Advanced search
Start date
(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Correlations Between Dark-Adapted Rod Threshold Elevations and ERG Response Deficits in Duchenne Muscular Dystrophy

Full text
Salgueiro Barboni, Mirella Telles [1, 2] ; Dias, Sarah Leonardo [1] ; Silva, Leonardo Aparecido [1] ; Damico, Francisco Max [1, 3] ; Vidal, Kallene Summer [1] ; Costa, Marcelo Fernandes [1] ; Nagy, Balazs Vince [4] ; Kremers, Jan [5] ; Ventura, Dora Fix [1]
Total Authors: 9
[1] Univ Sao Paulo, Inst Psychol, Dept Expt Psychol, Sao Paulo - Brazil
[2] Semmelweis Univ, Dept Ophthalmol, Maria U 39, H-1085 Budapest - Hungary
[3] Univ Sao Paulo, Fac Med, Dept Ophthalmol, Sao Paulo - Brazil
[4] Budapest Univ Technol & Econ, Dept Mechatron Opt & Mech Engn Informat, Budapest - Hungary
[5] Univ Hosp Erlangen, Sect Retinal Physiol, Erlangen - Germany
Total Affiliations: 5
Document type: Journal article
Web of Science Citations: 0

PURPOSE. The purpose of this study was to characterize changes in the full-field flash electroretinogram (ERG) in association with psychophysical dark-adapted visual thresholds in patients with genetically characterized Duchenne muscular dystrophy (DMD) either lacking Dp427 (Up 30) or at least Dp260 in addition to Dp427 (Down 30). METHODS. Twenty-one patients with DMD and 27 age-similar controls participated in this study. Dark-adapted (0.01, 3.0, and 10 cd.s/m(2) flashes) and light-adapted (3.0 cd.s/m(2) flash) ERGs were recorded following International Society for Clinical Electrophysiology of Vision (ISCEV) standard protocols. Visual detection thresholds to 625-nm (cone function) and 527-nm (rod function) light-emitting diode (LED) flashes (2 degree diameter) were measured during a dark adaptation period after a 1-minute exposure to a bleaching light (3000 cd/m(2)). Initially, 8 minutes of interleaved 625-nm and 527-nm thresholds were measured. After an additional 5 minutes of dark-adaptation, a second set of threshold measurements to 527-nm stimuli was performed during the subsequent 6 minutes. RESULTS. Dark-adapted b-wave amplitude was significantly reduced to all strengths of flash and a-wave in response to the strong flash stimulus was delayed (15.6 vs. 14.7 ms, P < 0.05) in patients with Down 30 compared with controls. Dark-adapted cone thresholds did not differ among the groups (-2.0, -1.8, and -1.7 log cd/m(2) for Down 30, Up 30, and controls, respectively, P = 0.21). In contrast, dark-adapted rod thresholds were elevated (F( 2,36) = 8.537, P = 0.001) in patients with Down 30 (mean = -3.2 +/- 1.1 log cd/m(2)) relative to controls (mean = -4.2 +/- 0.3 log cd/m(2)). Dark-adapted b-wave amplitudes were correlated with dark-adapted rod sensitivity in patients with DMD (Spearman Rho = 0.943, P = 0.005). The changes were much smaller or absent in patients with intact Dp260. CONCLUSIONS. Dp260 is particularly required for normal rod-system function in dark adaptation. (AU)

FAPESP's process: 14/26818-2 - Development and implementation of visual evaluation methods: clinical applications and animal models
Grantee:Dora Selma Fix Ventura
Support type: Research Projects - Thematic Grants
FAPESP's process: 17/16948-4 - Impact of different types of modification of the gene for dystrophin in the vision of patients with muscular dystrophy
Grantee:Leonardo Aparecido Silva
Support type: Scholarships in Brazil - Master
FAPESP's process: 15/22227-2 - Electrophysiological and psychophysical evaluation of visual functions: a comparative study in Alzheimer's disease, glaucomatous neuropathy and age-related macular degeneration
Support type: Scholarships in Brazil - Post-Doctorate
FAPESP's process: 16/04538-3 - Dystrophin and the human visual system: study of the visual phenotypes of Duchenne muscular dystrophy patients
Grantee:Dora Selma Fix Ventura
Support type: Regular Research Grants