Sheena Reilly

Prevalence of feeding problems and oral motor dysfunction in children with cerebral palsy: A community survey☆☆☆★

OBJECTIVE To determine the prevalence and nature of feeding difficulties and oral motor dysfunction among a representative sample of 49 children with cerebral palsy (12 to 72 months of age). STUDY DESIGN A population survey was undertaken by means of a combination of interview and home observational measures. RESULTS Sucking (57%) and swallowing (38%) problems in the first 12 months of life were common, and 80% had been fed nonorally on at least one occasion. More than 90% had clinically significant oral motor dysfunction. One in three (36.2%) was severely impaired and therefore at high risk of chronic undernourishment. There was a substantial discrepancy between the lengthy duration of mealtimes reported by mothers and those actually observed in the home (mean, 19 minutes 21 seconds; range, 5 minutes 21 seconds to 41 minutes 39 seconds). In 60% of the children, severe feeding problems preceded the diagnosis of cerebral palsy. CONCLUSIONS Using a standardized assessment of oral motor function, we found the majority of children to have clinically significant oral motor dysfunction. Contrary to maternal report, mealtimes were relatively brief, and this, combined with the severity of oral motor dysfunction, made it difficult for some children to achieve a satisfactory nutritional intake. The study illustrates the importance of observing feeding, preferably in the home.

Early treatment with parenteral penicillin in meningococcal disease.

OBJECTIVE--To measure the effect of parenteral antibiotics given before admission to hospital on mortality and on bacteriological investigations in meningococcal disease. DESIGN--Retrospective review of hospital notes and laboratory and public health medicine department records. SETTING--Three health districts in south west England. SUBJECTS--Patients with meningococcal disease in Gloucester district presenting between 1 January 1982 and 31 December 1991 (n = 190); patients with meningococcal disease in Plymouth (n = 118) and Bath (n = 73) districts presenting between 1 January 1988 and 31 December 1991 (total = 381). MAIN OUTCOME MEASURE--Number of deaths from meningococcal disease. RESULTS--Parenteral antibiotic given by general practitioners was associated with a substantial reduction in mortality (from 9% to 5%; relative risk 0.6, 95% confidence interval 0.2 to 1.5); patients with a rash were more likely to be given parenteral antibiotics, and mortality was further reduced (from 12% to 5%; 0.5, 0.2 to 1.4). In a district where such treatment was regularly encouraged its use increased from 5% to 40% of cases over 10 years (p = 0.00001). Treatment with parenteral antibiotics before admission made isolation of meningococci from blood and cerebrospinal fluid less likely but did not affect nasopharyngeal cultures. CONCLUSIONS--General practitioners should carry benzylpenicillin in their emergency bags at all times and should administer it promptly, preferably intravenously, whenever meningococcal disease is suspected, unless the patient has had an anaphylactic reaction to penicillin. Specimens for culture should include a nasopharyngeal swab.

Predicting Language Outcomes at 4 Years of Age: Findings From Early Language in Victoria Study

OBJECTIVE: To quantify the contributions of child, family, and environmental predictors to language ability at 4 years. METHODS: A longitudinal study was performed with a sample of 1910 infants recruited at 8 months in Melbourne, Australia. Predictors were child gender, prematurity, birth weight and order, multiple birth, socioeconomic status, maternal mental health, vocabulary, education, and age at childs birth, non–English-speaking background, and family history of speech/language difficulties. Outcomes were Clinical Evaluation of Language Fundamentals-Preschool, language scores, low language status (scores >1.25 SDs below the mean), and specific language impairment (SLI) (scores >1.25 SDs below the mean for children with normal nonverbal performance). RESULTS: A total of 1596 children provided outcome data. Twelve baseline predictors explained 18.9% and 20.9% of the variation in receptive and expressive scores, respectively, increasing to 23.6% and 30.4% with the addition of late talking status at age 2. A total of 20.6% of children (324 of 1573 children) met the criteria for low language status and 17.2% (251 of 1462 children) for SLI. Family history of speech/language problems and low maternal education levels and socioeconomic status predicted adverse language outcomes. The combined predictors discriminated only moderately between children with and without low language levels or SLIs (area under the curve: 0.72–0.76); this improved with the addition of late talking status (area under the curve: 0.78–0.84). CONCLUSIONS: Measures of social disadvantage helped explain more variation in outcomes at 4 years than at 2 years, but ability to predict low language status and SLI status remained limited.

