Abstract
Visual electrodiagnostic tests can contribute significantly to pediatric ophthalmology. The tests are objective, safe, relatively swift, and easy to administer. They can give unique insight into the functional integrity of different levels of the visual pathway. The electroretinogram (ERG) indicates retinal function, the electro-oculogram (EOG) expresses pigment epithelium function, and the visual evoked potential (VEP ) reflects optic pathway function beyond the eye to the visual cortex. These tests complement, and supplement, other visual methods of assessment. Thus,depending on the clinical context, an abnormal ERG may suggest the necessity for metabolic screening, and an abnormal VEP in association with a normal ERG can indicate the need for structural imaging studies.
In Memoriam: On October 5, 2001, Dr. Kriss passed away after a long illness. Despite the gravity of his ill health, Dr. Kriss worked hard to finish this chapter and submitted it one week before his death. His tenacity in completing this definitive work on Pediatric Electrophysiology is a testament to his passion for this field. I have known Dr. Kriss professionally for almost 20 years and have the highest respect for his research, teaching, and clinical abilities. This chapter exemplifies Dr. Kriss’ commitment to excellence, attention to detail, and his world-class expertise in the field of Pediatric Electrophysiology. It is my honor and privilege to include his chapter in this second edition by Kenneth W. Wright, MD.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Acland G, Aguirre G, Ray J, et al. Gene therapy restores vision in a canine model of childhood blindness. Nat Genet 2001;28(1):92–95.
Agamanolis D, Chester E, Victor M, et al. Neuropathology of experimental vitamin B12 deficiency in monkeys. Neurology 1976;26:905–914.
Alani S. Pattern-reversal visual evoked potentials in patients with hydrocephalus. J Neurosurg 1985;62(2):234–237.
Allen D, Tyler C, Norcia A. Development of grating acuity and contrast sensitivity in the central and peripheral visual field of the human infant. Vision Res 1996;36(13):1945–1953.
Apkarian P. Electrodiagnosis in paediatric ophthalmogenetics. Int J Psychophysiol 1994;16(2–3):229–243.
Apkarian P, Bour L, Bart P. A unique achiasmatic anomaly detected in non-albinos with misrouted retinal fugal projections. Eur J Neurosci 1994;6:501–507.
Apkarian P, Spekreijse H. The use of the electroretinogram and visual evoked potentials in ophthalmogenetics. In: Desmedt JE (ed) Visual evoked potentials. Amsterdam: Elsevier, 1990:169–223.
Apkarian P, Reits D, Spekreijse H. Component specificity in albino VEP asymmetry: maturation of the visual pathway anomaly. Exp Brain Res 1984;53:285–294.
Arden G, Kelsey J. Changes produced by light in the standing potential of the human eye. J Physiol 1962;61:189–204.
Arden G, Wolf J, Singbarti F, et al. Effect of alcohol and light on the retinal pigment epithelium of normal subjects and patients with retinal dystrophies. Br J Ophthalmol 2000;84(8):881–883.
Arndt C, Derambure P, Defoort-Dhellemmes S, et al. Outer retinal dysfunction in patients treated with vigabatrin. Neurology 1999;52(6):1201–1205.
Bach M, Hyalina M, Holder G, et al. Standard for pattern electroretinography. Doc Ophthalmol 2000;101:11–18.
Backhouse O, Leitch R, Thompson D, et al. A case of reversible blindness in maple syrup urine disease [letter]. Br J Ophthalmol 1999;83(2):250–251.
Barrett G, Blumhardt L, Halliday A, et al. A paradox in the lateralization of the visual evoked response. Nature (Lond) 1976;261:253–255.
Bayer A, Zrenner E, Ried S, et al. Effect of anticonvulsant drugs on retinal function. Pyschophysical and electrophysiological findings in patients with epilepsy. Investig Ophthalmol Vis Sci 1990;31:427.
Beales P, Elcioglu N, Woolf A, et al. New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey. J Med Genet 1999;36(6):437–446.
Bech-Hansen NT, Naylor M, Maybaum T, et al. Mutations in NYX, encoding the leucine-rich proteoglycan nyctalopin, cause X-linked complete congenital stationary night blindness. Nat Genet 2000;26(3):319–323.
Berson E. Nutrition and retinal generations. Int Ophthalmol Clin 2000;40(4):93–111.
Berson E. Electrical phenomena in the retina. In: Hart WH (ed) Adler’s physiology of the eye: clinical application. St. Louis: Mosby Yearbook, 1992:641–707.
Birch E, Fawcett S, Stager D. Co-development of VEP motion response and binocular vision in normal infants and infantile esotropes. Investig Ophthalmol Vis Sci 2000;41:1719–1723.
Birch E, Birch D, Uauy R. Retinal and cortical function of very low birthweight infants at 36 and 57 weeks post conception. Clin Vis Res 1990;5:363–373.
Blumhardt L, Barratt G, Kriss A, et al. The pattern-evoked potential in lesions of the posterior visual pathways. Ann NY Acad Sci 1982;388:264–289.
Bodis-Wollner I. Recovery from cerebral blindness: evoked potential and psychophysical measurements. Electroencephalogr Clin Neurophysiol 1977;42(2):178–184.
Boycott K, Maybaum T, Naylor M, et al. A summary of 20 CACNA1F mutations identified in 36 families with incomplete X-linked congenital stationary night blindness, and characterization of splice variants. Hum Genet 2001;108(2):91–97.
Bradshaw K, George N, Moore A, et al. Mutations of the XLRS1 gene causing abnormalities of photoreceptor as well as inner retinal responses of the ERG. Doc Ophthalmol 1999;98:153–173.
