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Optical Coherence Tomography: Imaging Mouse Retinal Ganglion Cells In Vivo
Published on: September 22, 2017
Secondary post-geniculate involvement in Leber's hereditary optic neuropathy
Giovanni Rizzo1, Kevin R Tozer, Caterina Tonon
1Department of Biomedical and NeuroMotor Sciences (DiBiNeM), University of Bologna, Bologna, Italy.
Plos One
|December 5, 2012
Summary
Leber's hereditary optic neuropathy (LHON) causes retinal ganglion cell degeneration. This study reveals secondary damage in the post-geniculate visual pathway, not primary mitochondrial dysfunction, in LHON patients.
Area of Science:
- Neuroscience
- Ophthalmology
- Genetics
Background:
- Leber's hereditary optic neuropathy (LHON) primarily affects the optic nerve, leading to retinal ganglion cell (RGC) degeneration.
- The extent of post-geniculate visual pathway involvement in LHON pathogenesis remains unclear.
Purpose of the Study:
- To investigate the involvement of the post-geniculate visual pathway in LHON patients using in vivo and post-mortem analyses.
- To differentiate between primary neurodegeneration and secondary de-afferentation in the affected visual pathway.
Main Methods:
- Diffusion-weighted imaging (DWI) was used to assess mean diffusivity in the optic radiation (OR) of LHON patients, carriers, and controls.
- Post-mortem analysis of optic nerve and lateral geniculate nucleus (LGN) was performed in a severe LHON case and a control.
Main Results:
- Increased mean diffusivity in the optic radiation (OR) was observed in LHON patients compared to controls and carriers, correlating with disease duration and visual acuity loss.
- Post-mortem examination revealed significant atrophy and degeneration in the LGN and severe axonal loss in the optic nerve of the LHON patient.
Conclusions:
- The findings suggest that post-geniculate visual pathway involvement in LHON is a secondary phenomenon resulting from de-afferentation due to optic nerve damage.
- This contrasts with a primary neurodegenerative process within the LGN driven by mitochondrial dysfunction.
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