Short latency somatosensory evoked potentials in Charcot-Marie-Tooth disease. A family with an intermediate form.
9 patients with Charcot-Marie-Tooth Disease (CMTD) of intermediate type (PMA type II, 10), all from the same family, presented with a significant increase of the interpeak N9-N13 latency. This increase is already present in the pre-symptomatic phase of the disease and there is no significant difference between the various patients of different ages and clinical severity, indicating that the lesions appears very early and tends to establish itself equally early. Similar behaviour is also seen in the distal conduction velocity along the sensitive fibres, while the more proximal areas seem to be relatively spared. The authors interpret these data as an expression of a distal central peripheral sensory neuropathy. In contrast, the lesion of the peripheral motor fibres, particularly in the legs, has a different and more severe pattern of evolution. Alterations in central conduction time (N13-N20) were not seen in any of the 9 patients studied.
Rossi
A
A
Paradiso
C
C
Dell'Anna
P
P
Mondelli
M
M
D000293:Adolescent; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology; D002540:Cerebral Cortex / Q000503:physiopathology; D002607:Charcot-Marie-Tooth Disease / Q000235:genetics* / Q000503:physiopathology; D002648:Child; D004558:Electric Stimulation; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009133:Muscular Atrophy / Q000235:genetics*; D010543:Peroneal Nerve / Q000503:physiopathology; D011930:Reaction Time / Q000502:physiology; D012677:Sensation / Q000502:physiology; D013116:Spinal Cord / Q000503:physiopathology; D013497:Sural Nerve / Q000503:physiopathology; D009435:Synaptic Transmission