Brain-stem auditory and visual evoked potentials in children with Krabbe disease.

Journal Article (Journal Article)

OBJECTIVE: There are few reports in the literature detailing brain-stem auditory and visual evoked potentials (BAEP and VEP) in children with Krabbe disease (KD). The purpose of this study was to provide a descriptive analysis of the BAEP and VEP findings in these children. METHODS: Charts of children with KD were reviewed. BAEP and VEP studies performed on these children were reviewed; findings in the early infantile and late onset KD (EIKD, LOKD, respectively) groups were also examined. Likelihood ratios (LR) for the neurophysiologic tests being abnormal in the various groups were also determined. RESULTS: BAEP abnormalities were seen in 15/17 (88%) children with EIKD and 2/5 (40%) children with LOKD, LR statistically significant. In the EIKD group, all 13 symptomatic children had an abnormal BAEP, whereas 2/4 (50%) of the pre-symptomatic children had similar findings, LR statistically significant. VEP abnormalities were noted in 8/15 (53%) children with EIKD and none of the children with LOKD, LR statistically significant. In the EIKD group, the 8/12 (67%) symptomatic children had an abnormal VEP, whereas none of the pre-symptomatic children did, LR statistically significant. Subgroup analyses of the LOKD subgroups did not yield significant findings. CONCLUSIONS: BAEP abnormalities are among the first objective indications of central nervous system disease in children with EIKD. VEP abnormalities occur later in the course of the illness. Both tests are less helpful in children with LOKD. SIGNIFICANCE: BAEP and VEP testing is helpful in objective evaluation of children with KD.

Full Text

Duke Authors

Cited Authors

  • Aldosari, M; Altuwaijri, M; Husain, AM

Published Date

  • July 1, 2004

Published In

Volume / Issue

  • 115 / 7

Start / End Page

  • 1653 - 1656

PubMed ID

  • 15203066

International Standard Serial Number (ISSN)

  • 1388-2457

Digital Object Identifier (DOI)

  • 10.1016/j.clinph.2004.02.022


  • eng

Conference Location

  • Netherlands