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

Publication ,  Journal Article
Aldosari, M; Altuwaijri, M; Husain, AM
Published in: Clin Neurophysiol
July 2004

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.

Duke Scholars

Published In

Clin Neurophysiol

DOI

ISSN

1388-2457

Publication Date

July 2004

Volume

115

Issue

7

Start / End Page

1653 / 1656

Location

Netherlands

Related Subject Headings

  • Severity of Illness Index
  • Retrospective Studies
  • Neurology & Neurosurgery
  • Likelihood Functions
  • Leukodystrophy, Globoid Cell
  • Infant
  • Humans
  • Evoked Potentials, Visual
  • Evoked Potentials, Auditory, Brain Stem
  • Disease Progression
 

Citation

APA
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ICMJE
MLA
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Aldosari, M., Altuwaijri, M., & Husain, A. M. (2004). Brain-stem auditory and visual evoked potentials in children with Krabbe disease. Clin Neurophysiol, 115(7), 1653–1656. https://doi.org/10.1016/j.clinph.2004.02.022
Aldosari, Mohammed, Maha Altuwaijri, and Aatif M. Husain. “Brain-stem auditory and visual evoked potentials in children with Krabbe disease.Clin Neurophysiol 115, no. 7 (July 2004): 1653–56. https://doi.org/10.1016/j.clinph.2004.02.022.
Aldosari M, Altuwaijri M, Husain AM. Brain-stem auditory and visual evoked potentials in children with Krabbe disease. Clin Neurophysiol. 2004 Jul;115(7):1653–6.
Aldosari, Mohammed, et al. “Brain-stem auditory and visual evoked potentials in children with Krabbe disease.Clin Neurophysiol, vol. 115, no. 7, July 2004, pp. 1653–56. Pubmed, doi:10.1016/j.clinph.2004.02.022.
Aldosari M, Altuwaijri M, Husain AM. Brain-stem auditory and visual evoked potentials in children with Krabbe disease. Clin Neurophysiol. 2004 Jul;115(7):1653–1656.
Journal cover image

Published In

Clin Neurophysiol

DOI

ISSN

1388-2457

Publication Date

July 2004

Volume

115

Issue

7

Start / End Page

1653 / 1656

Location

Netherlands

Related Subject Headings

  • Severity of Illness Index
  • Retrospective Studies
  • Neurology & Neurosurgery
  • Likelihood Functions
  • Leukodystrophy, Globoid Cell
  • Infant
  • Humans
  • Evoked Potentials, Visual
  • Evoked Potentials, Auditory, Brain Stem
  • Disease Progression