Corticomotoneuronal latencies after non-invasive stimulation of the motor cortex in adrenomyeloneuropathy and adrenoleukodystrophy: results in patients and their relatives.
Corticomotoneuronal latencies after non-invasive stimulation of the motor cortex were studied in two patients with adrenomyeloneuropathy (AMN), in one patient with adrenoleukodystrophy (ALD), in two obligate heterozygotes, and in three clinically not affected sisters of the patients with AMN. Corticomotoneuronal latencies were prolonged in the patients as well as in obligate female carriers, but normal in the clinically and biochemically not affected relatives. The noninvasive cortex stimulation was found to be a sensitive method to detect clinical and subclinical deficits in patients and heterozygous carriers.
Department of Neurology, University of Münster, FRG.
H
H
Masur
S
S
Zierz
A C
AC
Ludolph
C E
CE
Elger
D G
DG
Palm
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000235:genetics / Q000503:physiopathology*; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology; D002648:Child; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000503:physiopathology*; D005072:Evoked Potentials, Auditory / Q000502:physiology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006580:Genetic Carrier Screening; D006801:Humans; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000503:physiopathology*; D009046:Motor Neurons / Q000502:physiology*; D009132:Muscles / Q000294:innervation; D011930:Reaction Time / Q000502:physiology*; D013979:Tibial Nerve / Q000503:physiopathology