May 2004
Volume 45, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2004
Abnormal multifocal ERG and VEP responses in carriers of 11778 LHON
Author Affiliations & Notes
  • E.E. Sutter
    Smith–Kettlewell Eye Research, San Francisco, CA
  • A. Berezovsky
    Institute of Vision, Federal University of Sao Paulo, Sao Paulo, Brazil
  • S. Rios Salomao
    Institute of Vision, Federal University of Sao Paulo, Sao Paulo, Brazil
  • J. Pereira
    Institute of Vision, Federal University of Sao Paulo, Sao Paulo, Brazil
  • A.A. Sadun
    Doheny Eye Institute, Los Angeles, CA
  • Footnotes
    Commercial Relationships  E.E. Sutter, Electro–Diagnostic Imaging, Inc. P; A. Berezovsky, None; S. Rios Salomao, None; J. Pereira, None; A.A. Sadun, None.
  • Footnotes
    Support  EY06861, IFOND
Investigative Ophthalmology & Visual Science May 2004, Vol.45, 1015. doi:
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      E.E. Sutter, A. Berezovsky, S. Rios Salomao, J. Pereira, A.A. Sadun; Abnormal multifocal ERG and VEP responses in carriers of 11778 LHON . Invest. Ophthalmol. Vis. Sci. 2004;45(13):1015.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Abstract: : Purpose: To conduct a comprehensive electrophysiologic investigation of members of a 11778 LHON pedigree using multifocal VEP and ERG techniques. Methods: All multifocal records were collected and analyzed with the VERIS 5.1 multifocal recording system. Fixation was monitored throughout data collection using an IR fundus camera. MfERGs were collected from 11 asymptomatic carriers, two affected patients and 26 normal subjects. Stimulation: A special ganglion cell response enhancing protocol was used. It consisted of multifocal flash stimuli (base interval 67 ms) interleaved with two global flashes presented 13.3 ms and 40 ms after each multifocal frame. The intensity of multifocal and global flashes was (2.7 cdos/m2). The stimulus array consisted of 103 scaled hexagons. Recording time was 9 minutes per eye. Pupils were dilated. MfVEPs were recorded from both eyes of 29 asymptomatic carriers and 4 affected patients. The mfVEP stimulus consisted of a 60–sector dartboard grid with each sector containing a contrast reversing check pattern. The mean stimulus luminance was 200 cd/m^2. Pupils were natural. All multifocal stimulus arrays subtended ca. 45 deg. The net recording time was 7 minutes per eye. Analysis: The effect induced by the focal flashes on the second one of the following global flashes contains a prominent signal contribution from ganglion cell fibers near the optic nerve head where they become myelinated (Optic Nerve Head Component or ONHC). This response contribution was used to evaluation ganglion cell function by means of the mfERG. The analysis of the mfVEP records included implicit time measurements of the focal response components as well as an evaluation by mapping focal inter–ocular amplitude ratios. Results: MfERG: The two affected patients showed almost no detectable ONHC except for some very minor regional sparing. Three carriers exhibited severe diffuse losses while in four others the losses were regional. Minor localized anomalies were seen in three carriers and one was judged normal. MfVEP: All of the affected showed severely depressed central responses. Responses in Nine other carriers also were centrally depressed. Nine carriers exhibited abnormal inter–ocular asymmetries and two showed abnormal inter–ocular differences in implicit times. The remaining carriers were in the normal range. Conclusions: Both mfERG and mfVEP records detected substantial local abnormalities in many of the unaffected carriers. MfERG abnormalities did not always translate into abnormal mfVEP.

Keywords: electroretinography: clinical 
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