Visual electrophysiology in congenital nystagmus with normal fundus

Surachatkumtonekul, T.; Ruangvaravate, N.; Sriyakul, C.

Journal of the Medical Association of Thailand 92(2): 224-228

2009


ISSN/ISBN: 0125-2208
PMID: 19253798
Document Number: 16046
To identify the ophthalmic causes of congenital nystagmus with normal eye examination by visual electrophysiologic tests. Medical records of patients who presented with nystagmus and no other neurological sign before 6 months of age with normal eye examination, between February 2004 and February 2005, were reviewed Complete ophthalmic examination and visual electrophysiologic tests were performed to identify the ophthalmic causes of congenital nystagmus. Thirty-four patients had met the criteria. Leber's congenital amaurosis was diagnosed in 13 patients (38.2%) by non-recordable electroretinography (ERG) and normal visual evoked potential (VEP). Idiopathic infantile nystagmus was diagnosed in 12 patients (35.3%) by normal ERG and VEP. Achromatopsia was diagnosed in 8 patients (23.5%) by flat retinal cone ERG but normal rod ERG and VEP. Rod-cone dystrophy was diagnosed in one patient (3%) by abnormal rod and cone ERG CONCLUSION: Visual electrophysiologic study is helpful to identify the ophthalmic causes of congenital nystagmus. Leber's congenital amaurosis is a common cause in congenital nystagmus.

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Visual electrophysiology in congenital nystagmus with normal fundus