Parental view of epilepsy in Rett Syndrome.

UNLABELLED: Few instruments exist to measure the impact of epilepsy on the quality of life in Rett Syndrome (RS). METHODS: We attended to describe seizures characteristics, parental opinion and quality of life related in RS by using a newly developed self administered questionnaire, postal sent to parents of French Association for Rett Syndrome (AFSR). RESULTS: Two-hundred completed questionnaires were returned. Mean age of patients was 14.8+/-8.1 years [3-42]. Parents reported that 70% of children had epileptic and non-epileptic seizures and mean age at first seizures was 7.3+/-5.1 years [1-24]. No statistical difference was found between the ages of first seizures, diagnosis of epilepsy and introduction of treatment. Seizures had a negative impact on child and family's life (68% of cases), strongly correlated to the existence of generalized, prolonged, cyanotic and drug-resistant seizures, on the child's level of alertness and progress in communication skills and psycho-social consequences such as fear of seizures, and difficulties to find home care aids. CONCLUSIONS: We identified major concerns of parents with RS that determine the impact of seizures on children and their family's quality of life. Our results suggest that in order to improve seizures management in RS, better information should reduce fear about seizures and should improve the quality of life of RS.

Deficit of preparatory attention in children with frontal lobe epilepsy.

We compared the performance of a sustained attention task by children with epilepsy in either the frontal or temporal lobe. In a new simple task that specifically measures preparatory attention, developed recently by LaBerge, Auclair, and Siéroff [LaBerge, D., Auclair, L., & Siéroff, E. (2000). Preparatory attention: Experiment and theory. Consciousness and Cognition, 9, 396-434], patients responded to a target presented in the centre of the display and ignored a distracter presented at locations to the right or the left side of the target. The distracter was presented prior to the onset of the target and the relative frequency of the distracter to target was varied within a block of trials (from 0% to 67%). Children with frontal lobe epilepsy showed a higher mean slope of response time to the target as a function of distracter probability compared to children with temporal lobe epilepsy or compared to the response time slope of control subjects. The response time slope of children with temporal lobe epilepsy did not differ from that of control subjects. These results indicate that the presence of frontal lobe epilepsy selectively affects the capacity of these patients to resist the interference a distracter.