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Alternating hemiplegia in childhood: a cross-sectional study
S Nevsimalova, J Dittrich, M Havlova, A Tauberova, B Korsova, M Hajek, J Masopust, K Michalova
Jazyk angličtina Země Nizozemsko
Typ dokumentu kazuistiky, klinické zkoušky
Grantová podpora
IZ658
MZ0
CEP - Centrální evidence projektů
- 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
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.
Department of Neurology 1st Medical Faculty Charles University Prague Czech Republic
Citace poskytuje Crossref.org
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- $a 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.
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