| Title: | Electroretinographic oscillatory potentials in Leber hereditary optic neuropathy |
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| Authors: | ID Barboni, Mirella T. S. (Author)
ID Šuštar Habjan, Maja (Author)
ID Petrović Pajić, Sanja (Author)
ID Hawlina, Marko (Author) |
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| Files: |  PDF - Presentation file, download (832,89 KB)
MD5: 4BACEC256CAAD550044C7647BAEB7000
 URL - Source URL, visit https://link.springer.com/article/10.1007/s10633-024-09968-9
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| Language: | English |
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| Typology: | 1.01 - Original Scientific Article |
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| Organization: |  UKC LJ - Ljubljana University Medical Centre
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| Abstract: | 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. |
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| Keywords: | LHON, electroretinogram, oscillatory potentials, retina, photopic negative response |
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| Publication status: | Published |
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| Publication version: | Version of Record |
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| Year of publishing: | 2024 |
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| Number of pages: | str. 133-143 |
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| Numbering: | Vol. 148, no. 3 |
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| PID: | 20.500.12556/DiRROS-27695  |
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| UDC: | 617.7 |
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| ISSN on article: | 0012-4486 |
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| DOI: | 10.1007/s10633-024-09968-9 |
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| COBISS.SI-ID: | 267986691 |
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| Publication date in DiRROS: | 19.02.2026 |
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| Views: | 167 |
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| Downloads: | 48 |
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