Brecelj J, Stirn-Kranjc B, Skrbec M. Visual electrophysiology in children with tumors affecting the visual pathways. Doc Ophthalmol 2000;101:125–154.
Brecelj J. Electrodiagnosis of chiasmal compressive lesions. Int J Psychophysiol 1994;16:263–272.
Breton M, Schueller A, Lamb T, et al. Analysis of ERG a-wave amplification and kinetics in terms of the G-protein cascade of photo-transduction. Investig Ophthalmol Vis Sci 1994;35(1):295–309.
Breton M, Quinn G, Schueller A. Development of electroretinogram and rod phototransduction response in human infants. Investig Ophthalmol Vis Sci 1995;36(8):1588–1602.
Campbell F, Gubisch R. Optical quality of the human eye. J Physiol 1966;186(3):558–578.
Campbell F, Maffei L. Electrophysiological evidence for the existence of orientation and size detectors in the human visual system. J Physiol 1970;207(3):635–652.
Carroll W, Mastaglia F. Leber’s optic neuropathy: a clinical and visual evoked potential study of affected and asymptomatic members of a six-generation family. Brain 1979;1102:559–580.
Carroll W, Kriss A, Baraitser M, et al. The incidence and nature of visual pathway involvement in Friedreich’s ataxia: a clinical and visual evoked potential study of 22 patients. Brain 1980;103:413–434.
Celesia G, Archer C, Kurroiwa Y, et al. Visual function of the extrageniculate-calcarine system in man. Arch Neurol 1980;37:704–706.
Celesia G, Brigell M. Recommended standards for pattern electroretinograms and visual evoked potentials. The International Federation of Clinical Neurophysiology. Electroencephalogr Clin Neurophysiol Suppl 1999;52:53–67.
Celesia G, Brigell M, Peachey N. Recommended standards for electroretinograms. The International Federation of Clinical Neurophysiology. Electroencephalogr Clin Neurophysiol Suppl 1999;52:45–52.
Celesia G, Meredith J, Pluff K. Perimetry, visual evoked potentials spectrum in homonymous hemianopsia. Electroencephalogr Clin Neurophysiol 1983;56:16–30.
Chokroverty S, Duvoisin R, Sachdeo R, et al. Neurophysiologic study of olivopontocerebellar atrophy with or without glutamate dehydrogenase deficiency. Neurology 1985;35:652–659.
Cibis G, Fitzgerald K, Harris D, et al. The effects of dystrophin gene mutations on the ERG in mice and humans. Investig Ophthalmol Vis Sci 1993;34(13):3646–3652.
Cibis G, Fitzgerald K. Abnormal electroretinogram associated with developmental brain anomalies. Trans Am Ophthalmol Soc 1995;93:147–158;discussion 158–161.
Cicedyian A, Jacobsen S. Negative electroretinograms in retinitis pigmentosa. Investiag Ophthalmol Vis Sci 1993;34:3253–3263.
Cohen S, Brown F, Martyn L, et al. Ocular histopathologic and biochemical studies of the cerebrohepatorenal syndrome (Zellweger’s syndrome) and its relationship to neonatal adrenoleucodystrophy. Am J Ophthalmol 1983;96:488–501.
Creel D, Bendel C, Wiesner G, et al. Abnormalities of the central visual pathways in Prader-Willi syndrome associated with hypopigmentation. N Engl J Med 1986;314(25):1606–1609.
Creel D, Spekreijse H, Reits D. Evoked potentials in albinos: efficacy of pattern stimulation in detecting misrouted optic fibres. Electroencephalogr Clin Neurophysiol 1981;52:595–603.
Crews S, Thompson C, Harding G. The ERG and VEP in patients with severe eye injury. Doc Ophthalmol 1978;15:203–209.
Dekaban A. Hereditary syndrome of congenital blindness (Leber), polycystic kidneys and maldevelopment of the brain. Am J Ophthalmol 1969;689:1029–1036.
Dell’Osso L. Seesaw nystagmus in dogs and humans: an international, across discipline, serendipitous collaboration. Neurology 1996;47:1372–1374.
De Vries Khoe L, Spekreijse H. Maturation of luminance and pattern EPs in man. Doc Ophthalmol 1982;31:461–475.
Dorfman L, Nikoskelianen E, Rosenthal A, et al. Visual evoked potentials in Leber’s optic neuropathy. Ann Neurol 1977;1:565–568.
Drasdo N, Edwards L, Thompson D. Models of the visual cortex based on visual evoked potentials. In: Gulyas B, Ottoson D, Roland P (eds) Functional organization of the human visual cortex. Wenner Gren international series, vol 61. Oxford: Pergamon Press, 1993:255–270.
Drasdo N, Thompson D, Arden G. A comparison of pattern ERG amplitudes and nuclear layer thickness in different zones of the retina. Clin Vis Sci 1990;5:415–420.
Eke T, Talbot J, Lawden M. Severe persistent visual field constriction associated with vigabatrin [see comments]. Br Med J 1997;314(7075):180–181.
Esakowitz L, Kriss A, Shawkat F. A comparison of flash electroretinograms recorded from Burian Allen, JET, C-Glide, gold foil, DTL, and skin electrodes. Eye 1993;7:169–171.
Fagan E, Taylor M. Longitudinal multimodal evoked potential studies in abetalipoproteinaemia. Can J Neurol Sci 1987;14:617–621.
Farley M, Heckenlively J. Blue cone monochromatism. In: Heckenlively J, Arden G (eds) Principles and practice of clinical electrophysiology of vision. St. Loius: Mosby, 1991:753–755.
Fellman D, Van Essen D. Distributed hierarchical processing in the primate visual cortex. Cereb Cortex 1991;1:1–47.
Fitzgerald K, Hashimoto T, Hug T, et al. Autosomal dominant inheritance of a negative electroretinogram in three generations. Am J Ophthalmol 2001;131(4):495–502.
