Sleep slow-wave homeostasis and cognitive functioning in children with electrical status epilepticus in sleep.

STUDY OBJECTIVES: Encephalopathy with electrical status epilepticus in sleep (ESES) is characterized by non-rapid eye movement (non-REM)-sleep-induced epileptiform activity and acquired cognitive deficits. The synaptic homeostasis hypothesis describes the process of daytime synaptic potentiation balanced by synaptic downscaling in non-REM-sleep and is considered crucial to retain an efficient cortical network. We aimed to study the overnight decline of slow waves, an indirect marker of synaptic downscaling, in patients with ESES and explore whether altered downscaling relates to neurodevelopmental and behavioral problems. METHODS: Retrospective study of patients with ESES with at least one whole-night electroencephalogram (EEG) and neuropsychological assessment (NPA) within 4 months. Slow waves in the first and last hour of non-REM-sleep were analyzed. Differences in slow-wave slope (SWS) and overnight slope course between the epileptic focus and non-focus electrodes and relations to neurodevelopment and behavior were analyzed. RESULTS: A total of 29 patients with 44 EEG ~ NPA combinations were included. Mean SWS decreased from 357 to 327 µV/s (-8%, p < 0.001) across the night and the overnight decrease was less pronounced in epileptic focus than in non-focus electrodes (-5.6% vs. -8.7%, p = 0.003). We found no relation between SWS and neurodevelopmental test results in cross-sectional and longitudinal analyses. Patients with behavioral problems showed less SWS decline than patients without and the difference was most striking in the epileptic focus (-0.9% vs. -8.8%, p = 0.006). CONCLUSIONS: Slow-wave homeostasis-a marker of synaptic homeostasis-is disturbed by epileptiform activity in ESES. Behavioral problems, but not neurodevelopmental test results, were related to severity of this disturbance.

Reliability and Validity of the Supports Intensity Scale (SIS) Measured in Adults with Physical Disabilities.

The objective of this study was to investigate the internal consistency and the construct validity of the Dutch version of the Supports Intensity Scale (SIS-NL1.0; Buntinx 2006) in individuals with physical disabilities (N = 65). To investigate the construct validity, the relationship between SIS subscales and practical skills (Barthel Index; BI) was calculated. Support was provided for the internal consistency. The SIS subscales (except Behavior) had moderate to high intercorrelations and the SIS was able to discriminate between groups with different number of disabilities. However, weak relationships were found between the BI and four out of eight SIS subscales. For people with physical disabilities, future revisions of the SIS should also take into consideration limitations in practical skills in other support domains.

Polyfunctional compounds containing the 4,6-dialkoxy-7-arylthioheptene moiety as synthetically useful intermediates. The course of lewis acid-induced transformations.

Data on the selectivity of the Lewis acids induced transformations of the title compounds are presented, and the routes leading to formation of products containing either cyclohexane or 1,3-diene units are described.

New C-C coupling reaction of cyclic nitronates with carbon nucleophiles. Umpolung of the conventional reactivity of nitronates.

Cyclic six-membered nitronates 1 are involved in diastereoselective C-C coupling reactions with various nucleophiles in the presence of either catalytic or stoichiometric amounts of TBDMSOTf to give the previously unknown N-siloxytetrahydrooxazines. The intermediacy of N,N-bis(oxy)iminium cations was proven by NMR data.