Electroencephalography in juvenile neuronal ceroid lipofuscinosis: visual and quantitative analysis.
Eur J Paediatr Neurol
; 5 Suppl A: 179-83, 2001.
Article
em En
| MEDLINE
| ID: mdl-11588993
ABSTRACT
Fourteen patients with a confirmed diagnosis of juvenile neuronal ceroid lipofuscinosis (JNCL) (aged 6-12.5 years at the beginning of the study) were prospectively followed for 5 years. An electroencephalogram (EEG) was recorded and analysed both visually and quantitatively and a neuropsychological examination was performed once a year. In addition, a cross-sectional EEG study of 32 patients aged 5-27 years was performed. The EEG was often normal before the age of 9 years, and thereafter a progressive background abnormality and increase in paroxysmal activity took place. The EEGs were significantly slower than those of the controls, and the speed of slowing of EEG correlated to the decrease in intelligence quotients (IQ). Quantitative analysis was superior to visual analysis in detecting the deterioration of the background activity. The best parameter describing this was the fast/slow ratio. Peak frequency, percentage of theta and the fast/slow ratio correlated with IQ.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Eletroencefalografia
/
Lipofuscinoses Ceroides Neuronais
Tipo de estudo:
Observational_studies
/
Prevalence_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Child
/
Humans
Idioma:
En
Revista:
Eur J Paediatr Neurol
Ano de publicação:
2001
Tipo de documento:
Article