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Electronegative electroretinogram

MedGen UID:
867203
Concept ID:
C4021561
Finding
Synonyms: Electronegative ERG; Electroretinogram: reduced b-wave amplitude; Reduced amplitude of dark-adapted bright flash electroretinogram b-wave; Reduced electroretinogram rod b-wave; Reduced ERG amplitude of b-wave
 
HPO: HP:0007984

Definition

A dark-adapted bright flash electroretinogram in which the b-wave that is of markedly lower amplitude than the associated a-wave (source [from HPO]

Conditions with this feature

Congenital stationary night blindness 2A
MedGen UID:
376299
Concept ID:
C1848172
Disease or Syndrome
X-linked congenital stationary night blindness (CSNB) is characterized by non-progressive retinal findings of reduced visual acuity ranging from 20/30 to 20/200; defective dark adaptation; refractive error, most typically myopia ranging from low (-0.25 diopters [D] to -4.75 D) to high (=-10.00 D) but occasionally hyperopia; nystagmus; strabismus; normal color vision; and normal fundus examination. Characteristic ERG findings can help distinguish between complete X-linked CSNB and incomplete X-linked CSNB.
Cone-rod dystrophy 3
MedGen UID:
349030
Concept ID:
C1858806
Disease or Syndrome
Cone-rod dystrophy-3 (CORD3) is an autosomal recessive, clinically heterogeneous retinal disorder with typical findings of reduced visual acuity, impairment of the central visual field, color vision deficits, and fundoscopic evidence of maculopathy, with no or few midperipheral retinal pigment deposits. Cone degeneration appears early in life with a central involvement of the retina, followed by a degeneration of rods several years later (summary by Klevering et al., 2002 and Ducroq et al., 2002). Both cone and rod a- and b-wave electroretinogram (ERG) amplitudes are reduced (Fishman et al., 2003). For a general phenotypic description and a discussion of genetic heterogeneity of cone-rod dystrophy, see 120970.
Retinal cone dystrophy 4
MedGen UID:
355308
Concept ID:
C1864849
Disease or Syndrome
Any cone dystrophy in which the cause of the disease is a mutation in the CACNA2D4 gene.
Congenital stationary night blindness 1F
MedGen UID:
767313
Concept ID:
C3554399
Disease or Syndrome
Autosomal recessive congenital stationary night blindness is a disorder of the retina, which is the specialized tissue at the back of the eye that detects light and color. People with this condition typically have difficulty seeing and distinguishing objects in low light (night blindness). For example, they may not be able to identify road signs at night or see stars in the night sky. They also often have other vision problems, including loss of sharpness (reduced acuity), nearsightedness (myopia), involuntary movements of the eyes (nystagmus), and eyes that do not look in the same direction (strabismus).\n\nThe vision problems associated with this condition are congenital, which means they are present from birth. They tend to remain stable (stationary) over time.
Juvenile retinoschisis
MedGen UID:
811458
Concept ID:
C3714753
Disease or Syndrome
X-linked congenital retinoschisis (XLRS) is characterized by symmetric bilateral macular involvement with onset in the first decade of life, in some cases as early as age three months. Fundus examination shows areas of schisis (splitting of the nerve fiber layer of the retina) in the macula, sometimes giving the impression of a spoke wheel pattern. Schisis of the peripheral retina, predominantly inferotemporally, occurs in approximately 50% of individuals. Affected males typically have 20/60 to 20/120 vision. Visual acuity often deteriorates during the first and second decades of life but then remains relatively stable until the fifth or sixth decade.
Cone-rod synaptic disorder, congenital nonprogressive
MedGen UID:
874422
Concept ID:
C4041558
Disease or Syndrome
Congenital nonprogressive cone-rod synaptic disorder (CRSD) is characterized by stable low vision, nystagmus, photophobia, a normal or near-normal fundus appearance, and no night blindness. Electroretinography shows an electronegative waveform response to scotopic bright flash, near-normal to subnormal rod function, and delayed and/or decreased to nonrecordable cone responses (Traboulsi, 2013; Khan, 2014).

Professional guidelines

PubMed

Nishiguchi KM, Kunikata H, Fujita K, Hashimoto K, Koyanagi Y, Akiyama M, Ikeda Y, Momozawa Y, Sonoda KH, Murakami A, Wada Y, Nakazawa T
Clin Exp Ophthalmol 2020 Jul;48(5):644-657. Epub 2020 Mar 17 doi: 10.1111/ceo.13743. PMID: 32112665

