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Titolo:
Epilepsy in a representative series of Rett syndrome
Autore:
Steffenburg, U; Hagberg, G; Hagberg, B;
Indirizzi:
Univ Gothenburg, Queen Silvia Childrens Hosp, Dept Pediat, SE-41685 Gothenburg, Sweden Univ Gothenburg Gothenburg Sweden SE-41685 , SE-41685 Gothenburg, Sweden
Titolo Testata:
ACTA PAEDIATRICA
fascicolo: 1, volume: 90, anno: 2001,
pagine: 34 - 39
SICI:
0803-5253(200101)90:1<34:EIARSO>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
MENTALLY-RETARDED CHILDREN; NATURAL-HISTORY; SEIZURES; GIRLS; EPIDEMIOLOGY; POPULATION; CRITERIA; CORTEX;
Keywords:
epilepsy; Rett syndrome;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
34
Recensione:
Indirizzi per estratti:
Indirizzo: Steffenburg, U Univ Gothenburg, Queen Silvia Childrens Hosp, Dept Pediat, SE-41685 Gothenburg, Sweden Univ Gothenburg Gothenburg Sweden SE-41685 enburg, Sweden
Citazione:
U. Steffenburg et al., "Epilepsy in a representative series of Rett syndrome", ACT PAEDIAT, 90(1), 2001, pp. 34-39

Abstract

In a representative series of 53 females with Rett syndrome (RS), aged 5-55 y, a history of epilepsy was present in 50 (94%), 45 of whom had 5-y active epilepsy. Compared with severe mental retardation in general, the medianage of seizure onset was significantly later (4 vs 0.8 y) and partial complex seizures were more frequent (54% vs 23%). Neonatal seizures had occurred in only one and infantile spasms in none compared with 26% and 12%. Afterteenage, the severity of epilepsy tended to decrease, i.e. lower seizure frequency and relatively more partial seizures. The rate of being seizure-free for 1 y was 8% after 10 y and 40% after 27 y of epilepsy duration. Frequent seizures were associated with smaller head circumference. Conclusion: This epilepsy profile could fit in with present-day knowledge of RS as a form of dendrito-synaptogenic developmental failure with mainly late postnatal consequences, as well as being a relatively stationary condition in adulthood.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/01/20 alle ore 11:14:04