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


CONCLUSIONS
BAEP and VEP testing is helpful in objective evaluation of children with KD.
Aldosari
Mohammed
M
Department of Pediatrics (Neurology), Duke University Medical Center, Durham, NC 27710, USA.
Altuwaijri
Maha
M
Husain
Aatif M
AM
D017668:Age of Onset; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D016057:Evoked Potentials, Auditory, Brain Stem; D005074:Evoked Potentials, Visual; D006801:Humans; D007223:Infant; D007965:Leukodystrophy, Globoid Cell / Q000453:epidemiology / Q000503:physiopathology*; D016013:Likelihood Functions; D012189:Retrospective Studies; D012720:Severity of Illness Index