Fitzgerald K, Cibis G, Giambrone S, et al. Retinal signal transmission in Duchenne muscular dystrophy: evidence for dysfunction in the photoreceptor depolarising bipolar cell pathway. J Clin Investig 1994;93:2425–2430.
Flanagan J, Harding G. Multichannel visual evoked potentials in early compressive lesions of the optic chiasm. Doc Ophthalmol 1987;69:271–282.
Flaxel C, Jay M, Thiselton D, et al. Difference between RP2 and RP3 phenotypes in X linked retinitis pigmentosa. Br J Ophthalmol 1999;83(10):1144–1148.
Fulton A, Hansen R, Glynn R. Natural course of visual functions in the Bardet-Biedl syndrome. Arch Ophthalmol 1993;111(11):1500–1506.
Fulton A, Hansen R. Electroretinography: application to clinical studies of infants. J Pediatr Ophthalmol Strabismus 1985;22(6):251–255.
Fulton A, Hartmann E, Hansen R. Electrophysiological testing techniques for children. Doc Ophthalmol 1989;71:341–354.
Galloway N. Electrophysiological testing of eyes with opaque media. Eye 1988;2:615–624.
Garbern J, Cambi F, Shy M, et al. The molecular pathogenesis of Pelizaeus-Merzbacher disease. Arch Neurol 1999;56(10):1210–1214.
Garner A, Fielder A, Primavesi R, et al. Tapetoretinal degeneration in the cerebro-hepato-renal (Zellweger) syndrome. Br J Ophthalmol 1982;66:422–431.
Gerritsen E, Vossen J, van Loo, et al. Autosomal recessive osteopetrosis: variability of findings at diagnosis and during the natural course. Pediatrics 1994;93:247–253.
Givre S, Schroeder C, Arezzo J. Contribution of extra striate area V4 to the surface recorded flash VEP in the awake macaque. Vision Res 1994;34:415–428.
Gottlob I, Fendick M, Guo S, et al. Visual acuity measurements by swept spatial frequency visual-evoked-cortical potentials (VECPs): clinical application in children with various visual disorders. J Pediatr Ophthalmol Strabismus 1990;27(1):40–47.
Granit R. Sensory mechanisms of the retina. London: Oxford University Press, 1947.
Grant C, Berson E. Treatable forms of retinitis pigmentosa associated with systemic neurological disorders. Int Ophthalmol Clin 2001;41(1):103–110.
Grieshaber M, Boltshauser E, Niemeyer G. Leber’s congenital amaurosis. Clinical heterogeneity and electroretinography in 27 patients. In: Hollyfield X, et al. (eds) Retinal degenerative diseases and experimental therapy. New York: Kluwer, 1999:95–104.
Groswasser Z, Kriss A, Halliday AM, et al. Pattern and flash evoked potentials in the assessment and management of optic nerve gliomas. J Neurol Neurosurg Psychiatry 1985;48:1125–1134.
Grover S, Fishman G, Anderson R, et al. A longitudinal study of visual function in carriers of X-linked recessive retinitis pigmentosa. Ophthalmology 2000;107(2):386–396.
Haegerstrom-Portnoy G, Schneck M, Verdon W, et al. Clinical vision characteristics of the congenital achromatopsias. II. Color vision. Optom Vis Sci 1996;73(7):457–465.
Haegerstrom-Portnoy G, Schneck M, Verdon W, et al. Clinical vision characteristics of the congenital achromatopsias. I. Visual acuity, refractive error, and binocular status. Optom Vis Sci 1996;73(7):446–456.
Haider N, Jacobsen S, Cideciyan A, et al. Mutation of a nuclear receptor gene, NR2E3, causes enhanced cone syndrome, a disorder of retinal cell fate. Nat Genet 2000;24:127–131.
Halliday A. Evoked potentials in clinical testing, 2nd edn. Edinburgh: Churchill Livingstone, 1993.
Halliday A, Barrett G, Blumhardt L, et al. The macular and paramacular components of the pattern evoked response. In: Lehmann D, Calloway E (eds) Human evoked potentials: applications and problems. New York: Plenum Press, 1979:135–151.
Halliday A, McDonald W, Mushin J. Delayed pattern evoked responses in optic neuritis in relation to visual acuity. Trans Ophthalmol Soc UK 1973;93:315–324.
Halliday A, Kriss A, Cuendent F, et al. Childhood optic neuritis: a study of flash and pattern evoked potentials. In: Gallai V (ed) Maturation of the CNS and evoked potentials. Amsterdam: Elsevier, 1986:41–50.
Hamer R, Norcia A, Tyler C, et al. The development of monocular and binocular VEP acuity. Vision Res 1989;29(4):397–408.
Harden A, Pampiglione G. Neurophysiological studies (EEG/ERG/VEP/SEP) in 88 children with so-called neuronal ceroid lipofuscinosis. In: Armstrong D, Koppang N, Rider JA (eds) Ceroid lipofuscinosis (Batten’s disease). Amsterdam: Elsevier, 1982:61–70.
Harding G, Wild J, Robertson K, et al. Separating the retinal electrophysiologic effects of vigabatrin: treatment versus field loss [see comments]. Neurology 2000;55(3):347–352.
Harding G, Wild J, Robertson K, et al. Electro-oculography, electroretinography, visual evoked potentials, and multifocal electroretinography in patients with vigabatrin-attributed visual field constriction. Epilepsia 2000;41(11):1420–1431.
Harding G, Crews S. The VER in hereditary optic atrophy of the dominant type. In: Mauguiere F, Courjon F (eds) The clinical applications of evoked potentials in neurology. New York: Raven Press, 1982:21–30.
