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Multimodal Evoked Potential Studies in Leukodystrophies of Children

Published online by Cambridge University Press:  18 September 2015

L.J. De Meirleir ,
M.J. Taylor  and
W.J. Logan
L.J. De Meirleir
Affiliation:
Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto
M.J. Taylor*
Affiliation:
Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto
W.J. Logan
Affiliation:
Division of Neurology, Hospital for Sick Children, University of Toronto, Toronto
*
Division of Neurology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5J 1X8
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Abstract:

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Evoked potentials were studied in 22 children with leukodystrophy [10 metachromatic leukodystrophy (MLD), 4 Pelizaeus-Merzbacher (PM), 3 Krabbes, 2 adrenoleukodystrophy (ALD), and one each of Alexander's, Canavan's and multiple sulphatase deficiency (MSD) diseases]. The ABRs were abnormal in all patients (except for the younger ALD), but varied with the type of leukodystrophy. The PM and Krabbes patients had abnormal ABRs with a loss of the rostral waves, accompanied in Krabbes with delayed I-III interpeak latencies; in MLD, ALD and MSD prolonged interpeak latencies were found. Three patients who had no clinical signs, but were positively diagnosed as MLD on the basis of absent arylsulphatase A, also had abnormal ABRs. The SEPs were abnormal in all patients. Cortical SEPs were absent in 16 and abnormal in 5 who were in the earlier stages of their disease. Cervical SEPs were within normal limits except for the Krabbes and MLD patients studied, who showed peripheral slowing. The VEPs were normal in only 6 and, unlike the ABRs and SEPs, did not seem to covary with clinical severity across the various leukodystrophies but did correlate with disease progression. Thus, multimodal EPs are useful in the diagnostic differentiation of the leukodystrophies.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 15 , Issue 1 , February 1988 , pp. 26 - 31
Copyright
Copyright © Canadian Neurological Sciences Federation 1988

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