Negative electroretinograms in the pediatric and adult population

Abstract

Objective To assess the frequency of negative waveform electroretinograms (ERGs) in a tertiary referral center. Design Retrospective chart review. Participants All patients who had an ERG performed at the electrophysiology clinic at Emory University from January 1999 through March 2008 were included in the study. Methods Patients with b-wave amplitude ≤ a-wave amplitude during the dark-adapted bright flash recording, in at least one eye, were identified as having a "negative ERG". Clinical information, such as age, gender, symptoms, best corrected visual acuity, and diagnoses were recorded for these patients when available. Results A total of 1,837 patients underwent ERG testing during the study period. Of those, 73 patients had a negative ERG, for a frequency of 4.0%. Within the adult (≥ 18 years of age) and pediatric populations, the frequencies of a negative ERG were 2.5 and 7.2%, respectively. Among the 73 cases, negative ERGs were more common among male than female patients, 6.7% versus 1.8% (P < 0.0001). Negative ERGs were most common among male children and least common among female adults, 9.6% versus 1.1%, respectively, (P < 0.0001). Overall in this group of patients, the most common diagnoses associated with a negative ERG were congenital stationary night blindness (CSNB, n = 29) and X-linked retinoschisis (XLRS, n = 7). Conclusions The overall frequency of negative ERGs in this large retrospective review was 4.0%. Negative ERGs were most common among male children and least common among female adults. Despite the growing number of new diagnoses associated with negative ERGs, CSNB, and XLRS appear to be the most likely diagnoses for a pediatric patient who presents with a negative ERG.

Electronegative electroretinograms in the United Arab Emirates

“…Our findings are consistent with results from the four previous studies from different areas of the world that assessed the incidence and causes of negative ERGs. [ 6 7 8 9 ] Koh et al . [ 6 ](the United Kingdom) reported a 5.0% incidence, the most common diagnoses being photoreceptor dystrophy (34/128), XLRS (19/128), and CSNB (17/128).…”

“…We are aware of four prior studies that have assessed the incidence and causes of electronegative ERGs in the United Kingdom, the United States, Germany, and Brazil with a reported incidence of approximately 5.0%, 4.0%, 3.0%, and 2.50%, respectively. [ 6 7 8 9 ] The incidence and causes of electronegative ERGs in the Arabian Gulf, where recessive diseases can be more common,[ 10 ] has not been studied to the best of our knowledge. The purpose of this study is to report the incidence and causes for electronegative ERGs in a referral center in the United Arab Emirates.…”

Sorting out Co-occurrence of Rare Monogenic Retinopathies: Stargardt Disease Co-existing with Congenital Stationary Night Blindness

“…CSNB and X-linked retinoschisis are the more common causes of electronegative ERGs [30][31][32] and are typically diagnosed in childhood. In an adult patient such as ours, the differential for an electronegative ERG includes acquired causes such as MAR, CAR, Birdshot chorioretinopathy, central retinal vein occlusion, and drug toxicity (e.g.…”

Constructing the rod bipolar cell signalplex using animal models of retinal dysfunction.

“…The absence of the b-wave in the ERG has been associated with the non-progressive disease congenital stationary night blindness (CSNB). It has been reported that between 2.9 and 4.8% of the human population has a negative ERG and the underlying cause of the vast majority of negative ERGs is CSNB (Koh et al, 2001;Renner et al, 2006;Kim et al, 2012).…”