Harding G, Williams D, Innes J. The visual evoked potentials and psychophysics during ethambutol therapy. In: Nodar RH, Barber C (eds) Evoked potentials, vol ll. Boston: Butterworth, 1984:339–344.
Harris C. Nystagmus and eye movement disorders. In: Taylor D (ed) Pediatric ophthalmology. Oxford: Blackwell, 1997:869–896.
Hawksworth N, Headland S, Good P, et al. Aland Island eye disease: clinical and electrophysiological studies of a Welsh family. Br J Ophthalmol 1995;79(5):424–430.
Holder G. Pattern electroretinography (PERG) and an integrated approach to visual pathway diagnosis. In: Fishman GA, Birch D, Holder GE, Brigell M (eds) Electrophysiological testing in disorders of retinal optic nerve, and visual pathway. 2001.
Hood D, Birch D. Abnormalities of the retinal cone system in retinitis pigmentosa. Vision Res 1996;36(11):1699–1709.
Hood D, Birch D. Assessing abnormal rod photoreceptor activity with the a-wave of the electroretinogram: applications and methods. Doc Ophthalmol 1996–97;92(4):253–267.
Hoyt C, Billson F. Visual loss in osteopetrosis. Am J Dis Child 1979;133:955–958.
Hutton W, Fuller D. Factors influencing final visual results in severely injured eyes. Am J Ophthalmol 1984;97:715–722.
Iannoccone A, De-Propris G, Roncati S, et al. The ocular phenotype of the Bardet-Biedl syndrome. Comparison to non-syndromic retinitis pigmentosa. Ophthalmic Genet 1997;18:13–26.
Jabbari B, Maitland C, Morris L, et al. The value of visual evoked potential as a screening test in neurofibromatosis. Arch Neurol 1985; 42:1072–1074.
Jacobs M, Shawkat F, Harris C, et al. Eye movement and electrophysiological findings in an infant with hemispheric pathology. Dev Med Child Neurol 1993;35(5):431–435.
Jeffreys D, Axford J. Source localisations of pattern-specific components of human visual evoked potentials l. Component of striate cortical origin. Exp Brain Res 1972;6:1–21.
Jeffreys D, Axford J. Source localisations of pattern-specific components of human visual evoked potentials. ll. Component of extrastriate cortical origin. Exp Brain Res 1972;6:22–40.
Jensen H, Warburg M, Sjo O, et al. Duchenne muscular dystrophy: negative electroretinograms and normal dark adaptation. Reappraisal of assignment of X-linked incomplete congenital stationary night blindness. J Med Genet 1995;32(5):348–351.
Jiang C, Hansen R, Gee B, et al. Rod and rod mediated function in patients with beta-thalassemia major. Doc Ophthamol 1998–99; 96(4):333–345.
Joubert M, Eisenring J, Robb J, et al. Familial agenesis of the cerebellar vermis: a syndrome of episodic hyperapnea, abnormal eye movements, ataxia, and retardation. 1969 [classical article]. J Child Neurol 1999;14(9):554–564.
Keith C. Retinal atrophy in osteopetrosis. Arch Ophthalmol 1968;79:234–241.
Kellner U, Weleber R, Kennaway N, et al. Gyrate atrophy-like phenotype with normal plasma ornithine. Retina 1997;17(5):403–413.
Kellner U, Foerster M. Cone dystrophies with negative photopic electroretinogram. Br J Ophthalmol 1993;77(7):404–409.
Klevering B, van-Driel M, van de Pol D, et al. Phenotypic variations in a family with retinal dystrophy as result of different mutations in the ABCR gene. Br J Ophthalmol 1999;83(8):914–918.
Kondo M, Sieving P. Primate photopic sine-wave flicker ERG: vector modeling and component origins using glutamate analogs. Investig Ophthalmol Vis Sci 2001;42(1):305–312.
Kraemer M, Abrahamsson M, Sjostrum A. The neonatal development of the light flash visual evoked potential. Doc Ophthalmol 1999;99:21–39.
Krauss G, Johnson M, Miller N. Vigabatrin-associated retinal cone system dysfunction: electroretinogram and ophthalmologic findings [see comments]. Neurology 1998;51(6):1778–1779; discussion 1779–1781.
Kretschmann U, Seeliger M, Ruether K, et al. Multifocal electroretinography in patients with Stargardt’s macular dystrophy. Br J Ophthalmol 1998;82(3):267–275.
Kriss A, Thompson D, Lavy T, et al. Pattern VEPs and craniopharyngiomas in children. Investig Ophthalmol Vis Sci 1996;37:1075.
Kriss A, Russell-Eggitt I, Harris C, et al. Aspects of albinism. Ophthalmol Paediatr Genet 1992;13:89–100.
Kriss A, Thompson D, Lloyd I, et al. Pattern VEP findings in young children treated for unilateral congenital cataract. In: Cottlier E (ed) Congenital cataracts. Austin: Landes, 1994:79–88.
Kriss A, Carroll W, Blumhardt L, et al. Pattern and flash evoked potential changes in toxic (nutritional) optic neuropathy. In: Courjon J, Maugiere F, Revol M (eds) Clinical applications of evoked potentials in neurology. Advances in neurology, vol 32. New York: Raven Press, 1982:11–19.
Kriss A, Francis D, Cluendet F, et al. Recovery from optic neuritis in childhood. J Neurol Neurosurg Psychiatry 1988;51:1253–1258.
Kriss A, Thompson D. Visual electrophysiology. In: Taylor D (ed) Pediatric ophthalmology. Oxford: Blackwell, 1997:93–121.
Kriss A, Russell-Eggitt I. Electrophysiological assessment of visual pathway function in infants. Eye 1992;6:145–153.