Diversity of participation in children with cerebral palsy

The aim of this study was to investigate the participation of children with cerebral palsy (CP) in activities outside school and to compare their participation with a large representative sample of children. A population‐based survey was conducted of children with CP born in Victoria, Australia in 1994 and 1995. Of 219 living children identified, 114 (52.1%) returned completed surveys. The children (65 males, 49 females) were aged between 10 years 9 months and 12 years 9 months (mean age 11y 9mo, SD 6mo). Thirty‐eight per cent had hemiplegia, 23% diplegia, 4% triplegia, 34% quadriplegia, and 1% was of unknown topography. Distribution according to the Gross Motor Function Classification System (GMFCS) was 22.8% Level I, 36% Level II, 10.5% Level III, 8.8% Level IV, and 21.9% Level V. Distribution according to the Manual Ability Classification System (MACS) was: 19.3% Level I, 38.6% Level II, 14.0% Level III, 8.8% Level IV, and 19.3% Level V. Participation was measured using the Childrens Assessment of Participation and Enjoyment. Participation in selected sport, cultural, and quiet leisure activities was compared with population‐based data for 11‐year‐olds from the Australian Bureau of Statistics. Children with CP undertook a median of 26.5 activities (interquartile range 10) in 4 months which were commonly informal rather than formal. Intensity of participation was low. Diversity and intensity of participation was similar for children in each level of the MACS and the GMFCS, except for participants in Level V. More children with CP participated in organized sports (p<0.001) compared with other Australian children, although with lower frequency (p<0.001). Participation diversity and level of intensity of Australian children with CP were similar to those reported in a Canadian study.

Predicting Language at 2 Years of Age: A Prospective Community Study

OBJECTIVE. This article responds to evidence gaps regarding language impairment identified by the US Preventive Services Task Force in 2006. We examine the contributions of putative child, family, and environmental risk factors to language outcomes at 24 months of age. METHODS. A community-ascertained sample of 1720 infants who were recruited at 8 months of age were followed at ages 12 and 24 months in a prospective, longitudinal study in metropolitan Melbourne, Australia. Outcomes at 24 months were parent-reported infant communication (Communication and Symbolic Behavior Scales and MacArthur-Bates Communicative Development Inventories vocabulary production score). Putative risk factors were gender, preterm birth, birth weight, multiple birth, birth order, socioeconomic status, maternal mental health, maternal vocabulary and education, maternal age at birth of child, non–English-speaking background, and family history of speech-language difficulties. Linear regression models were fitted to total standardized Communication and Symbolic Behavior Scales and Communicative Development Inventories vocabulary production scores; a logistic regression model was fitted to late-talking status at 24 months. RESULTS. The regression models accounted for 4.3% and 7.0% of the variation in the 24-month Communication and Symbolic Behavior Scales and Communicative Development Inventories scores, respectively. Male gender and family history were strongly associated with poorer outcomes on both instruments. Lower Communication and Symbolic Behavior Scales scores were also associated with lower maternal vocabulary and older maternal age. Lower vocabulary production scores were associated with birth order and non–English-speaking background. When the 12-month Communication and Symbolic Behavior Scales Total score was added as a covariate in the linear regression of 24-month Communication and Symbolic Behavior Scales Total score, it was by far the strongest predictor. CONCLUSIONS. These early risk factors explained no more than 7% of the variation in language at 24 months. They seem unlikely to be helpful in screening for early language delay.

