks, or until drug discontinuation, on compliance, drooling, adverse effects, and reasons for cessation. We evaluated and compared best drooling response, side effects, and drug cessation rates using survival analysis, and the effect of baseline variables on the discontinuation rate using proportional hazards regression. RESULTS: Among 110 participants (71 males, 39 females; mean age 8y 5mo [SD 4y 3mo], range 1y 11mo-18y 11mo), benzhexol, glycopyrrolate, and scopolamine were prescribed 81, 62, and 17 times respectively, with respective response rates of 85%, 75%, and 65%. Poor head control and poor oromotor function were predictive of poor response. Side effects frequently prompted drug cessation in males more than females (hazard ratio 1.8 [95% confidence interval 1.0-3.2], p=0.048). Glycopyrrolate had the fewest side effects. INTERPRETATION: Benzhexol, glycopyrrolate, and scopolamine reduce drooling, but improvement is offset by adverse side effects. Overall, glycopyrrolate performs best. WHAT THIS PAPER ADDS: In drooling, glycopyrrolate produced the greatest improvement with fewer side effects compared with benzhexol and scopolamine. Poor head control and poor oromotor function were associated with poor response. Medication side effects were common and often led to treatment discontinuation. Behavioural issues instigated cessation of benzhexol more often in males than females.

MEDICAMENTOS ANTICOLINÉRGICOS PARA REDUCIR EL BABEO EN NIÑOS CON TRASTORNOS DEL DESARROLLO: OBJETIVO: Determinar: la eficacia de tres medicamentos anticolinérgicos en la reducción del babeo en niños con trastornos del desarrollo (como parálisis cerebral, discapacidad intelectual y el trastorno del espectro autista), la frecuencia y la naturaleza de los efectos secundarios y su impacto en la interrupción del tratamiento. MÉTODO: Después de la prescripción de trihexifenidilo, glicopirrolato o parches de escopolamina en una clínica terciaria de control de saliva, fueron reclutados los cuidadores de 110 pacientes elegibles durante un período de 5 años. Estos proporcionaron datos sobre el cumplimiento, babeo, efectos adversos y razones para el cese, durante 52 semanas o hasta la interrupción del medicamento. Se evaluó y comparó la mejor respuesta al babeo, efectos secundarios y tasas de cese de drogas, utilizando el análisis de supervivencia y el efecto de las variables basales en la tasa de interrupción utilizando el modelo de riesgos proporcionales. RESULTADOS: Entre 110 participantes (71 varones y 39 mujeres; edad promedio de 8 años 5 meses [DE 4 años 3 meses], con rango de 1 año 11 meses - 18 años 11 meses), se prescribió trihexifenidilo, glicopirrolato y escopolamina, 81, 62, y 17 veces respectivamente, con tasas de respuesta respectivas de 85%, 75% y 65%. El pobre control cefálico y la mala función oromotora eran predictores de una respuesta deficiente. Los efectos secundarios provocaron el cese de las drogas con más frecuencia en los varones que en las mujeres (relación de riesgo 1,8 [intervalo de confianza del 95% 1,0-3,2], p 0,048). Glicopirrolato tuvo el menor número de efectos adversos INTERPRETACIÓN: El trihexifenidilo, el glicopirrolato y la escopolamina reducen el babeo, sin embargo, la mejora se contrarresta por los efectos secundarios. En general, el glicopirrolato tiene mejores resultados.

MEDICAÇÕES ANTICOLINÉRGICAS PARA REDUZIR SIALORRÉIA EM CRIANÇAS COM DEFICIÊNCIAS DESENVOLVIMENTAIS: OBJETIVO: Determinar: a efetividade de três medicações anticolinérgicas na redução da sialorréia em crianças com deficiências desenvolvimentais (como paralisia cerebral, deficiência intelectual, e transtorno do espectro autista), a frequência e natureza dos efeitos colaterais, e seu impacto na descontinuação do tratamento. MÉTODO: Após prescrição de hidroclorido benzexol, glicopirrolato, ou faixas de escopolamina em uma clínica terciária de controle da salivação, todos os cuidadores de 110 pacientes elegíveis consecutivos foram recrutados em um período de 5 anos. Eles forneceram dados de 52 semanas, ou até a descontinuação da medicação, sobre adesão, sialorréia, efeitos adversos, e razões para interrupção. Avaliamos e comparamos a melhor resposta para salivação, efeitos colaterais e interrupção da medicação usando análise de sobrevivência, e o efeito das variáveis na linha de base na taxa de descontinuação usando regressão de riscos proporcionais. RESULTADOS: Entre 110 participantes (71 do sexo masculino, 39 do sexo feminino; média de idade 8a 5m [DP 4a 3m], variação 1a 11m-18a 11m), benzexol, glicopirrolato, e escopolamina foram prescritos 81, 62, e 17 vezes respectivamente, com as respectivas taxas de resposta de 85%, 75%, and 65%. Pouco controle de cabeça e função oromotora foram preditivos de pior resposta. Efeitos colaterais mais frequentemente causaram interrupção da medicação em meninos do que em meninas (taxa de risco 1,8 [intervalo de confiança a 95% 1,0-3,2], p=0,048). Glicopirrolato teve menos efeitos colaterais. INTERPRETAÇÃO: Benzexol, glicopirrolato, ou escopolamina reduzem a sialorréia, mas as melhoras são atenuadas pelos efeitos colaterais. Em geral, glicopirrolato teve o melhor desempenho.

