"IZ658" Dotaz Zobrazit nápovědu
The clinical and polygraphic characteristics of narcolepsy in children were established on the analysis of 97 reported cases in children (including 12 personal cases). In idiopathic narcolepsies (77 cases) narcoleptic attacks occurred in 97% of the cases, cataplexy in 80.5%, hypnagogic hallucination in 39% and sleep paralysis in 29%; 13% of the children had the tetrad; dyssomnia was a prominent feature. Polygraphic data showed no significant differences between adults and children. In symptomatic narcolepsies (20 cases): cataplexy was the prominent feature occurring in 95% of the cases, 26% of the children had status cataplecticus; in these narcoleptic-cataplectic syndromes there was often an absence of polygraphic evidence of narcolepsy. Symptomatic narcolepsy should be suspected in cases where narcolepsy is detected in preteenage children, where cataplectic attacks are abnormally frequent, where there is an absence of polygraphic evidence of classical narcolepsy (although this criterion may not apply in the case of younger children) or where human leukocyte antigen typing for DR2 is negative. An association with a Niemann-Pick disease type C was found in 12 out of the 20 symptomatic cases, this association merits further study.
- MeSH
- dítě MeSH
- HLA-DR2 antigen MeSH
- katalepsie MeSH
- lidé MeSH
- narkolepsie * diagnóza MeSH
- polysomnografie MeSH
- předškolní dítě MeSH
- spánek REM MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- srovnávací studie MeSH
Six patients with alternating hemiplegia in childhood (AHC) were followed, some of them up to childhood. Progressive intellectual deterioration and disturbance of pyramidal, extrapyramidal and cerebellar functions were found in all of them. Multimodal evoked potential abnormalities, changes of sleep structure and HMPAO-SPECT results were correlated with increasing clinical handicap. In older patients increases in the plasma level of lactate, as well as in the lactate: pyruvate ratio, were revealed, accompanied by elevated inorganic phosphate (Pi) values on 31P MR spectroscopy. These findings support the hypothesis of a possible secondary mitochondrial deficit in AHC. However, no specific bioptic changes (muscle, skin, or buccal mucous membrane) were found, and thus the etiology of AHC remains unclear.
- MeSH
- dítě MeSH
- dospělí MeSH
- elektroencefalografie MeSH
- elektromyografie MeSH
- energetický metabolismus fyziologie MeSH
- flunarizin terapeutické užití MeSH
- hemiplegie farmakoterapie patologie radiografie MeSH
- jednofotonová emisní výpočetní tomografie MeSH
- kyselina mléčná MeSH
- kyselina pyrohroznová MeSH
- laktáty krev MeSH
- lidé MeSH
- mladiství MeSH
- průřezové studie MeSH
- pyruváty krev MeSH
- sluchové evokované potenciály fyziologie MeSH
- spánek fyziologie MeSH
- svalová spasticita patologie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
- klinické zkoušky MeSH
- MeSH
- dospělí MeSH
- fenmetrazin aplikace a dávkování terapeutické užití MeSH
- hypertenze MeSH
- kataplexie farmakoterapie komplikace MeSH
- koronární nemoc MeSH
- lidé MeSH
- narkolepsie farmakoterapie komplikace MeSH
- senioři MeSH
- spánek REM MeSH
- stárnutí MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
Excessive fragmentary myoclonus during sleep consists of high amounts of brief twitch-like movements occurring asynchronously and asymmetrically in different body areas and has been reported to occur in association with a number of sleep disorders. It was analyzed using a new technique of quantification, the fragmentary myoclonus index (FMI). The FMI exhibited high rates in all stages of sleep but with a somewhat lower frequency in slow wave sleep explaining, as well, a significantly lower rate in the first hour after onset compared to later hours. There was no evidence for greater sleep fragmentation or lighter sleep compared to a matched patient group in whom it had not been noted.
- MeSH
- časové faktory MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- myoklonus * patofyziologie MeSH
- polysomnografie MeSH
- senioři MeSH
- spánek REM fyziologie MeSH
- stadia spánku * fyziologie MeSH
- stárnutí fyziologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- práce podpořená grantem MeSH
- MeSH
- dítě MeSH
- dospělí MeSH
- evokované potenciály MeSH
- hemiplegie patofyziologie MeSH
- laktáty krev MeSH
- lidé MeSH
- mitochondrie MeSH
- neurologické manifestace MeSH
- poruchy spánku a bdění MeSH
- vývojové poruchy u dětí MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
