ERG and VEP follow-up study in children with Leber's congenital amaurosis.
OBJECTIVE
Our prospective clinical and electrophysiological study of children suspected of Leber's congenital amaurosis (LCA) was aimed to follow-up the course of their visual dysfunction.


METHODS
Electroretinography (ERG) and visual evoked potentials (VEP) to white flash stimulation were simultaneously recorded in 9 children at least twice.


RESULTS
The first flash ERG and flash VEP recordings were performed when children were 3-17 months old (mean age 7.6 months). Flash ERG was not recordable in 8 children; flash VEP to binocular stimulation could not be detected in 3, was delayed in 2, attenuated in 2, both attenuated and delayed in 1, and without evident abnormality in 1 of the 9 children. On the last examination (mean age 33.8 months) flash VEP activity was recordable in all children, while flash ERG was recordable in 1. Electrophysiological follow-up (mean duration 26.2 months) showed no deterioration of flash VEP in 8 children.


CONCLUSIONS
In children of LCA simultaneous recording of flash ERG and flash VEP in alert children was helpful to indicate the nature of the visual problem for diagnostic and follow-up purposes.
Institute of Clinical Neurophysiology, University Medical Centre, Ljubljana, Slovenia. jelka.brecelj@uikn.mf.uni-lj.si
J
J
Brecelj
B
B
Stirn-Kranjc
D001766:Blindness / Q000151:congenital* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D011930:Reaction Time; D014792:Visual Acuity