Electroencephalographic (EEG) findings of twocases with Tay-Sachs' disease (TSD) wereexamined during the course of the illness.
In the first case, EEG findings were characterized by low amplitude fast activity without paroxysmal discharges at 8 months of life. At 10months of age, slow waves which were superimposed by fast activity appeared, and at 16 months, high voltage slow waves (HVS) with multipleseizure discharges were observed.
In the second case, HVS with seizure discharges were found at the age of 17 months, andthose findings became not obvious at the age of 3years, and EEG became almost flat by 3 yearsand 4 months of age.
Frequency analyses of basic waves in both thecases, showed delta-dominancy after Morrell'sstage II.
Mean visual evoked potentials showed alreadyno responses to photic stimulations at the age of15 months for the first case, and 17 months for thesecond case.
These results revealed that at Morrell's stage I-II, EEG findings were low amplitude fastwaves and from the end of stage II, slow wavesand seizure discharges appeared, and that HVSwith multiple seizure discharges were found during stage III, and that amplitude of EEG decreased during stage IV, and finally. EEG becamefla.
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