Objective: Behavior problems in Dravet syndrome (DS) are common and can impact the lives of patients tremendously. The current study aimed to give more insight into (1) the prevalence of a wide range of specific behavior difficulties and aspects of health-related quality of life (HRQoL) in patients with DS compared with the general population (gp) and patients with epilepsy without DS, (2) the relations between these behavior problems and different aspects of HRQoL, and (3) the associations between seizure frequency, cognitive impairment (CI), behavior problems, and HRQoL, based on a conceptual model.
Methods: One hundred and sixteen patients (aged between 2 and 67 years), affected by SCN1A-related seizures, were included in the study.
Purpose: Differentiating between Dravet syndrome and non-Dravet SCN1A-related phenotypes is important for prognosis regarding epilepsy severity, cognitive development, and comorbidities. When a child is diagnosed with genetic epilepsy with febrile seizures plus (GEFS+) or febrile seizures (FS), accurate prognostic information is essential as well, but detailed information on seizure course, seizure freedom, medication use, and comorbidities is lacking for this milder patient group. In this cross-sectional study, we explore disease characteristics in milder SCN1A-related phenotypes and the nature, occurrence, and relationships of SCN1A-related comorbidities in both patients with Dravet and non-Dravet syndromes.
View Article and Find Full Text PDFObjective: The PAL project develops a conversational agent with a physical (robot) and virtual (avatar) embodiment to support diabetes self-management of children ubiquitously. This paper assesses 1) the effect of perceived similarity between robot and avatar on children's' friendship towards the avatar, and 2) the effect of this friendship on usability of a self-management application containing the avatar (a) and children's motivation to play with it (b).
Methods: During a four-day diabetes camp in the Netherlands, 21 children participated in interactions with both agent embodiments.