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Documenta Ophthalmologica

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01.07.2008 | Original Research Article

Photopic ON- and OFF-responses in complete type of congenital stationary night blindness in relation to stimulus intensity

verfasst von: Maja Sustar, Branka Stirn-Kranjc, Marko Hawlina, Jelka Brecelj

Erschienen in: Documenta Ophthalmologica | Ausgabe 1/2008

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Abstract

The complete type of congenital stationary night blindness (complete CSNB) is a retinal disorder where the ON-response b-wave of the photopic electroretinogram is reduced, while the ON-response a-wave and OFF-response d-wave are preserved. These findings are related to ON-bipolar cell dysfunction, whereas the effects of myopia, which is usually associated with complete CSNB, have not been studied yet. The aim was to investigate ON-bipolar cell dysfunction in complete CSNB with variable-intensity stimuli, while excluding effects of myopia. ON- and OFF-responses were recorded from six complete CSNB patients, 10 control subjects (no refractive error; emmetropic control group) and 14 myopic subjects with similar refractive error and axial length as those with complete CSNB (myopic control group). Responses were elicited with stimulus intensities from 0.4 to 2.1 log cd s/m² on a 20 cd/m² background. Stimulus/response (S/R) functions were fitted to the Naka–Rushton equation and statistically evaluated. In complete CSNB, the S/R function of the a-wave measured at the time corresponding to the controls showed a lower V _max than for the emmetropic controls, while all parameters were similar to the myopic control group. The b- and d-wave S/R functions of the complete CSNB group were similarly different to both control groups—for the b-wave, the V _max and n were significantly lower, and for the d-wave, the σ was significantly higher. These findings suggest that in complete CSNB, dysfunction of ON-bipolar cells influences the dynamics of the ON-response b-wave and OFF-response d-wave, independently of the effects of myopia.

Miyake Y, Yagasaki K, Horiguchi M, Kawase Y, Kanda T (1986) Congenital stationary night blindness with negative electroretinogram. A new classification. Arch Ophthalmol 104(7):1013–1020PubMed

Miyake Y (2002) Establishment of the concept of new clinical entities—complete and incomplete form of congenital stationary night blindness. Nippon Ganka Gakkai Zasshi 106(12):737–755PubMed

Bech-Hansen NT, Naylor MJ, Maybaum TA, Sparkes RL, Koop B, Birch DG, Bergen AA, Prinsen CF, Polomeno RC, Gal A, Drack AV, Musarella MA, Jacobson SG, Young RS, Weleber RG (2000) Mutations in NYX encoding the leucine-rich proteoglycan nyctalopin cause X-linked complete congenital stationary night blindness. Nat Genet 26(3):319–323PubMedCrossRef

Pusch CM, Zeitz C, Brandau O, Pesch K, Achatz H, Feil S, Scharfe C, Maurer J, Jacobi FK, Pinckers A, Andreasson S, Hardcastle A, Wissinger B, Berger W, Meindl A (2000) The complete form of X-linked congenital stationary night blindness is caused by mutations in a gene encoding a leucine-rich repeat protein. Nat Genet 26(3):324–327PubMedCrossRef

Dryja TP, McGee TL, Berson EL, Fishman GA, Sandberg MA, Alexander KR, Derlacki DJ, Rajagopalan AS (2005) Night blindness and abnormal cone electroretinogram ON responses in patients with mutations in the GRM6 gene encoding mGluR6. Proc Nat Acad Sci USA 102(13):4884–4889PubMedCrossRef

Houchin KW, Purple RL, Wirtschafter JD (1991) X-linked congenital stationary night blindness and depolarizing bipolar system function. Invest Ophthalmol Vis Sci 32(suppl):1229

Young RSL (1991) Low-frequency component of the photopic ERG in patients with x-linked congenital stationary night blindness. Clin Vision Sci 6(4):309–315

Quigley M, Roy MS, Barsoum-Homsy M, Chevrette L, Jacob JL, Milot J (1996) On- and off-responses in the photopic electroretinogram in complete-type congenital stationary night blindness. Doc Ophthalmol 92(3):159–165PubMedCrossRef

Kim SH, Bush RA, Sieving PA (1997) Increased phase lag of the fundamental harmonic component of the 30 Hz flicker ERG in Schubert–Bornschein complete type CSNB. Vision Res 37(17):2471–2475PubMedCrossRef

10.

