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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Retinal nerve fiber layer evaluation by optical coherence tomography in Leber's hereditary optic neuropathy.

PURPOSE: To study retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (StratusOCT) in patients with Leber's hereditary optic neuropathy (LHON). DESIGN: Cross-sectional study. PARTICIPANTS AND/OR CONTROLS: Thirty-eight patients with LHON were analyzed and compared with an age-matched control group of 75 patients. Patients with LHON were classified as having early LHON (E-LHON, n = 8) when the duration of the disease was shorter than 6 months and atrophic LHON (A-LHON, n = 30) when the duration was longer than 6 months. METHODS: The fast RNFL thickness (3.4) scan acquisition protocol was used. MAIN OUTCOME MEASURE: Retinal nerve fiber layer thickness as measured by StratusOCT. RESULTS: Compared with the control group, eyes with E-LHON showed a thicker RNFL in the 360 degrees average measurement (P<0.01) and in the superior (P<0.01), nasal (P<0.05), and inferior quadrants (P<0.05); no significant changes were detected in the temporal quadrant. Eyes with A-LHON revealed a thinner RNFL in all measurements (P<0.001); the fibers of the nasal quadrant showed the lowest amount of reduction (38% vs. 42%-49.8% in the other quadrants). In cases with A-LHON and visual recovery, RNFL was significantly thicker in all measurements (P<0.001), except the temporal quadrant, with respect to A-LHON without visual recovery. CONCLUSIONS: On the basis of OCT data, the RNFL is thickened in E-LHON and severely thinned in A-LHON. RNFL is likely to be partially preserved in A-LHON with visual recovery. The temporal fibers (papillomacular bundle) are the first and most severely affected; the nasal fibers seem to be partially spared in the late stage of the disease.[1]


  1. Retinal nerve fiber layer evaluation by optical coherence tomography in Leber's hereditary optic neuropathy. Barboni, P., Savini, G., Valentino, M.L., Montagna, P., Cortelli, P., De Negri, A.M., Sadun, F., Bianchi, S., Longanesi, L., Zanini, M., de Vivo, A., Carelli, V. Ophthalmology (2005) [Pubmed]
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