Kriss A, Russell-Eggitt I, Harris C, et al. Aspects of albinism. Ophthalmol Paediatr Genet 1992;13:89–100.
Kubova Z, Kuba M. Clinical application of motion onset potentials. Doc Ophthalmol 1992;81:209–218.
Kupersmith M, Siegel I, Carr R, et al. Visual evoked potentials in chiasmal gliomas in four adults. Arch Neurol 1981;38:362–366.
Lachapelle P, Rousseau S, McKerral M, et al. Evidence supportive of a functional discrimination between photopic oscillatory potentials as revealed with cone and rod mediated retinopathies. Doc Ophthalmol 1998;95(1):35–54.
Lachapelle P, Little J, Polomeno R. The photopic electroretinogram in congenital stationary night blindness with myopia. Investig Ophthalmol Vis Sci 1983;24:442–450.
Lambert R, Kriss A, Taylor D. Delayed visual maturation; a longitudinal clinical and electrophysiological assessment. Ophthalmology 1989;96:534–529.
Lambert S, Taylor D, Kriss A. The infant with nystagmus, normal appearing fundi but an abnormal ERG. Surv Ophthalmol 1989;34: 176–186.
Lambert S, Kriss A, Taylor D. Detection of isolated occipital lobe anomalies during early childhood. Dev Med Child Neurol 1990;32(5):451–455.
Lambert S, Kriss A, Taylor D. Joubert syndrome. Arch Ophthalmol 1989;107:709–713.
Lambert S, Taylor D, Kriss A, et al. Follow-up and diagnostic reappraisal of 75 patients with Leber’s congenital amaurosis. Am J Ophthalmol 1989;107:624–631.
Lang G, Maumenee I. Retinal dystrophies associated with storage diseases. In: Newsome DA (ed) Retinal dystrophies and degenerations. New York: Raven Press, 1988:319–340.
Lavy T, Harris C, Shawkat F, et al. Electrophysiological and eye movement abnormalities in children with the Bardet-Biedl syndrome. J Pediatr Ophthalmol Strabismus 1995;32(6):364–367.
Lei B, Bush R, Milam A, et al. Human melanoma-associated retinopathy (MAR) antibodies alter the retinal ON-response of the monkey ERG in vivo. Investig Ophthalmol Vis Sci 2000;41(1): 262–266.
Lennerstrand G. Delayed visual evoked cortical potentials in retinal disease. Acta Ophthalmol (Copenh) 1982;60:497–504.
Livingstone M, Hubel D. Segregation of form, colour, movement and depth: anatomy, physiology and perception. Science 1988;240:740–750.
Lois N, Holder G, Bunce C, et al. Phenotypic subtypes of Stargardt’s macular dystrophy-fundus flavimaculatus. Arch Ophthalmol 2001;119(3):359–369.
Lorenz B, Gampe E. Analyse von 180 Patienten mit sensorischem Defektnystagmus (SDN) und kongenitalem idiopathischen Nystagmus (CIN). [Analysis of 180 patients with sensory defect nystagmus (SDN) and congenital idiopathic nystagmus (CIN).] Klin Monatsbl Augenheilkd 2001;218(1):3–12.
Mandelbaum S, Cleary P, Ruan S, et al. Bright flash electroretinography and vitreous haemorrhage. Arch Ophthalmol 1980;98:1823–1828.
Marg E, Freeman D, Peltzman P, et al. Visual acuity development in human infants: evoked potential measurements. Investig Ophthalmol Vis Sci 1976;15:150–153.
Markwardt F, Gopfert E, Muller R. Influence of velocity, temporal frequency and initial phase position of gratings on the motion VEP. Biomed Biochim Acta 1988;47:753–760.
Marmor M, Jacobsen S, Foerster M, et al. Diagnostic findings of a new syndrome with night blindness, maculopathy, and enhanced s cone sensitivity. Am J Ophthalmol 1990;110:124–134.
Maugeri A, Klevering B, Rohrschneider K, et al. Mutations in the ABCA4 (ABCR) gene are the major cause of autosomal recessive cone-rod dystrophy. Am J Hum Genet 2000;67(4):960–966.
McCulloch D, Skarf B. Pattern reversal visual evoked potentials following early treatment of unilateral, congenital cataract [see comments]. Arch Ophthalmol 1994;112(4):510–518.
McCulloch D, Orbach H, Skarf B. Maturation of the pattern reversal VEP in human infants: a theoretical framework. Vision Res 1999;39:3673–3680.
Merigan W. P and M pathway specialisation in the macaque. In: Valberg A, Lee B (eds) From pigments to perception: advances in understanding visual processes. NATO ASI series no. 203. New York: Plenum Press, 1991;117–125.
Miyake Y, Horiguchi M, Suzuki S, et al. Electrophysiological findings in patients with Oguchi’s disease. Jpn J Ophthalmol 1996;40(4):511–519.
Miyake Y, Horiguchi M, Terasaki H, et al. Scotopic threshold response in complete and incomplete types of congenital stationary night blindness. Investig Ophthalmol Vis Sci 1994;35:3770–3775.
Miyake Y, Yagasaki K, Horiguchi M, et al. Congenital stationary night blindness with a negative electroretinogram: a new classification. Arch Ophthalmol 1986;104:1013–1020.
Miyake Y, Shiroyama N, Horiguchi M, et al. Bull’s eye maculopathy and negative electroretinograms. Retina 1989;9:210–215.
Mohri I, Taniike M, Fujimura H, et al. A case of Kearns-Sayre syndrome showing a constant proportion of deleted mitochondrial DNA in blood cells during 6 years of follow-up. J Neurol Sci 1998;158(1): 106–109.
