Author/Editor		Brecelj, Jelka; Stirn-Kranjc, Branka
Title		ERG and VEP follow-up study in children with Leber's congenital amaurosis
Type		članek
Source		Eye
Vol. and No.		Letnik 13
Publication year		1999
Volume		str. 47-54
Language		eng
Abstract		Purpose: Our prospective clinical and eletrophysilogical 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. Conclusion: 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 followup purposes.
Descriptors		OPTIC ATROPHY BLINDNESS NYSTAGMUS HYPEROPIA VISUAL PATHWAYS RETINAL DISEASES HEREDITARY DISEASES ELECTRORETINOGRAPHY ENOPHTHALMOS ESOTROPIA EXOTROPIA ELECTROPHYSIOLOGY RETINAL VESSELS