EEG as a possible prognostic tool in phenylketonuria

Giorgis, Giulia Fanny De; Antonozzi, I.; Castello, Philippe; Rosano, Marcello; Loizzo, Alberto

doi:10.1016/0013-4694(83)90147-5

Electroencephalography and Clinical Neurophysiology

1983

DOI: 10.1016/0013-4694(83)90147-5

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EEG as a possible prognostic tool in phenylketonuria

Giulia Fanny De Giorgis

I. Antonozzi

Philippe Castello

et al.

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Cited by 8 publications

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“…No initative or spike and wave complexes were seen (1 2). Di Giorgis et al ( 13) in their prospective study of PKU infants in the 1st yr of life concluded that the longer and higher the increase was in blood phenylalanine concentration, the more prone the infant was to epileptiform alterations of EEG. None of the groups investigated older, early-treated patients under conditions in which electrical changes were used to monitor brain function within the same patient.…”

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Phenylalanine Alters the Mean Power Frequency of Electroencephalograms and Plasma L-DOPA in Treated Patients with Phenylketonuria

et al. 1986

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ABSTRACT. Phenylketonuria is a human model for the study of the effects of phenylalanine on brain function. We found previously a correlation between high blood phenylalanine, prolonged performance times on neuropsychological tests of higher integrative function, and decreased urinary dopamine in 10 patients. In this protocol we examine changes in triplicate of plasma dihydroxyphenylalanine (L-DOPA) and the mean power frequency of the electroencephalogram in eight additional older patients with phenylketonuria using longer intervals in a blinded, cross-over design. Mean power frequency was obtained by Fourier transform of the power spectrum from traditional eight channel electroencephalograms. Plasma L-DOPA was quantitated by radioenzymatic methods. In all patients statistically significant decreases were found in the mean power frequency of the electroencephalogram and in plasma L-DOPA when plasma L-phenylalanine increased. These findings were reversible and correlated in the reverse direction when plasma L-phenylalanine was reduced. PKU is the prototypic experiment of nature by which adverse effects of high circulating blood phenylalanine on human brain structure and function are studied. Two different pathological phenomena are postulated as caused by elevated concentrations of phenylalanine. First, in the developing human brain, from fetal life to 6 months of age, irreversible changes in myelin, brain structure, and neuronal migration are described (1-5). Second, elevated blood phenylalanine concentrations may impair the fully myelinated human brain by reversible mechanisms involving neurotransmitter production (6-9). This latter phenomenon Received April 2. 1986: accepted June 6, 1986. Address all correspondence and request for reprints to Dr. Louis J. Elsas, Medical Genetics, Emory University, 2040 Ridgewood Drive, Atlanta, GA 30322.This work was supported by Grant 12-1 10 from the March of Dimes and a Grant 2-MOI-RR00039-25 from the National Institutes of Health.has considerable importance when one considers the adverse effects of diet discontinuation on intellectual achievement and behavior of early treated, 6 yr olds with PKU (10).Recently, we approached the issue of developing noninvasive, sensitive discriminants of brain function by quantitating catecholamine production and neuropsychological tests of performance in patients with high and low blood phenylalanine concentrations. In this study we found prolonged performance times in tests of higher integrative function but not for tests of lower integrative function when plasma phenylalanine rose (8). When plasma phenylalanine concentration was elevated, urinary excretion of dopamine decreased (8). Since in well patients it is not ethical to measure neurotransmitters in brain or cerebrospinal fluid and since neuropsychological tests are subject to many variables such as the individual's competence, environmental conditions, and rapport with examiners, we sought better tests which were safe, noninvasive, repeatable, and subject to statistical analysis. W...

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Phenylalanine Alters the Mean Power Frequency of Electroencephalograms and Plasma L-DOPA in Treated Patients with Phenylketonuria

et al. 1986

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Pattern-reversal visual evoked potentials in phenylketonuric children

et al. 1987

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Pattern-reversal visual evoked potentials (PR-VEPs) and EEG were recorded in 14 phenylketonuric (PKU) children on a low-phenylalanine (phe) diet; the data obtained were correlated with metabolic parameters, namely, the actual phe plasma level, the mean phe plasma level in the last year, an the beginning of the diet. PR-VEPs seem to be more sensitive than EEG in detecting neurophysiological derangements in these subjects; in fact PR-VEPs were pathological in six patients while EEG detected three; no significant alterations were found in the neurophysiological tests among the children with good metabolic control, and only one child was abnormal among the six on an early dietetic regimen; in contrast, six of the nine subjects presenting with high mean phe plasma levels (greater than 10 mg/100 ml) and five of the eight whose diet started after the 2nd month of life showed pathological PR-VEPs.

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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

Cited by 8 publications

References 27 publications

Phenylalanine Alters the Mean Power Frequency of Electroencephalograms and Plasma L-DOPA in Treated Patients with Phenylketonuria

Phenylalanine Alters the Mean Power Frequency of Electroencephalograms and Plasma L-DOPA in Treated Patients with Phenylketonuria

Pattern-reversal visual evoked potentials in phenylketonuric children

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

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