Moskowitz A, Sokol S. Spatial and temporal interaction of patternevoked cortical potentials in human infants. Vision Res 1980;20(8): 699–707.
Mullie M, Harding A, Petty R, et al. The retinal manifestations of mitochondrial myopathy: a study of 22 cases. Arch Ophthalmol 1985;103:1825–1830.
Multifocal electroretinography: special issue. The multifocal technique: topographic ERG and VEP responses. Doc Ophthalmol 2001;100:49–251.
Nakamura M, Ito S, Terasaki H, et al. Novel CACNA1F mutations in Japanese patients with incomplete congenital stationary night blindness. Investig Ophthalmol Vis Sci 2001;42(7):1610–1616.
Niemeyer G. Pharmacological effects in retinal electrophysiology. In: Heckenlively JR, Arden GB (eds) Principles and practice of clinical electrophysiology of vision. St. Louis: Mosby, 1991:151–162.
Norcia A, Tyler C, Hamer R, et al. Measurement of spatial contrast sensitivity with the swept contrast VEP. Vision Res 1989;29(5):627–637.
Norcia A, Tyler C. Infant VEP acuity measurements: analysis of individual differences and measurement error. Electroencephalogr Clin Neurophysiol 1985;61(5):359–369.
Norcia A, Tyler C. Spatial frequency sweep VEP: visual acuity during the first year of life. Vision Res 1985;25(10):1399–1408.
Norcia A, Garcia H, Humphrey R, et al. Anomalous motion VEPs in infants and infantile esotropia. Investig Ophthalmol Vis Sci 1991;32:436–439.
Novack G. Ocular toxicology. Curr Opin Ophthalmol 1997;8(6):88–92.
Nusinowitz S, Birch D, Birch E. Rod photoresponses in 6-week and 4-month-old human infants. Vision Res 1998;38(5):627–635.
Nuwer M, Perlman S, Packwood J, et al. Evoked potential abnormalities in the various inherited ataxia. Ann Neurol 1983;13:20–27.
Orel-Bixler D, Norcia A. Differential growth for steady state pattern reversal and transient onset offset VEPs. Clin Vis Sci 1987;2:1–10.
Orel-Bixler D, Haegerstrom-Portnoy G, Hall A. Visual assessment of the multiply handicapped patient. Optom Vis Sci 1989;66(8):530–536.
Ossenblock P, Spekreijse H. The extra-striate generators of the EP to checkerboard onset. A source localisation approach. Electroencephalogr Clin Neurophysiol 1991;80:181–193.
Palczewski K. Is vertebrate phototransduction solved? New insights into the molecular mechanism of phototransduction. Investig Ophthalmol Vis Sci 1994;35(10):3577–3581.
Papaioannou M, Ocaka L, Bessant D, et al. An analysis of ABCR mutations in British patients with recessive retinal dystrophies. Investig Ophthalmol Vis Sci 2000;41(1):16–19.
Papakostopoulos D, Hart C, Cooper R, et al. Combined electrophysiological assessment of the visual system in central serous retinopathy. Electroencephalogr Clin Neurophysiol 1984;59(1):77–80.
Patel C, Taylor D, Russell-Eggitt I, et al. Congenital third nerve palsy associated with mid-trimester amniocentesis. Br J Ophthalmol 1993;77:530–533.
Pepperberg D, Birch D, Hood D. Photoresponses of human rods derived from paired flash ERGs. Vis Neurosci 1997;14:73–82.
Poggi-Travert F, Fournier B, Poll B, et al. Clinical approach to inherited peroxisomal disorders. J Inherit Metab Dis 1995;18(suppl 1):1–18.
Porteous W, James A, Sheard P, et al. Bioenergetic consequences of accumulating the common 4977-bp mitochondrial DNA deletion. Eur J Biochem 1998;257(1):192–201.
Pryds O, Greisen G. Preservation of single flash visual evoked potentials at very low cerebral oxygen delivery in preterm infants. Pediatr Neurol 1990;6:151–158.
Pugh E, Lamb T. Phototransduction in vertebrate rods and cones: molecular mechanisms of amplification, recovery and light adaptation. In: Stavenga DG, de Grip WJ, Pugh EN Jr (eds) Handbook of biological physics, vol 3. Amsterdam: Elsevier, 2000:183–254.
Purvin V, Kawasaki A, Yee R. Papilledema and obstructive sleep apnea syndrome. Arch Ophthalmol 2000;118:1626–1630.
Reardon W, Winter R. The molecular pathology of syndromic craniosynostosis. Mol Med Today 1995:432–437.
Regan D. Human brain electrophysiology. Amsterdam: Elsevier, 1989.
Regan D, Spekreijse H. Evoked potential indicators of colour blindness. Vision Res 1974;14:89–95.
Regan D, Regal D, Tibbles J. Evoked potentials during recovery from blindness recorded serially from an infant and his normally sighted twin. Electroencephalogr Clin Neurophysiol 1982;54:465–468.
Ripps H, Mahaffy L, Siegel I, et al. Vincristine-induced changes in the retina of isolated arterially perfused cat eye. Exp Eye Res 1989;48:771–790.
Robson J, Frishman L. Dissecting the dark-adapted electroretinogram. Doc Ophthalmol 1998–99;95(3–4):187–215.
Rozet J, Gerber S, Ghazi I, et al. Mutations of the retinal specific ATP binding transporter gene (ABCR) in a single family segregating both autosomal recessive retinitis pigmentosa RP19 and Stargardt’s disease: evidence of clinical heterogeneity at this locus. J Med Genet 1999;36(6):447–451.
Runge P, Muler D, McAllister J, et al. Oral vitamin E can prevent the retinopathy of abetalipoproteinaemia. Br J Ophthalmol 1986;70: 166–173.
