April 2011
Volume 52, Issue 14
Free
ARVO Annual Meeting Abstract  |   April 2011
On and Off ERGs in Duchenne Muscular Dystrophy Patients with Deletion of Dystrophin Isoform Dp260
Mirella T. Barboni; Balázs V. Nagy; Ana Laura A. Moura; Francisco M. Damico; Marcelo F. Costa; Jan Kremers; Dora F. Ventura
Author Affiliations & Notes
  • Mirella T. Barboni
    Neurosciences and Behavior and Department of Experimental Psychology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Balázs V. Nagy
    Neurosciences and Behavior and Department of Experimental Psychology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Ana Laura A. Moura
    Neurosciences and Behavior and Department of Experimental Psychology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Francisco M. Damico
    Neurosciences and Behavior and Department of Experimental Psychology,
    Department of Ophthalmology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Marcelo F. Costa
    Neurosciences and Behavior and Department of Experimental Psychology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Jan Kremers
    Department of Ophthalmology, University of Erlangen, Erlangen, Germany
  • Dora F. Ventura
    Neurosciences and Behavior and Department of Experimental Psychology,
    University of Sao Paulo, Sao Paulo, Brazil
  • Footnotes
    Commercial Relationships  Mirella T. Barboni, None; Balázs V. Nagy, None; Ana Laura A. Moura, None; Francisco M. Damico, None; Marcelo F. Costa, None; Jan Kremers, None; Dora F. Ventura, None
  • Footnotes
    Support  MTSB and ALM had doctoral scholarship (FAPESP 07/55125-1 and 08/52427-0). BVN and FMD had post doctoral scholarship (FAPESP 09/54292-7, CNPq 105614/2009-8). FAPESP grants: 02/12733-8 and 08/58731-2. G
Investigative Ophthalmology & Visual Science April 2011, Vol.52, 3513. doi:
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      Mirella T. Barboni, Balázs V. Nagy, Ana Laura A. Moura, Francisco M. Damico, Marcelo F. Costa, Jan Kremers, Dora F. Ventura; On and Off ERGs in Duchenne Muscular Dystrophy Patients with Deletion of Dystrophin Isoform Dp260. Invest. Ophthalmol. Vis. Sci. 2011;52(14):3513.

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Abstract

Purpose: : Dystrophin is required for normal electrophysiology of the retina. However its specific function is not yet well established, its absence may affect ON and OFF pathways differently. This study investigated ON and OFF pathways in Duchenne muscular dystrophy (DMD) patients using full field ERG to evaluate possible asymmetric retinal alterations associated with the deletion of the dystrophin retinal isoform Dp260.

Methods: : ERGs were obtained from one dilated eye of eight DMD patients (mean age = 17 ± 5 years) who underwent previous ophthalmologic examination. 4 Hz sawtooth (rapid-ON and rapid-OFF) luminance modulation of white LEDs was used at mesopic (1 cd/m²) and photopic (250 cd/m²) conditions. In addition, standard flash ERGs were measured at dark-adapted (0.09 cd/m²) and bright-adapted (28 cd/m²) conditions. The analysis was carried out in the time domain in which amplitude and implicit time of the 1st negative and the 1st positive averaged peak was measured. The results were compared with an age-matched control group.

Results: : DMD patients had reduced b-wave amplitudes both for the dark-adapted and for the bright-adapted standard flash ERGs (p<0.04). For the dark-adapted standard flash ERG they also had increased b-wave implicit times (p<0.01). For the ON protocol DMD patients had reduced amplitudes in both mesopic and photopic positive peaks (p<0.02). For the OFF protocol DMD patients had reduced amplitudes in the mesopic responses (p<0.02) but not in the photopic responses (p>0.05).

Conclusions: : Since the Dp260 is located in the invaginated part of the synaptic terminal of the photoreceptors where both rods and cones connect to ON bipolar cells, its deletion might explain the reduced ERG amplitudes found in these patients. The reduction has been detected for both ON and OFF protocols in the mesopic condition, as the rod-to-ON bipolar cell signals are sent to both ON and OFF pathways via AII cells, and for the ON protocol in the photopic condition in which the ON pathway is affected exclusively.

Keywords: electrophysiology: clinical • electroretinography: clinical • retina 
© 2011, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. Permission to republish any abstract or part of an abstract in any form must be obtained in writing from the ARVO Office prior to publication.
Copyright © 2024 Association for Research in Vision and Ophthalmology.
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