Abstract
The electrophysiological findings in optic nerve and primary ganglion cell dysfunction are reviewed. The value of the pattern reversal visual-evoked potential (VEP) in the diagnosis of optic nerve disease, and the pattern appearance VEP in the demonstration of the intracranial misrouting associated with albinism, are discussed. The pattern electroretinogram (PERG) is used in the direct assessment of ganglion cell function. The use of PERG or multifocal electroretinography (mfERG), to enable the distinction between VEP delay due to optic nerve disease and that due to macular dysfunction, is described.
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Holder GE . The pattern electroretinogram and an integrated approach to visual pathway diagnosis. Prog Retin Eye Res 2001; 20: 531–561.
Holder GE . The pattern electroretinogram. In: Fishman GA, Birch DG, Holder GE, Brigell MG (eds). Electrophysiologic Testing in Disorders of the Retina, Optic Nerve, and Visual Pathway, 2nd ed. Ophthalmology Monograph 2. The Foundation of the American Academy of Ophthalmology: San Francisco, CA, 2001.
Holder GE, Robson AG, Hogg CR, Kurz-Levin M, Lois N, Bird AC . Pattern ERG: clinical overview, and some observations on associated fundus autofluorescence imaging in inherited maculopathy. Doc Ophthalmol 2003; 106: 17–23.
Bach M, Hawlina M, Holder GE, Marmor MF, Meigen T, Vaegan et al. Standard for pattern electroretinography. Doc Ophthalmol 2000; 101: 11–18.
Holder GE . The significance of abnormal pattern electroretinography in anterior visual pathway dysfunction. Br J Ophthalmol 1987; 71: 166–171.
Viswanathan S, Frishman LJ, Robson JG . The uniform field and pattern ERG in macaques with experimental glaucoma: removal of spiking activity. Invest Ophthalmol Vis Sci 2000; 41: 2797–2810.
Bush RA, Sieving PA . A proximal retinal component in the primate photopic ERG a-wave. Invest Ophthalmol Vis Sci 1994; 35: 635–645.
Shiells RA, Falk G . Contribution of rod, on-bipolar, and horizontal cell light responses to the ERG of dogfish retina. Vis Neurosci 1999; 16: 503–511.
Green DG, Kapousta-Bruneau NV . A dissection of the electroretinogram from the isolated rat retina with microelectrodes and drugs. Vis Neurosci 1999; 16: 727–741.
Lei B, Perlman I . The contributions of voltage- and time-dependent potassium conductances to the electroretinogram in rabbits. Vis Neurosci 1999; 16: 743–754.
Marmor MF, Holder GE, Seeliger M, Yamamoto S . Standard for clinical electroretinography. Doc Ophthalmol 2004, in press.
Sutter EE, Tran D . The field topography of ERG components in man — I. The photopic luminance response. Vision Res 1992; 32: 433–446.
Hood DC . Assessing retinal function with the multifocal technique. Prog Retin Eye Res 2000; 19: 607–646.
Richey ET, Kooi KA, Tourtellotte WW . Visually evoked responses in multiple sclerosis. J Neurol Neurosurg Psychiatry 1971; 34: 275–280.
Halliday AM, McDonald WI, Mushin J . Delayed visual evoked response in optic neuritis. Lancet 1972; i: 982–985.
Halliday AM, McDonald WI, Mushin J . Visual evoked response in diagnosis of multiple sclerosis. BMJ 1973; 4: 661–664.
Holder GE . Multiple sclerosis. In: Heckenlively JR, Arden GB (eds). Principles and Practice of Clinical Electrophysiology of Vision. Mosby Year Book: St Louis, MO, 1991 pp 797–805.
Jones SJ, Brusa A . Neurophysiological evidence for long-term repair of MS lesions: implications for axon protection. J Neurol Sci 2003; 206: 193–198.
Holder GE . The incidence of abnormal pattern electroretinography in optic nerve demyelination. Electroencephalogr clin Neurophysiol 1991; 78: 18–26.
Berninger TA, Heider W . Pattern electroretinograms in optic neuritis during the acute stage and after remission. Graefe's Arch Clin Exp Ophthalmol 1990; 228: 410–414.
