The objective of this study was to describe the changes in the retinal ganglion cell complex (GCC) relative to the retinal nerve fibre layer (RNFL) over time, in patients with Leber’s hereditary optic neuropathy (LHON). For this study, 21 patients with LHON were evaluated and a profile of OCT changes emerged. Of the 21 patients, seven met the inclusion criteria. The sequence of GCC and RNFL changes on OCT were observed throughout the presymptomatic, acute and chronic phases of the optic neuropathy. Average RNFL and GCC thickness was measured in the included seven patients in the early acute, late acute and chronic phases. Patients showed thinning of the GCC and RNFL swelling in the early acute phase. GCC thinning became severe within weeks and persisted. These GCC changes appear at the commencement of visual loss, in some cases prior to vision loss. Detection of the profile of ganglion cell complex thinning along with RNFL swelling on OCT in the unaffected eye after the first eye is symptomatic is typical of LHON. This finding may aid in early diagnosis and provide an opportunity for early intervention and treatment. 

The optical coherence tomography profile of Leber hereditary optic neuropathy.
Hedges TR, Gobuty M, Manfready RA, et al.
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Claire Howard

Salford Royal NHS Foundation Trust, Salford, UK.

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