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Hereditary Congenital Nystagmus: An Intrafamilial Study

Hereditary Congenital Nystagmus: An Intrafamilial Study Abstract Three members of the same family with hereditary congenital nystagmus (CN) were studied. Nystagmus amplitude, frequency, and intensity functions determined the gaze angle with least nystagmus. Visual acuity was increased in all cases by the use of version or composite prisms. In both pendular and jerk forms of CN, the fovea oscillated on alternate sides of the fixation spot and exhibited simultaneous bilateral shifts in the resulting fixation bias. The nystagmus waveforms were often complex and required velocity information for distinction of type and direction. The "attempt" to fixate was a driving stimulus for CN and ambient illumination or eyelid position were unrelated to its genesis. Pendular and jerk nystagmus are different manifestations of the same ocular motor instability and simple classification on the basis of waveform is erroneous when used to infer etiology. References 1. Dell'Osso LF, et al: Eye movement recordings as a diagnostic tool in a case of congenital nystagmus . Am J Optom 49:3-13, 1972.Crossref 2. Dell'Osso LF: Fixation characteristics in hereditary congenital nystagmus . Am J Optom 50:85-90, 1973.Crossref 3. Young LR: Measuring eye movements . Am J Med Electronics 2:300-307, 1963. 4. Weber RB, Daroff RB: The metrics of horizontal saccadic eye movements in normal humans . Vision Res 11:921-928, 1971.Crossref 5. Kestenbaum A: Frequenz und Amplitude des Nystagmus . Grafe Arch Ophthalmol 114:550-582, 1924. 6. Kestenbaum A: Clinical Methods of Neuro-Ophthalmologic Examination. New York, Gruneand Stratton, 1961, pp 335-400. 7. Jung R, Kornhuber HH: Results of electronystagmography in man: The value of optokinetic, vestibular, and spontaneous nystagmus for neurologic diagnosis and research , in Bender MB (ed): The Oculomotor System. New York, Harper and Row, 1964, chap 19, pp 428-488. 8. Zuber BL, Stark L, Cook G: Microsaccades and the velocity-amplitude relationship for saccadic eye movements . Science 150:1459-1460, 1965.Crossref 9. Forsmann B: A study of congenital nystagmus . Acta Otolaryngol 57:427-449, 1964.Crossref 10. Pratt RTC: The Genetics of Neurological Disorders . London, Oxford University Press, 1968, chap 4 , p 31. 11. Cogan DG: Congenital nystagmus . Can J Ophthalmol 2:4-10, 1967. 12. Anderson JR: The treatment of congenital nystagmus , in: Ocular Vertical Deviations and the Treatment of Nystagmus , ed 2. Philadelphia, J B Lippincott Co, 1959, chap 10, pp 165-172. 13. Dell'Osso LF: Improving visual acuity in congenital nystagmus , in Smith JL, Glaser JS (eds): Neuro-Ophthalmology . St. Louis, CV Mosby Co, 1973, chap 9, pp 98-106. 14. Anderson JR: Causes and treatment and therapy of congenital eccentric nystagmus . Br J Ophthalmol 37:267-281, 1953.Crossref 15. Kestenbaum A: A nystagmus operation . Acta Concilium Ophthalmologicum 2:1071-1078, 1955. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Hereditary Congenital Nystagmus: An Intrafamilial Study

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Publisher
American Medical Association
Copyright
Copyright © 1974 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1974.01010010378002
Publisher site
See Article on Publisher Site

Abstract

Abstract Three members of the same family with hereditary congenital nystagmus (CN) were studied. Nystagmus amplitude, frequency, and intensity functions determined the gaze angle with least nystagmus. Visual acuity was increased in all cases by the use of version or composite prisms. In both pendular and jerk forms of CN, the fovea oscillated on alternate sides of the fixation spot and exhibited simultaneous bilateral shifts in the resulting fixation bias. The nystagmus waveforms were often complex and required velocity information for distinction of type and direction. The "attempt" to fixate was a driving stimulus for CN and ambient illumination or eyelid position were unrelated to its genesis. Pendular and jerk nystagmus are different manifestations of the same ocular motor instability and simple classification on the basis of waveform is erroneous when used to infer etiology. References 1. Dell'Osso LF, et al: Eye movement recordings as a diagnostic tool in a case of congenital nystagmus . Am J Optom 49:3-13, 1972.Crossref 2. Dell'Osso LF: Fixation characteristics in hereditary congenital nystagmus . Am J Optom 50:85-90, 1973.Crossref 3. Young LR: Measuring eye movements . Am J Med Electronics 2:300-307, 1963. 4. Weber RB, Daroff RB: The metrics of horizontal saccadic eye movements in normal humans . Vision Res 11:921-928, 1971.Crossref 5. Kestenbaum A: Frequenz und Amplitude des Nystagmus . Grafe Arch Ophthalmol 114:550-582, 1924. 6. Kestenbaum A: Clinical Methods of Neuro-Ophthalmologic Examination. New York, Gruneand Stratton, 1961, pp 335-400. 7. Jung R, Kornhuber HH: Results of electronystagmography in man: The value of optokinetic, vestibular, and spontaneous nystagmus for neurologic diagnosis and research , in Bender MB (ed): The Oculomotor System. New York, Harper and Row, 1964, chap 19, pp 428-488. 8. Zuber BL, Stark L, Cook G: Microsaccades and the velocity-amplitude relationship for saccadic eye movements . Science 150:1459-1460, 1965.Crossref 9. Forsmann B: A study of congenital nystagmus . Acta Otolaryngol 57:427-449, 1964.Crossref 10. Pratt RTC: The Genetics of Neurological Disorders . London, Oxford University Press, 1968, chap 4 , p 31. 11. Cogan DG: Congenital nystagmus . Can J Ophthalmol 2:4-10, 1967. 12. Anderson JR: The treatment of congenital nystagmus , in: Ocular Vertical Deviations and the Treatment of Nystagmus , ed 2. Philadelphia, J B Lippincott Co, 1959, chap 10, pp 165-172. 13. Dell'Osso LF: Improving visual acuity in congenital nystagmus , in Smith JL, Glaser JS (eds): Neuro-Ophthalmology . St. Louis, CV Mosby Co, 1973, chap 9, pp 98-106. 14. Anderson JR: Causes and treatment and therapy of congenital eccentric nystagmus . Br J Ophthalmol 37:267-281, 1953.Crossref 15. Kestenbaum A: A nystagmus operation . Acta Concilium Ophthalmologicum 2:1071-1078, 1955.

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Nov 1, 1974

References