先天代謝異常と脳波

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  • EEG Findings in Inborn Errors of Metabolism

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In phenylketonuria, an improvement of EEG findings was observed in 5 of 7 cases after the treatment with a low phenylalanine diet. The EEG findings were deteriorated by the phenylalanine load.<BR>In hypervalinemia, the EEG abnormalities such as spike discharges improved on a low valine diet, but were aggravated on an unrestricted diet. In these disorders, the brain dysfunction seemed to be mainly based on excess of metabolites.<BR>In vitamin B6 dependent convulsion, seizure discharges disappeared immediately after administration of vitamin B6. It was assumed that both excess of glutamate and lack of GABA might cause brain dysfunction in this disorder.<BR>In phosphoribosylpyrophosphate synthetase deficiency, hypsarhythmia improved after intra muscular administration of ACTH and activity of this enzyme increased synchronously. One of the effects of ACTH on hypsarhythmia seemed to be related, to some extent, to an elevated enzyme activity.<BR>In Tay-Sachs disease, EEG showed low amplitude fast waves at the early stage, and slow waves mixed with seizure discharges appeared after about 1 year of life, and EEGs had tendency to be of low voltage after about 2 years of life, and the EEG became flat later.<BR>In globoid cell leukodystrophy, EEGs were almost normal at the early stage, the slow waves and seizure discharges gradually increased with the progress of the illness.

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  • 脳と発達

    脳と発達 7 (3), 213-221, 1975

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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