Pathophysiological aspects of central and peripheral myelin lesions.
The results of nerve conduction velocities, evoked potentials and EEGs in adrenoleukodystrophy, metachromatic leukodystrophy and Pelizaeus Merzbacher's disease are discussed. As shown on the example of four own cases with metachromatic leukodystrophy, the slowing of nerve conduction velocities and the latency prolongation of evoked potentials components or loss of evoked potential components is dependent on the type of MLD (infantile and juvenile) and of the duration of the disease. The pathophysiology of the slowed peripheral and central impulse conduction is discussed.
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000503:physiopathology*; D001921:Brain / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000503:physiopathology*; D004569:Electroencephalography; D005071:Evoked Potentials; D005260:Female; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000503:physiopathology*; D008297:Male; D009046:Motor Neurons / Q000502:physiology; D009186:Myelin Sheath / Q000502:physiology; D009413:Nerve Fibers, Myelinated / Q000502:physiology*; D009431:Neural Conduction; D011901:Ranvier's Nodes / Q000502:physiology; D011930:Reaction Time / Q000502:physiology; D011984:Sensory Receptor Cells / Q000503:physiopathology; D009435:Synaptic Transmission