Youl BD, Turano G, Miller DH, Towell AD, MacManus DG, Moore SG et al. The pathophysiology of acute optic neuritis. An association of gadolinium leakage with clinical and electrophysiological deficits. Brain 1991; 114: 2437–2450.
Hickman SJ, Brierley CM, Brex PA, MacManus DG, Scolding NJ, Compston DA et al. Continuing optic nerve atrophy following optic neuritis: a serial MRI study. Mult Scler 2002; 8: 339–342.
Hood DC, Odel JG, Zhang X . Tracking the recovery of local optic nerve function after optic neuritis: a multifocal VEP study. Invest Ophthalmol Vis Sci 2000; 41: 4032–4038.
Rinalduzzi S, Brusa A, Jones SJ . Variation of visual evoked potential delay to stimulation of central, nasal, and temporal regions of the macula in optic neuritis. J Neurol Neurosurg Psychiatry 2001; 70: 28–35.
Wilson WB . Visual evoked response differentiation of ischaemic optic neuritis from the optic neuritis of multiple sclerosis. Am J Ophthalmol 1978; 86: 530–535.
Hennerici M, Wenzel D, Freund HJ . The comparison of small size rectangle and checkerboard stimulation for the evaluation of delayed visual evoked responses in patients suspected of multiple sclerosis. Brain 1977; 100: 119–136.
Glaser JS, Laflamme P . The visual evoked response: methodology and application in optic nerve disease. In: Thompson HS (ed). Topics in Neuro-ophthalmology. Williams & Wilkins: Baltimore, MD, 1979, pp 199–218.
Harding GFA, Crews SJ, Good PA . VEP in neuroophthalmic disease. In: Barber C (ed). Evoked Potentials.. MTP Press Ltd: Lancaster, England, 1980, pp 235–241.
Holder GE . The visual evoked potential in ischaemic optic neuropathy. Doc Ophthalmol Proc Ser 1981; 27: 123–129.
Cox TA, Thompson HS, Hayreh SS, Snyder JE . Visual evoked potential and pupillary signs. Arch Ophthalmol 1982; 100: 1603–1607.
Wildberger H . Pattern-evoked potentials and visual field defects in ischaemic optic neuropathy. Doc Ophthalmol Proc Ser 1984; 40: 193–201.
Lehmann D, Skrandies W . Visually evoked scalp potential fields in hemiretinal stimulation. Doc Ophthalmol Proc Ser 1980; 23: 237–243.
Thompson PD, Mastaglia FL, Carroll WM . Anterior ischaemic optic neuropathy. A correlative clinical and visual evoked potential study of 18 patients. J Neurol Neurosurg Psychiatry 1986; 49: 128–135.
Holder GE . Ischaemic optic neuropathy. In: Heckenlively JR and Arden GB (eds). Principles and Practice of Clinical Electrophysiology of Vision. Mosby Year Book: St Louis, MO, 1991, pp 636–639.
Almarcegui C, Dolz I, Alejos MV, Fernandez FJ, Valdizan JR, Honrubia FM . Pattern electroretinogram in anterior ischemic optic neuropathy. Rev Neurol 2001; 32: 18–21.
Salazar JJ, Ramirez AI, De Hoz R, Triviño A, Ramirez JM . Apoptosis in optical ischaemic neuropathy. Abstracts, EVER Meeting, Palma 1999.
Muller W . Untersuchungen uber das Verhalten der Corticalzeit bei bitemporaler Hemianopsie. Graefes Arch Ophthalmol 1962; 165: 214–218.
Vaughan HG, Katzman R, Taylor J . Alterations of visual evoked response in the presence of homonymous visual defects. Electroencephalogr Clin Neurophysiol 1963; 15: 737–746.
Jacobsen JH, Hirose T, Suziki TA . Simultaneous ERG and VER in lesions of the optic pathway. Invest Ophthalmol 1968; 7: 279–292.
Kooi KA, Yamada T, Marshall RE . Field studies of monocularly evoked cerebral potentials in bitemporal hemianopsia. Neurology 1973; 23: 1217–1225.