Russell-Eggitt I, Harris M, Kriss A. Delayed visual maturation: an update. Dev Med Child Neurol 1998;40:130–136.
Sandberg M, Wiegel-DiFranco C, Drya T, et al. Clinical expression correlates with location of rhodopsin mutation in dominant retinitis pigmentosa. Investig Ophthalmol Vision Sci 1995;36:1934–1942.
Schaumberg D, Moyes A, Gomes J, et al. Corneal transplantation in young children with congenital hereditary endothelial dystrophy. Multicenter Pediatric Keratoplasty Study. Am J Ophthalmol 1999;127(4):373–378.
Schroeder C, Tenke C, Givre S, et al. Striate cortical contribution to the surface recorded pattern reversal VEP in the alert monkey. Vision Res 1991;3197(80):1143–1157 [also published erratum Vision Res 191;31 (11):1].
Seiple W, Holopigian K. An examination of VEP response phase. Electroencephalogr Clin Neurophysiol 1989;73(6):520–531.
Sharon D, Bruns G, Mcgee T, et al. X-linked retinitis pigmentosa: mutation spectrum of the RPGR and RP2 genes and correlation with visual function. Investig Ophthalmol Vis Sci 2000;41:2712–2721.
Sharpe L, Stockman A. Rod pathways: the importance of seeing nothing. Trends Neurosci 1999;22:497–504.
Shawkat F, Kriss A. A study of the effects of contrast change on pattern VEPs, and the transition between onset, reversal and offset modes of stimulation. Doc Ophthalmol 2000;101(1):73–89.
Shepherd A, Saunders K, McCulloch D, et al. Prognostic value of flash visual evoked potentials in preterm infants. Dev Med Child Neurol 1999;41:9–155.
Sieving P. Photopic ON-and OFF-pathway abnormalities in retinal dystrophies. AOS thesis. Trans Am Ophthalmol Soc 1993;LXXXXl:701–773.
Sieving P, Murayama K, Naarendorp F. Push-pull model of the primate photopic electroretinogram: a role for hyperpolarizing neurons in shaping the b-wave. Vis Neurosci 1994;11:519–532.
Sieving P, Bingham E, Kemp J, et al. Juvenile X-linked retinoschisis from XLRS1 Arg213Trp mutation with preservation of the electroretinogram scotopic b-wave. Am J Ophthalmol 1999;128(2):179–184.
Sieving P, Frishman L, Steinberg R. Scotopic threshold response of proximal retina in cat. J Neurophysiol 1986;5694:1049–1061.
Sigesmund D, Weleber R, Pillers D, et al. Characterization of the ocular phenotype of Duchenne and Becker muscular dystrophy. Ophthalmology 1994;101(5):856–865.
Skoczenski A, Norcia A. Development of VEP vernier acuity and grating acuity in human infants. Investig Ophthalmol Vis Sci 1999;40:2411–2417.
Smith L, Kriss A, Gregson R, et al. Gaze evoked amaurosis in neuro-fibromatosis type ll. Br J Ophthalmol 1998;82:584–585.
Smith N, Lamb T. The a-wave of the human electroretinogram recorded with a minimally invasive technique. Vision Res 1997;37(21):2943–2952.
Smith R, Berlin C, Hejtmancik J, et al. Clinical diagnosis of the Usher syndromes. Am J Med Genet 1994;50:32–38.
Snead M, Payne S, Barton D, et al. Stickler syndrome: correlation between vitreoretinal phenotypes and linkage to COL 2A1. Eye 1994;8:609–614.
Sokol S. Measurements of infant visual acuity from pattern-reversal evoked potentials. Vision Res 1978;18(1):33–39.
Sokol S, Moskowitz A. Comparison of pattern VEPs and preferential-looking behavior in 3-month-old infants. Investig Ophthalmol Vis Sci 1985;26(3):359–365.
Soong F, Levin A, Westall C. Comparison of techniques for detecting visually evoked potential asymmetry in albinism. J Am Assoc Pediatr Ophthalmol Sci 2000;4:302–310.
Spileers W, Maes H, Van Hulle M, et al. Contrast modulated steady state evoked potentials (CMSS VEPS) measuring static and dynamic contrast sensitivity. Clin Vis Res 1992;7:93–106.
Sridharan R. Visual evoked potentials in spinocerebellar degenerations. Clin Neurol Neurosurg 1983;85:235–243.
Stanesu Segal B, Evrard P. Zellweger syndrome, retinal involvement. Metab Pediatr Syst Ophthalmol 1989;9:96–99.
Stavrou P, Good P, Misson G, et al. Electrophysiological findings in Stargardt’s-fundus flavimaculatus disease. Eye 1998;12 (pt 6):953–958.
Stefan H, Bernatik J, Knorr J. Gesichtsfeldstorungen bei Antiepileptikabehandlung. [Visual field defects due to antiepileptic drugs. Nervenarzt 1999;70(6):552–555.
Strasburger H, Remky A, Murray I, et al. Objective measurement of contrast sensitivity and visual acuity with the steady-state visual evoked potential. Ger J Ophthalmol 1996;5(1):42–52.
Taylor M, McCulloch D. Visual evoked potentials in infants and children. J Clin Neurophysiol 1992;9:357–372.
Taylor M, McCulloch D. The prognostic value of VEPs in young children with acute onset cortical blindness. Pediatr Neurol 1991;7:86–90.
Thompson D, Drasdo N. Temporal patterns and the topography of the visual evoked potential. In: Non-invasive assessment of the visual system, vol 1. Tech digest series. Ophthalmology Society of America, 1992;1:150–153.
Thompson D, Kriss A, Chong K, et al. Visual evoked potential evidence of chiasmal hypoplasia. Ophthalmology 1999;106:2354–2361.
