Aspects Évolutifs De L'E.E.G. Dans Les Hyperphénylalaninémies

In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n = 34) and 15th (n = 18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th-15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with "strict" dietary control showed a significantly higher IQ than those with "loose" control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.

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Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early

Pietz

Benninger

Schmidt

et al. 1988

Eur J Pediatr

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EEG development in early treated PKU patients from birth to 6 years of age

et al. 1990

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In 126 early treated PKU patients (type I and type II) a close EEG follow up was performed from birth up to 6 years of age. A total of 1465 EEGs were performed before and after onset of dietary treatment and on 11 more subsequent occasions. The composition of the background activity was normal up to 6 years when only a small number of the children (19) showed no dominant alpha activity. The frequency of epileptiform activity of generalised as well as focal type was low in the first 2 years of life, but afterwards slightly enhanced in comparison to normal control groups. Other findings like generalised theta paroxysms or focal slow waves were rarely observed. Under a standardised protein load at 6 months (52 patients) and at 5 years of age (42 patients) a moderate generalised slowing of the background activity but no other abnormalities were noted.

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EEG as a possible prognostic tool in phenylketonuria

Giorgis

Antonozzi

Castello

et al. 1983

Electroencephalography and Clinical Neurophysiology

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Aspects Évolutifs De L'E.E.G. Dans Les Hyperphénylalaninémies

Cited by 4 publications

References 6 publications

Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early

Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early

EEG development in early treated PKU patients from birth to 6 years of age

EEG as a possible prognostic tool in phenylketonuria

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