Wildberger HG, Van Lith GH, Wijngaarde R, Mak GT . Visually evoked cortical potentials in the evaluation of homonymous and bitemporal visual field defects. Br J Ophthalmol 1976; 60: 273–278.
Halliday AM, Halliday M, Kriss A et al. The pattern evoked potential in compression of the anterior visual pathways. Brain 1976; 99: 357–374.
Blumhardt LD, Barrett G, Halliday AM . The asymmetrical visual evoked potential to pattern reversal in one half field and its significance for the analysis of visual field defects. Br J Ophthalmol 1977; 61: 454–461.
Holder GE . The effects of chiasmal compression on the pattern visual evoked potential. Electroencephalogr Clin Neurophysiol 1978; 45: 278–280.
Holder GE, Bullock PR . Visual evoked potentials in the assessment of patients with non-functioning chromophobe adenomas. J Neurol Neurosurg Psychiatry 1989; 52: 31–37.
Gott PS, Weiss MH, Apuzzo M, van der Meulen JP . Checkerboard visual evoked response in evaluation and management of pituitary tumours. Neurosurgery 1979; 5: 553–558.
Stark DJ, Lenton L . Electrophysiological assessment of compressive lesions of anterior visual pathways. Aust J Ophthalmol 1981; 9: 135–141.
Haimovic IC, Pedley TA . Hemi-field pattern reversal visual evoked potentials. II. Lesions of the chiasm and posterior visual pathways. Electroencephalogr Clin Neurophysiol 1982; 54: 121–131.
Maitland CG, Aminoff MJ, Kennard C, Hoyt WF . Evoked potentials in the evaluation of visual field defects due to chiasmal or retrochiasmal lesions. Neurology 1982; 32: 986–991.
Onofrj M, Bodis-Wollner I, Mylin L . Visual evoked potential diagnosis of field defects in patients with chiasmatic and retrochiasmatic lesions. J Neurol Neurosurg Psychiatry 1982; 45: 294–302.
Flanagan JG, Harding GFA . Multi-channel visual evoked potentials in early compressive lesions of the optic chiasm. Doc Ophthalmol 1987; 69: 271–282.
Brecelj J . Electrodiagnostics of chiasmal compressive lesions. Int J Psychophysiol 1994; 16: 263–272.
Kaufman DI, Lorance RW, Woods M, Wray SH . The pattern electroretinogram: a long-term study in acute optic neuropathy. Neurology 1988; 38: 1767–1774.
Ruther K, Ehlich P, Philipp A, Eckstein A, Zrenner E . Prognostic value of the pattern electroretinogram in cases of tumors affecting the optic pathway. Graefes Arch Clin Exp Ophthalmol 1998; 236: 259–263.
Parmar DN, Sofat A, Bowman R, Bartlett JR, Holder GE . Prognostic value of the pattern electroretinogram in chiasmal compression. Br J Ophthalmol 2000; 84: 1024–1026.
Kjer P . Infantile optic atrophy with dominant mode of inheritance: a clinical and genetic study of 19 Danish families. Acta Ophthalmol 1959; 37 (Suppl 54): 1–146.
Borruat FX, Green WT, Graham EM, Sweeney MG, Morgan-Hughes JA, Sanders MD . Late onset Leber's optic neuropathy: a case confused with ischaemic optic neuropathy. Br J Ophthalmol 1992; 76: 571–573.
Riordan-Eva P, Sanders MD, Govan GG, Sweeney MG, Da Costa J, Harding AE . The clinical features of Leber's hereditary optic neuropathy defined by the presence of a pathogenic mitochondrial DNA mutation. Brain 1995; 118: 319–337.
Johns DR, Newman NJ . Hereditary optic neuropathies. Semin Ophthalmol 1995; 10: 203–213.
Holder GE . The pattern electroretinogram in anterior visual pathway dysfunction and its relationship to the pattern visual evoked potential: a personal clinical review of 743 eyes. Eye 1997; 11: 924–934.
