Neurophysiological findings in the hereditary presenile dementia characterised by polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy.
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.
D000328:Adult; D001845:Bone Cysts / Q000235:genetics* / Q000503:physiopathology; D001842:Bone and Bones / Q000473:pathology; D003704:Dementia / Q000235:genetics* / Q000503:physiopathology; D004569:Electroencephalography; D004576:Electromyography; D004828:Epilepsies, Partial / Q000503:physiopathology; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D007968:Leukoencephalopathy, Progressive Multifocal / Q000235:genetics* / Q000503:physiopathology; D050356:Lipid Metabolism; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D009778:Occipital Lobe / Q000503:physiopathology; D010525:Peripheral Nerves / Q000503:physiopathology