17 patients suffering from the presenile dementia characterised by polycystic lipomenbranous osteodysplasia and sclerosing leukoencephalopathy were studied neurophysiologically. Noteworthy findings were diffuse slowing, the accentuation of 6-8 Hz parasagittal activity, slowing of the dominant occipital rhythm and, in the late phase when 13 of patients got epileptic seizures, there was paroxysmal activity in the EEG.
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