Hung HL, Kao LY, Huang CC . Clinical features of Leber's hereditary optic neuropathy with the 11778 mitochondrial DNA mutation in Taiwanese patients. Chang Gung Med J 2003; 26: 41–47.
Votruba M, Fitzke FW, Holder GE, Carter A, Bhattacharya SS, Moore AT . Clinical features in affected individuals from 21 pedigrees with dominant optic atrophy. Arch Ophthalmol 1998; 116: 351–358.
Johnston PB, Gaster RN, Smith VC, Tripathi RC . A clinicopathological study of autosomal dominant optic atrophy. Am J Ophthalmol 1979; 88: 868–875.
Kjer P . Histopathology of eye, optic nerve and brain in a case of dominant optic atrophy. Acta Ophthalmol 1982; 61: 300–312.
Berninger TA, Jaeger W, Krastel H . Electrophysiology and colour perimetry in dominant infantile optic atrophy. Br J Ophthalmol 1991; 75: 49–52.
Holder GE, Votruba M, Carter AC, Bhattacharya SS, Fitzke FW, Moore AT . Electrophysiological findings in dominant optic atrophy (DOA) linking to the OPA1 locus on chromosome 3q28–qter. Doc Ophthalmol 1999; 95: 217–228.
Dorey SE, Neveu MM, Burton LC, Sloper JJ, Holder GE . The clinical features of albinism and their correlation with visual evoked potentials. Br J Ophthalmol 2003; 87: 767–772.
Apkarian P . A practical approach to albino diagnosis. VEP misrouting across the age span. Ophthalmic Paediatr Genet 1992; 13: 77–88.
Kriss A, Russell-Eggitt I, Harris CM, Lloyd IC, Taylor D . Aspects of albinism. Ophthalmic Paediatr Genet 1992; 13: 89–100.
Neveu MM, Jeffery G, Burton LC, Sloper JJ, Holder GE . Age-related changes in the dynamics of human albino visual pathways. Eur J Neurosci 2003; 18: 1939–1949.
Srivastava AK, Goel UC, Bajaj S, Singh KJ, Dwivedi NC, Tandon MP . Visual evoked responses in ethambutol induced optic neuritis. J Assoc Physicians India 1997; 45: 847–849.
Sadun AA . Distinguishing between clinical impairments due to optic nerve or macular disease. Metab Pediatr Syst Ophthalmol 1990; 13: 79–84.
Newman NJ . Optic disc pallor: a false localizing sign. Surv Ophthalmol 1993; 37: 273–282.
Thompson HS, Warsky RC, Weinstein JM . Pupillary dysfunction and macular disease. Trans Am Ophthalmol Soc 1980; 78: 311–317.
Newsome DA, Milton RC, Gass JD . Afferent pupillary defect in macular degeneration. Am J Ophthalmol 1981; 92: 396–402.
Folk JC, Thompson HS, Han DP, Brown CK . Visual function abnormalities in central serous retinopathy. Arch Ophthalmol 1984; 102: 1299–1302.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Holder, G. Electrophysiological assessment of optic nerve disease. Eye 18, 1133–1143 (2004). https://doi.org/10.1038/sj.eye.6701573
Received:
Accepted:
Published:
Issue date:
DOI: https://doi.org/10.1038/sj.eye.6701573
Keywords
This article is cited by
-
Analysis of the slope between P50 and N95 waves of the large field pattern electroretinogram as an additional indicator of ganglion cell dysfunction
Documenta Ophthalmologica (2023)
-
Pattern ERGs suggest a possible retinal contribution to the visual acuity loss in acute optic neuritis
Documenta Ophthalmologica (2022)
-
Evaluation of VEP parameters in patients before and after cardiopulmonary by-pass surgery
International Ophthalmology (2022)
-
White matter tract conductivity is resistant to wide variations in paranodal structure and myelin thickness accompanying the loss of Tyro3: an experimental and simulated analysis
Brain Structure and Function (2022)
-
The effect of optic neuritis treatment trial (ONTT) combined corticosteroid regimen on pattern reversal visual evoked potentials: a prospective follow-up study
BMC Ophthalmology (2021)