Recent clinical studies

Etiology

Hagedorn JL, Dunn TM, Bhattarai S, Stephan C, Mathews KD, Pfeifer W, Drack AV
Doc Ophthalmol 2023 Feb;146(1):7-16. Epub 2022 Nov 18 doi: 10.1007/s10633-022-09909-4. PMID: 36399172Free PMC Article
Alsalamah AK, Khan AO
Middle East Afr J Ophthalmol 2020 Apr-Jun;27(2):86-90. Epub 2020 Jul 20 doi: 10.4103/meajo.MEAJO_106_20. PMID: 32874040Free PMC Article
Nishiguchi KM, Kunikata H, Fujita K, Hashimoto K, Koyanagi Y, Akiyama M, Ikeda Y, Momozawa Y, Sonoda KH, Murakami A, Wada Y, Nakazawa T
Clin Exp Ophthalmol 2020 Jul;48(5):644-657. Epub 2020 Mar 17 doi: 10.1111/ceo.13743. PMID: 32112665
Ba-Abbad R, Robson AG, Yap YC, Moore AT, Webster AR, Holder GE
Retina 2014 Jun;34(6):1235-43. doi: 10.1097/IAE.0000000000000052. PMID: 24608669
Pradhan SM, Atchaneeyasakul LO, Appukuttan B, Mixon RN, McFarland TJ, Billingslea AM, Wilson DJ, Stout JT, Weleber RG
Arch Ophthalmol 2002 Jan;120(1):45-50. doi: 10.1001/archopht.120.1.45. PMID: 11786056

Diagnosis

Wang H, Ruan G, Yang S, Li H, Sun Z, Tian B, Yan P, Li Y, Yang H, Zhong Y, Qian J
Am J Med Genet A 2023 Dec;191(12):2819-2824. Epub 2023 Aug 2 doi: 10.1002/ajmg.a.63361. PMID: 37530213
Hagedorn JL, Dunn TM, Bhattarai S, Stephan C, Mathews KD, Pfeifer W, Drack AV
Doc Ophthalmol 2023 Feb;146(1):7-16. Epub 2022 Nov 18 doi: 10.1007/s10633-022-09909-4. PMID: 36399172Free PMC Article
Sakti DH, Ali H, Korsakova M, Saakova N, Mustafic N, Fraser CL, Jamieson RV, Cornish EE, Grigg JR
Clin Exp Ophthalmol 2022 May;50(4):429-440. Epub 2022 Mar 8 doi: 10.1111/ceo.14065. PMID: 35212129Free PMC Article
Alsalamah AK, Khan AO
Middle East Afr J Ophthalmol 2020 Apr-Jun;27(2):86-90. Epub 2020 Jul 20 doi: 10.4103/meajo.MEAJO_106_20. PMID: 32874040Free PMC Article
Schallhorn CS, Granet DB, Ferreyra HA
Retin Cases Brief Rep 2018 Spring;12(2):143-148. doi: 10.1097/ICB.0000000000000451. PMID: 27820752

Therapy

Tsui I, Casper D, Chou CL, Tsang SH
Doc Ophthalmol 2008 Jan;116(1):57-60. Epub 2007 Oct 3 doi: 10.1007/s10633-007-9084-7. PMID: 17912565Free PMC Article

Prognosis

Hung WC, Cheng HC, Wang AG
Doc Ophthalmol 2022 Dec;145(3):263-270. Epub 2022 Sep 29 doi: 10.1007/s10633-022-09901-y. PMID: 36173494
Alsalamah AK, Khan AO
Middle East Afr J Ophthalmol 2020 Apr-Jun;27(2):86-90. Epub 2020 Jul 20 doi: 10.4103/meajo.MEAJO_106_20. PMID: 32874040Free PMC Article
Yang P, Chiang PW, Weleber RG, Pennesi ME
JAMA Ophthalmol 2015 Jun;133(6):653-61. doi: 10.1001/jamaophthalmol.2015.0357. PMID: 25789692Free PMC Article
Pradhan SM, Atchaneeyasakul LO, Appukuttan B, Mixon RN, McFarland TJ, Billingslea AM, Wilson DJ, Stout JT, Weleber RG
Arch Ophthalmol 2002 Jan;120(1):45-50. doi: 10.1001/archopht.120.1.45. PMID: 11786056

Clinical prediction guides

Smirnov VM, Zeitz C, Soumittra N, Audo I, Defoort-Dhellemmes S
Doc Ophthalmol 2018 Apr;136(2):135-143. Epub 2018 Mar 10 doi: 10.1007/s10633-018-9629-y. PMID: 29525873
Yang P, Chiang PW, Weleber RG, Pennesi ME
JAMA Ophthalmol 2015 Jun;133(6):653-61. doi: 10.1001/jamaophthalmol.2015.0357. PMID: 25789692Free PMC Article
Ba-Abbad R, Robson AG, Yap YC, Moore AT, Webster AR, Holder GE
Retina 2014 Jun;34(6):1235-43. doi: 10.1097/IAE.0000000000000052. PMID: 24608669

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