This paper describes the communication development in 11 children with Angelman's syndrome. We have investigated the clinical observation that these children appear to have a greater ability with receptive rather than expressive language and assessed these skills using published communication schedules; the Bzoch‐League (REEL) Scale and the Pre‐verbal Communication Schedule. In addition, we looked at the understanding and use of non‐verbal communication, such as natural gesture. The data collected did not, in fact, show a significant gap between receptive and expressive skills, except in the older children, but it did highlight the fact that these children develop very few words and have difficulty in using gestural or sign systems. It also emphasised the different patterns of ability in familial and non‐familial cases, although the small numbers in this study made it inappropriate to conclude anything significant from the data. The results of the study will be presented and the implications for the children's remedial programmes will be discussed, particularly in the use of Makaton signing and other methods of augmentative communication.

The communication development in 11 children with Angelman's syndrome is described. The clinical observation that these children appear to have a greater ability with receptive rather than expressive language is investigated and these skills assessed using published communication schedules. In addition the understanding and the use of nonverbal communication such as natural gesture was studied. The data collected highlight the fact that these children have developed very few words and have difficulty in using gestural or sign systems. This has implications for speech and language therapists and the children's remedial programmes. Possible future longitudinal studies are suggested.

OBJECTIVE The high prevalence and impact of neurodevelopmental comorbidities in childhood epilepsy are now well known, as are the increased risks and familial aggregation of reading disability (RD) and speech sound disorder (SSD) in rolandic epilepsy (RE). The risk factors for RD in the general population include male sex, SSD and ADHD but it is not known if these are the same in RE or whether there is a contributory role of seizure and treatment related variables. METHODS An observational study of 108 RE probands (age range 3.6–22 years) and their 159 siblings (age range 1–29 years; 83 with EEG data) singly ascertained in the US or UK through an affected RE proband. We used a nested case-control design, multiple logistic regression and generalized estimating equations to test the hypothesis of association between RD and seizure variables or antiepileptic drug treatment in RE; we also assessed an association between EEG focal sharp waves and RD in siblings. RESULTS RD was reported in 42% of probands and 22% of siblings. Among probands, RD was strongly associated with a history of SSD (OR 9.64, 95% CI: 2.45–37.21), ADHD symptoms (OR 10.31, 95% CI: 2.15–49.44), and male sex (OR 3.62, 95% CI: 1.11–11.75), but not with seizure or treatment variables. Among siblings, RD was independently associated only with SSD (OR 4.30, 95%CI: 1.42–13.0) and not with the presence of interictal EEG focal sharp waves. SIGNIFICANCE The principal risk factors for RD in RE are SSD, ADHD and male sex, the same risk factors as for RD without epilepsy. Seizure or treatment variables do not appear to be important risk factors for RD in RE probands, and there was no evidence to support interictal EEG focal sharp waves as a risk factor for RD in siblings. Future studies should focus on the precise neuropsychological characterisation of RD in RE families, and on the effectiveness of standard oral-language and reading interventions.

AIM Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. As such, it falls within the cerebral palsies. The aim of this study was to describe the physical and neuropsychological profiles of children with WDS.METHOD Forty-two children with WDS (26 males, 16 females; mean age 7y 10mo, SD 3y 1mo; range 2y 6mo to 16y 5mo) were studied prospectively using a standard protocol.RESULTS All of the children had severe bulbar dysfunction; 36 out of 42 had feeding difficulties and 23 of 38 had unintelligible speech, which was poorly compensated for by augmentative communication. There were accompanying disturbances in cognition (mean non-verbal IQ 59), behaviour (12 ⁄ 40 attention-deficit-hyperactivity disorder [ADHD]), social communication (8 ⁄ 42 autism), and epilepsy (12 ⁄ 39). The severity of bulbar dysfunction and impact of additional impairments made it difficult to use formal assessments.

Some children with severe motor disorders have unintelligible speech, and may be recommended augmentative communication systems, such as a symbol chart or a voice output aid. The paper reports the outcome after 15-18 months for 35 children of recommendations for augmentative communication. Using structured questionnaires, parents were asked whether equipment was provided as recommended. Their perception of success in children's use of augmentative aids was recorded and related to potentially influential factors. Twenty-five symbol systems, 10 speech output devices and 11 switches were received; 18 symbol systems were used for communication and 10 were used frequently. Seven speech output devices were used for communication but only two were reported to be used frequently. Factors leading to more successful outcomes include early receipt of the aid, perceived adequate local training in the use of the aid, and children aged 6 years or more at initial assessment. The findings also suggest that referring professionals will need to be better informed about the nature and limitations of augmentative communication aids, and that improved local professional input and careful interagency planning and co-ordination are required to achieve optimal outcome.