Thompson D, Kriss A, Taylor D, et al. Early VEP and ERG evidence of visual dysfunction in autosomal recessive osteopetrosis. Neuropediatrics 1998;29:137–144.
Thompson D, Kriss A, Cottrell S, et al. Visual evoked potential evidence of albino-like chiasmal misrouting in a patient with Angelman syndrome with no ocular features of albinism. Dev Med Child Neurol 1999;41(9):633–638.
Thompson D, Lloyd I, Dowler J, et al. The development of spatial resolution measured by swept VEP and forced choice preferential looking techniques. Investig Ophthamol Vis Sci 1993;34:1354.
Tolhurst D. Separate channels for the analysis of the shape and movement of a moving visual stimulus. J Physiol 1973;231:385–402.
Tremblay F, De Becker I, Cheung C, et al. Visual evoked potentials with crossed asymmetry in incomplete congenital stationary night blindness. Investig Ophthalmol Vis Sci 1996;37:1783–1792.
Tremblay F, LaRoche R, Shea S, et al. Longitudinal study of the early electroretinographic changes in Alstrom’s syndrome. Am J Ophthalmol 1993;115(5):657–665.
Tremblay F, Laroche R, De-Becker I. The electroretinographic diagnosis of the incomplete form of congenital stationary night blindness. Vision Res 1995;35(16):2383–2393.
Tyler C, Apkarian P, Levi D, et al. Rapid assessment of visual function: an electronic sweep technique for the pattern visual evoked potential. Investig Ophthalmol Vis Sci 1979;18(7):703–713.
Tyler C, Apkarian P, Nakayama K. Multiple spatial-frequency tuning of electrical responses from human visual cortex. Exp Brain Res 1978;33(3–4):535–550.
Tzekov R, Locke K, Hood D, et al. Cone and rod phototransduction parameters in retinitis pigmentosa patients. Investig Ophthalmol Vis Sci 2001;42:420.
van-Lith G, Hekkert-Wiebenga W. Cataract, pattern stimulation and visually evoked potentials. Doc Ophthalmol 1983;28; 55(1–2):107–112.
Vigabatrin Paediatric Advisory Group Guideline for prescribing vigabatrin in children has been revised. Br Med J 2000;320:1404.
Wachtmeister L. Oscillatory potentials in the retina: what do they reveal. Prog Retina Eye Res 1998;17(4):485–521.
Weleber R, Kennaway N. Infantile Refsum’s disease. In: Gold DH, Weingeist TA (eds) The eye in systemic disease. Philadelphia: Lippincott, 1990:409–411.
Weleber R. The dystrophic retina in multisystem disorders: the electroretinogram in neuronal ceroid lipofuscinoses. Eye 1998;12 (pt 3b):580–590.
Wendel R, Mannis M, Keltner J. Role of electrophysiologic testing in the preoperative evaluation of corneal transplant patients. Ann Ophthalmol 1984;16(8):788–793.
Wenzel D, Brandl U. Maturation of pattern evoked potentials elicited by checkerboard reversal. Dev Ophthalmol 1984;9:87–93.
Westall C, Paton C, Levin A. Time courses for maturation of electroretinogram responses from infancy to adulthood. Doc Ophthalmol 1999;96:355–379.
Wild J, Martinez C, Reinshagen G, et al. Characteristics of a unique visual field defect attributed to vigabatrin. Epilepsia 1999;40(12):1784–1794.
Wilkie A. Craniosynostosis: genes and mechanisms. Hum Mol Genet 1997;6:1647–1656.
Willison H, Muller D, Matthews S, et al. A study of the relationship between neurological function and serum vitamin E concentrations in patients with cystic fibrosis. J Neurol Neurosurg Psychiatry 1985;48:1097–1102.
Wilson W. Peroxisomal disorders. In: Gold DH, Weingeist TA (eds) The eye in systemic disease. Philadelphia: Lippincott, 1990:402–406.
Wohlrab G, Boltshauser E, Schmitt B, et al. Visual field constriction is not limited to children treated with vigabatrin. Neuropediatrics 1999;30(3):130–132.
Wright K, Eriksen K, Shors T. Recording pattern evoked potentials under chloral hydrate sedation. Arch Ophthalmol 1986;104:718–721.
Wright K, Eriksen K, Shors T. Detection of amblyopia with P-VEP during chloral hydrate sedation. J Pediatr Ophthalmol Strabismus 1987;24(4):170–175.
Wright K, Fox B, Shors T, Eriksen K. The use of the PVEP with multiple large check stimuli for quantitating amblyopia in children. Binoc Vis Q 1990;5(1):19–26.
Wright K, Ary J, Shors T, Eriksen K. J Pediatr Ophthalmol Strabismus 1986;23(5):252–257.
Wright K, Fox B, Eriksen K. PVEP evidence of true suppression in adult onset strabismus. J Pediatr Ophthalmol Strabismus 1990;27(4):196–201.
Yiannikas C, Walsh J, McCleod J. Visual evoked potentials in the detection of sub-clinical optic toxic effects secondary to ethambutol. Arch Neurol 1983;40:645–648.
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2006 Springer Science+Business Media, Inc.
About this chapter
Cite this chapter
Kriss, A., Thompson, D. (2006). Pediatric Visual Electrophysiology. In: Wright, K.W., Spiegel, P.H., Thompson, L.S. (eds) Handbook of Pediatric Retinal Disease. Springer, New York, NY. https://doi.org/10.1007/0-387-27933-4_1
Download citation
DOI: https://doi.org/10.1007/0-387-27933-4_1
Publisher Name: Springer, New York, NY
Print ISBN: 978-0-387-27932-9
Online ISBN: 978-0-387-27933-6
eBook Packages: MedicineMedicine (R0)