ORAL‐MOTOR DYSFUNCTION AND FAILURE TO THRIVE AMONG INNER‐CITY INFANTS

Non‐organic failure to thrive among socio‐economically deprived inner‐city infants may be associated with abnormal oral‐motor functioning and aspects of social adversity, such as disorganised mealtimes. A Feeding Assessment Schedule was devised to rate oral‐motor behaviour objectively and was used to test nine pairs of case and comparison children while they were being fed at home. Video‐recordings were made for later analysis. The case infants had immature and abnormal oral‐motor development that made them less able to be fed successfully. Temperamentally they were more ‘difficult’ than the comparison infants, and they were less adept at signalling their needs during meal‐times. The case infants also were fed in inappropriate positions for their age, with more distractions and less suitable utensils.

Predicting Stuttering Onset by the Age of 3 Years: A Prospective, Community Cohort Study

OBJECTIVES. Our goals were to document (1) the onset of stuttering and (2) whether specific child, family, or environmental factors predict stuttering onset in children aged up to 3 years. METHODS. Participants included a community-ascertained cohort of 1619 2-year-old Australian children recruited at 8 months of age to study the longitudinal development of early language. The main outcome measure was parental telephone report of stuttering onset, verified by face-to-face expert diagnosis. Preonset continuous measures of the childs temperament (approach/withdrawal) and language development were available. Information on a range of predictor measures hypothesized to be associated with stuttering onset was obtained (maternal mental health and education levels, gender, premature birth status, birth weight, birth order, twinning, socioeconomic status, family history of stuttering). RESULTS. By 3 years of age, the cumulative incidence of stuttering onset was 8.5%. Onset often occurred suddenly over 1 to 3 days (49.6%) and involved the use of word combinations (97.1%). Children who stuttered were not more shy or withdrawn. Male gender, twin birth status, higher vocabulary scores at 2 years of age, and high maternal education were associated with stuttering onset. The multivariable model, however, had low predictive strength; just 3.7% of the total variation in stuttering onset was accounted for. CONCLUSIONS. The cumulative incidence of stuttering onset was much higher than reported previously. The hypothesized risk factors for stuttering onset together explained little of the variation in stuttering onset up to 3 years of age. Early onset was not associated with language delay, social and environmental factors, or preonset shyness/withdrawal. Health professionals can reassure parents that onset is not unusual up to 3 years of age and seems to be associated with rapid growth in language development.

The Rett Syndrome Behaviour Questionnaire (RSBQ): refining the behavioural phenotype of Rett syndrome

BACKGROUND Although physical features, including loss of hand skills, deceleration of head growth, spasticity and scoliosis, are cardinal features of Rett syndrome (RS), a number of behavioural features are also associated with the disorder, including hand stereotypies, hyperventilation and breath holding. No study has tested the specificity of these behavioural features to individuals with RS, compared to individuals with severe to profound mental retardation (SMR). METHOD A novel checklist of characteristic RS behavioural and emotional features, the Rett Syndrome Behaviour Questionnaire (RSBQ), was developed to test the type and specificity of behavioural features of RS against those found in girls with SMR. RESULTS After controlling for the effects of RS-related physical disabilities, the RSBQ discriminated between the groups. Some aspects of the behaviours found to be specific to RS are included in the necessary or supportive RS diagnostic criteria, notably hand behaviours and breathing problems. Additional behavioural features were also more frequently reported in the RS than the SMR group, including mood fluctuations and signs of fear/anxiety, inconsolable crying and screaming at night, and repetitive mouth and tongue movements and grimacing. CONCLUSIONS Full validation of the scale requires confirmation of its discriminatory power and reliability with independent samples of individuals with RS and SMR. Further delineation of the specific profile of behaviours seen in RS may help in identification of the function of the MECP2 gene and in improved differential diagnosis and management of individuals with RS.

Foreign Body Ingestion in Children with Severe Developmental Disabilities: A Case Study

Dysphagia is common in children with severe developmental disabilities. The nature of these difficulties can predispose them to foreign body ingestion. This article presents a case that highlights the need for vigilance in diagnosing dysphagia in children with multiple and complex developmental disabilities where severe cognitive impairment and an inability to communicate may mask the presence of underlying problems.

Growth of infant communication between 8 and 12 months: a population study.

Aim:  To describe changes in infant prelinguistic communication skills between 8 and 12 months, and identify factors associated with those skills.