Electroretinographic oscillatory potentials in Leber hereditary optic neuropathy

Barboni, Mirella T. S. (author), Šuštar Habjan, Maja (author), Petrović Pajić, Sanja (author), Hawlina, Marko (author)

Purpose Leber hereditary optic neuropathy (LHON) affects retinal ganglion cells causing severe vision loss. Pattern electroretinogram and photopic negative response (PhNR) of the light-adapted (LA) full-field electroretinogram (ERG) are typically affected in LHON. In the present study, we evaluated dark-adapted (DA) and LA oscillatory potentials (OPs) of the flash ERG in genetically characterized LHON patients to dissociate slow from fast components of the response. Methods Seven adult patients (mean age = 28.4 ± 5.6) in whom genetic diagnosis confirmed LHON with mtDNA or nuclear DNAJC30 (arLHON) pathogenic variants were compared to 12 healthy volunteers (mean age = 35.0 ± 12.1). Full-field ERGs were recorded from both eyes. Offline digital filters at 50, 75 and 100 Hz low cutoff frequencies were applied to isolate high-frequency components from the original ERG signals. Results ERG a-waves and b-waves were comparable between LHON patients and controls, while PhNR was significantly reduced (p = 0.009) in LHON patients compared to controls, as expected. OPs derived from DA signals (75 Hz low cutoff frequency) showed reduced peak amplitude for OP2 (p = 0.019). LA OP differences between LHON and controls became significant (OP2: p = 0.047, OP3: p = 0.039 and OP4: p = 0.013) when the 100 Hz low-cutoff frequency filter was applied. Conclusions Reduced OPs in LHON patients may represent disturbed neuronal interactions in the inner retina with preserved photoreceptoral (a-wave) to bipolar cell (b-wave) activation. Reduced DA OP2 and high-cutoff LA OP alterations may be further explored as functional measures to characterize LHON status and progression.

• Language: English
• Keywords: LHON, retina, electroretinogram, photopic negative response, oscillatory potentials
• Work type: Article
• Typology: 1.01 - Original Scientific Article
• Organization: MF - Faculty of Medicine
• Publication status: Published
• Publication version: Version of Record
• Year: 2024
• Number of pages: Str. 133-143
• Numbering: Vol. 148, iss. 3
• PID: 20.500.12556/RUL-179116
• ISSN on article: 1573-2622
• DOI: 10.1007/s10633-024-09968-9
• Publication date in RUL: 05.02.2026

Record is a part of a journal

• Title: Documenta ophthalmologica
• Shortened title: Doc. ophthalmol.
• Publisher: Springer Nature
• ISSN: 1573-2622
• COBISS.SI-ID: 513144601

Licences

• License: CC BY 4.0, Creative Commons Attribution 4.0 International
• Link: http://creativecommons.org/licenses/by/4.0/
• Description: This is the standard Creative Commons license that gives others maximum freedom to do what they want with the work as long as they credit the author.

Projects

• Funder: ARRS - Slovenian Research Agency
• Project number: P3-0333
• Name: Očesne bolezni odraslih in otrok

• Funder: Hungary, National Research, Development, and Innovation Fund
• Funding programme: OTKA-PD
• Project number: 134799