Cibis GW, Fitzgerald KM (2001) The negative ERG is not synonymous with nightblindness. Trans Am Ophthalmol Soc 99:171–176PubMed

11.

Holder GE (2001) Pattern electroretinography (PERG) and an integrated approach to visual pathway diagnosis. Prog Retin Eye Res 20(4):531–561PubMedCrossRef

12.

Langrova H, Gamer D, Friedburg C, Besch D, Zrenner E, Apfelstedt-Sylla E (2002) Abnormalities of the long flash ERG in congenital stationary night blindness of the Schubert–Bornschein type. Vision Res 42(11):1475–1483PubMedCrossRef

13.

Allen LE, Zito I, Bradshaw K, Patel RJ, Bird AC, Fitzke F, Yates JR, Trump D, Hardcastle AJ, Moore AT (2003) Genotype-phenotype correlation in British families with X linked congenital stationary night blindness. Br J Ophthalmol 87(11):1413–1420PubMedCrossRef

14.

Khan NW, Kondo M, Hiriyanna KT, Jamison JA, Bush RA, Sieving PA (2005) Primate retinal signaling pathways: suppressing ON-pathway activity in monkey with glutamate analogues mimics human CSNB1-NYX genetic night blindness. J Neurophysiol 93(1):481–492PubMedCrossRef

15.

Miyake Y, Yagasaki K, Horiguchi M, Kawase Y (1987) On- and off responses in photopic electroretinogram in complete and incomplete types of congenital stationary night blindness. Jpn J Ophthalmol 31:81–87PubMed

16.

Alexander KR, Fishman GA, Peachey NS, Marchese AL, Tso MOM (1992) ‘On’ response defect in paraneoplastic night blindness with cutaneous malignant melanoma. Invest Ophthalmol Vis Sci 33:477–483PubMed

17.

Sieving PA (1993) Photopic ON- and OFF-pathway abnormalities in retinal dystrophies. Trans Am Ophthalmol Soc 91:701–773PubMed

18.

Bush RA, Sieving PA (1994) A proximal retinal component in the primate photopic ERG a-wave. Invest Ophthalmol Vis Sci 35:635–645PubMed

19.

Ueno S, Kondo M, Ueno M, Miyata K, Terasaki H, Miyake Y (2006) Contribution of retinal neurons to d-wave of primate photopic electroretinograms. Vision Res 46(5):658–664PubMedCrossRef

20.

Knapp AG, Schiller PH (1984) The contribution of on-bipolar cells to the electroretinogram of rabbits and monkeys. A study using 2-amino-4-phosphonobutyrate (APB). Vision Res 24(12):1841–1846PubMedCrossRef

21.

Sieving PA, Murayama K, Naarendorp F (1994) Push-pull model of the primate photopic electroretinogram: a role for hyperpolarizing neurons in shaping the b-wave. Vis Neurosci 11(3):519–532PubMedCrossRef

22.

Evers HU, Gouras P (1986) Three cone mechanisms in the primate electroretinogram: two with one without off-center bipolar responses. Vision Res 26(2):245–254PubMedCrossRef

23.

Perlman I, Meyer E, Haim T, Zonis S (1984) Retinal function in high refractive error assessed electroretinographically. Br J Ophthalmol 68(2):79–84PubMedCrossRef

24.

Yamamoto S, Nitta K, Kamiyama M (1997) Cone electroretinogram to chromatic stimuli in myopic eyes. Vision Res 37(15):2157–2159PubMedCrossRef

25.

Westall CA, Dhaliwal HS, Panton CM, Sigesmun D, Levin AV, Nischal KK, Heon E (2001) Values of electroretinogram responses according to axial length. Doc Ophthalmol 102(2):115–130PubMedCrossRef

26.

Marmor MF, Holder GE, Seeliger MW, Yamamoto S, International Society for Clinical Electrophysiology of Vision (2004) Standard for clinical electroretinography (2004 update). Doc Ophthalmol 108(2):107–114

27.