Long-term impact of saliva control surgery in children with disability.

Sialorrhea is a common problem in children with disability, often negatively affecting socialization, self-esteem, and burden of care. Saliva control surgery is an available option to manage this problem, particularly when other conservative methods have failed. As little is known about the long-term impact of surgery, we followed up 62 patients who had combined bilateral submandibular duct translocation and bilateral sublingual gland excision at our pediatric hospital between 1994 and 2014. Eligible individuals were identified through a search of ICD procedure codes. When families of patients were contacted successfully, they were invited to complete a 14-item questionnaire designed specifically for this study. The results indicated that long-term outcomes of surgery were very good; 13/62 (21%) individuals no longer had a drooling problem and another 30 (48%) experienced only mild to moderate drooling. Although 84% families reported some or major improvement in drooling, 9 families reported that they would not go through the experience again because of a difficult recovery period, lack of effectiveness of the intervention, changes in saliva consistency that caused coughing and gagging, and dental decay. None of the collected variables were predictive of good or poor outcome. The study indicated that surgical intervention is effective in the long term in the majority of cases and can be recommended to other families who attend our saliva control clinic.

Paediatric emergency department presentations due to feeding tube complications in children with cerebral palsy.

AIM: To describe the characteristics of emergency department (ED) presentations due to complications from gastrostomy or gastrojejunal feeding tubes among children with cerebral palsy (CP), the complexity of complications and the management approaches taken. METHODS: The Victorian CP Register was linked to the ED databases of Victoria's two tertiary paediatric hospitals, and data on presentations due to feeding tube complications were identified based on discharge diagnosis codes. Additional data on presentations were extracted from medical records. RESULTS: Over 5 years, there were 234 ED presentations due to feeding tube-related complaints among a CP cohort (n = 2183). ED notes were located for 183 of the 234 presentations. The majority of presentations (90%) involved children with severe gross motor impairment. A total of 46% of presentations (n = 84) was triaged as lower urgency, and 68% (n = 124) took place between 08:00 am and 06:00 pm. The most common presenting complaint was tube dislodgement (n = 105; 70%). No investigations were recorded in the majority of cases, and in almost 90% of cases, the feeding tube was successfully replaced in the ED, usually by an ED physician (n = 74) and less frequently by a surgeon (n = 9), gastroenterologist (n = 2) or nurse (n = 8); 9% (n = 17) resulted in a hospital admission. CONCLUSIONS: Most ED presentations due to feeding tube complaints in children with CP are in children with severe gross motor impairment but are able to be managed in the ED. As such, it is likely that care givers and other health professionals could manage some of the complications experienced in primary health-care settings closer to home.

Cerebral palsy trends in Australia (1995-2009): a population-based observational study.

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.

Dyskinetic vs Spastic Cerebral Palsy: A Cross-sectional Study Comparing Functional Profiles, Comorbidities, and Brain Imaging Patterns.

The authors aimed to describe the distribution of predominant and secondary motor types and compare functional profiles, comorbidities, and brain imaging patterns between dyskinetic and spastic cerebral palsy. Children recruited from a cerebral palsy register were assessed at age 5, 10, or 15. Motor types, topography, functional classifications, and comorbidities were recorded. Univariable logistic regression was used to compare dyskinesia with spasticity, with and without adjustment for topography. Neuroimaging classifications were extracted from the register. Of 243 children with spasticity or dyskinesia, the predominant motor type was spastic in 183 and dyskinetic in 56. Dyskinesia was associated with comparatively poorer function, total body involvement, and gray matter injury. After adjustment for topography, dyskinesia was associated with similar or better function. The study suggests that practical tools routinely incorporated into clinical practice would facilitate accurate and reliable classification of predominant and secondary motor types, topography, and functional abilities.

Intellectual disability in cerebral palsy: a population-based retrospective study.

AIM: A population-based observational study design was used to describe the epidemiology of intellectual disability in cerebral palsy (CP) in terms of clinical and neuroimaging associations, and to report the impact of intellectual disability on utilization of health services and length of survival. METHOD: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns (n=1141). Data linkage was undertaken to assess usage of hospital inpatient and emergency department services. Survival analysis was performed in a 30-year birth cohort (n=3248). RESULTS: Intellectual disability, present in 45% of the cohort, was associated with non-ambulation (47% vs 8%), later walking (mean 2y 7mo vs 1y 9mo), hypotonic (8% vs 1%) or dyskinetic (9% vs 5%) CP, a quadriplegic pattern of motor impairment (42% vs 5%), epilepsy (52% vs 12%), more emergency and multi-day hospital admissions, and reduced 35-year survival (96% vs 71%). Grey matter injuries (13% vs 6%), malformations (18% vs 6%), and miscellaneous neuroimaging patterns (12% vs 4%) were more common in people with intellectual disability. INTERPRETATION: Intellectual disability adds substantially to the overall medical complexity in CP and may increase health and mortality disparities. WHAT THIS STUDY ADDS: Cerebral maldevelopments and grey matter injuries are associated with higher intellectual disability rates. Health care is more 'crisis-driven' and 'reactive' in children with co-occurring intellectual disability. Length of survival is reduced in individuals with CP and co-occurring intellectual disability.