A single case study of a family-centred intervention with a young girl with cerebral palsy who is a multimodal communicator

Background - This paper describes the impact of a family-centred intervention that used video to enhance communication in a young girl with cerebral palsy. This single case study describes how the video-based intervention worked in the context of multimodal communication, which included high-tech augmentative and alternative communication (AAC) device use. This paper includes the family's perspective of the video intervention and they describe the impact of it on their family. Methods - This single case study was based on the premise that the video interaction guidance intervention would increase attentiveness between participants during communication. It tests a hypothesis that eye gaze is a fundamental prerequisite for all communicative initiatives, regardless of modality in the child. Multimodality is described as the range of communicative behaviours used by the child and these are coded as AAC communication, vocalizations (intelligible and unintelligible), sign communication, nodding and pointing. Change was analysed over time with multiple testing both pre and post intervention. Data were analysed within INTERACT, a computer software to analyse behaviourally observed data. Behaviours were analysed for frequency and duration, contingency and co-occurrence. Results - Results indicated increased duration of mother's and girl's eye gaze, increased frequency and duration in AAC communication by the girl and significant change in frequency [χ2 (5, n = 1) = 13.25, P < 0.05] and duration [χ2 (5, n = 1) = 12.57, P < 0.05] of the girl's multimodal communicative behaviours. Contingency and co-occurrence analysis indicated that mother's eye gaze followed by AAC communication was the most prominent change between the pre- and post-intervention assessments. Conclusions - There was a trend for increased eye gaze in both mum and girl and AAC communication in the girl following the video intervention. The family's perspective concurs with the results

Outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression

OBJECTIVE: To determine whether multiple subpial transection in the posterior temporal lobe has an impact on long-term outcome in children who have drug-resistant Landau-Kleffner syndrome (LKS) or other "electrical status epilepticus during sleep" (ESES)-related regression. Given the wide variability in outcomes reported in the literature, a secondary aim was to explore predictors of outcome. METHODS: The current study includes a surgery group (n = 14) comprising patients who underwent multiple subpial transection of the posterior temporal lobe and a nonsurgery comparison group (n = 21) comprising patients who underwent presurgical investigations for the procedure, but who did not undergo surgery. Outcomes were assessed utilizing clinical note review as well as direct assessment and questionnaires. RESULTS: The distribution of nonclassical cases was comparable between groups. There were some differences between the surgery and nonsurgery groups at presurgical investigation including laterality of discharges, level of language impairment, and age; therefore, follow-up analyses focused on change over time and predictors of outcome. There were no statistically significant differences between the groups in language, nonverbal ability, adaptive behavior, or quality of life at follow-up. There was no difference in the proportion of patients showing improvement or deterioration in language category over time for either group. Continuing seizures and an earlier age of onset were most predictive of poorer quality of life at long-term follow-up (F2,23  = 26.2, p = <0.001, R(2)  = 0.714). SIGNIFICANCE: Both surgery and nonsurgery groups had similar proportions of classic LKS and ESES-related regression. Because no significant differences were found in the changes observed from baseline to follow-up between the two groups, it is argued that there is insufficient evidence to suggest that multiple subpial transection provides additional benefits over and above the mixed recovery often seen in LKS and related regressive epilepsies

Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech CBPP Congenital bilateral perisylvian poly- microgyria SOMA Schedule for Oral-Motor Assessment WDS Worster-Drought syndrome

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