Sustar M, Hawlina M, Brecelj J (2006) ON- and OFF-response of the photopic electroretinogram in relation to stimulus characteristics. Doc Ophthalmol 113(1):43–52PubMedCrossRef

28.

Hawlina M, Konec B (1992) New noncorneal HK-loop electrode for clinical electroretinography. Doc Ophthalmol 81(2):253–259PubMedCrossRef

29.

Chen JF, Elsner AE, Burns SA, Hansen RM, Lou PL, Kwong KK, Fulton AB (1992) The effect of eye shape on retinal responses. Clin Vision Sci 7(6):520–530

30.

Roecker EB, Pulos E, Bresnick GH, Severns M (1992) Characterization of the electroretinographic scotopic b-wave amplitude in diabetic and normal subjects. Invest Ophthalmol Vis Sci 33(5):1575–1583PubMed

31.

Anastasi M, Brai M, Lauricella M, Geracitano R (1993) Methodological aspects of the application of the Naka–Rushton eqation to clinical electroretinogram. Ophthalmic Res 25:145–156PubMedCrossRef

32.

Hood DC, Shady S, Birch DG (1994) Understanding changes in the b-wave of the ERG caused by heterogeneous receptor damage. Invest Ophthalmol Vis Sci 35(5):2477–2488PubMed

33.

Velten IM, Horn FK, Korth M, Velten K (2001) The b-wave of the dark adapted flash electroretinogram in patients with advanced asymmetrical glaucoma and normal subjects. Br J Ophthalmol 85:403–409PubMedCrossRef

34.

Hamilton R, Chaplin C, McCulloch D (2006) Maturation of the photopic hill in human infants. Doc Ophthalmol 112(2):84–85

35.

Kondo M, Piao CH, Tanikawa A, Horiguchi M, Terasaki H, Miyake Y (2000) Amplitude decrease of photopic ERG b-wave at higher stimulus intensities in humans. Jpn J Ophthalmol 44(1):20–28PubMedCrossRef

36.

Kawasaki K, Tsuchida Y, Jacobson JH (1971) Positive and negative deflections in the off response of the electroretinogram in man. Am J Ophthalmol 72(2):367–375PubMed

37.

Kawabata H, Adachi-Usami E (1997) Multifocal electroretinogram in myopia. Invest Ophthalmol Vis Sci 38(13):2844–2851PubMed

38.

Luu CD, Lau AM, Lee SY (2006) Multifocal electroretinogram in adults and children with myopia. Arch ophthalmol 124(3):328–334PubMedCrossRef

39.

Pallin E (1969) The influence of the axial size of the eye on the size of the recorded b-potential in the clinical single-flash electroretinogram. Acta Ophthalmol 191(suppl):1–57

40.

Kondo M, Miyake Y, Kondo N, Tanikawa A, Suzuki S, Horiguchi M, Terasaki H (2001) Multifocal ERG findings in complete type congenital stationary night blindness. Invest Ophthalmol Vis Sci 42(6):1342–1348PubMed

41.

Kondo M, Sieving PA (2002) Post-photoreceptoral activity dominates primate photopic 32-Hz ERG for sine-square- and pulsed stimuli. Invest Ophthalmol Vis Sci 43(7):2500–2507PubMed

42.

Sustar M, Stirn-Kranjc B, Hawlina M, Brecelj J (2006) Stimulus/response relation in normal ON- and OFF-response and in complete CSNB. Doc Ophthalmol 112(2):97

Titel: Photopic ON- and OFF-responses in complete type of congenital stationary night blindness in relation to stimulus intensity
verfasst von: Maja Sustar
Branka Stirn-Kranjc
Marko Hawlina
Jelka Brecelj
Publikationsdatum: 01.07.2008
Verlag: Springer-Verlag
Erschienen in: Documenta Ophthalmologica / Ausgabe 1/2008
Print ISSN: 0012-4486
Elektronische ISSN: 1573-2622
DOI: https://doi.org/10.1007/s10633-007-9101-x

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Photopic ON- and OFF-responses in complete type of congenital stationary night blindness in relation to stimulus intensity

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