Electrophysiologic observations in the classical form of Pelizaeus-Merzbacher disease.
In a 20-year-old patient with the classic form of Pelizaeus-Merzbacher disease, electroencephalograms during wakefulness were moderately diffusely abnormal, and an overnight polygraphic sleep recording showed distorted nonrapid eye movement sleep patterns without vertex sharp waves, K-complexes, spindles, or positive occipital sharp transients. Rapid eye movement sleep could be identified. Cerebral responses evoked by light flashes, clicks, and electric stimulation of the median nerves were abnormal.
D000328:Adult; D001307:Auditory Perception / Q000502:physiology; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000503:physiopathology*; D004569:Electroencephalography; D005071:Evoked Potentials; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D009431:Neural Conduction; D012894:Sleep Stages / Q000502:physiology; D014796:Visual Perception / Q000502:physiology