Central motor conduction time by magnetic stimulation of the cortex and peripheral nerve conduction follow-up studies in Friedreich's ataxia.
A follow-up clinical study, peripheral motor and sensory nerve conduction velocities and central motor conduction by magnetic stimulation of the cortex were performed in 13 patients with classical Friedreich's ataxia (FA) phenotype, for a period of 9-12 years. Clinical worsening was unrelated to peripheral nerve abnormalities. The amplitude of the nerve action potentials and delayed conduction velocity remained unchanged for several years. Central motor conduction times were abnormal in all patients. Clinical conditions worsened significantly between successive examinations with significant increments in threshold and significant decrement of the amplitude of motor evoked potentials. The results are consistent with progressive pyramidal and cerebellar pathways involvement as the cause of clinical worsening in FA.
Cruz-Martínez
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A
Unidad de Electromiografïa, Hospital La Luz, Madrid, Spain.
Palau
F
F
D000293:Adolescent; D000328:Adult; D002531:Cerebellum / Q000503:physiopathology; D002648:Child; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D005500:Follow-Up Studies; D005621:Friedreich Ataxia / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D008280:Magnetics; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009044:Motor Cortex / Q000503:physiopathology*; D009046:Motor Neurons; D018482:Muscle, Skeletal / Q000503:physiopathology; D009431:Neural Conduction; D009475:Neurons, Afferent; D010525:Peripheral Nerves / Q000503:physiopathology*; D010812:Physical Stimulation; D017966:Pyramidal Cells / Q000503:physiopathology; D011930:Reaction Time; D013497:Sural Nerve / Q000503:physiopathology; D018911:Trinucleotide Repeats