title: Retinal Ganglion Cell and Inner Plexiform Layer Loss Correlate with Visual Acuity Loss in LHON: A Longitudinal, Segmentation OCT Analysis.
abstract: OBJECTIVE
  Describe changes in the retina as vision loss progresses in Leber's Hereditary Optic Neuropathy (LHON) using spectral-domain optical coherence tomography (SD-OCT) autosegmentation, and determine if relationship exists between retinal changes and vision loss.
  
  
  METHODS
  From patient records we identified nine LHON patients who underwent periodic neuro-ophthalmologic examinations and high-resolution SD-OCT as part of their care. We describe the impact of LHON progression on each retinal layer, and the relationship between these structural changes and visual acuity using generalized estimating equations and nonparametric tests.
  
  
  RESULTS
  The thickness of the ganglion cell layer (GCL) and inner plexiform layer (IPL) decreased immediately or soon after symptom onset, and this decrease was associated with worsening vision: in the GCL a 1-mm3 volume loss was associated with a 3.2 increase in logMAR visual acuity (95% confidence interval [CI]: 2.1-4.1); in the IPL a 1-mm3 volume loss was associated with a 4.9 increase in visual acuity (95%CI: 6.5-3.2). The retinal nerve fiber layer (RNFL) also thinned, but not until after the GCL and IPL, and only in the papillomacular bundle (PMB) and temporal layers was thinning associated with vision loss.
  
  
  CONCLUSIONS
  For the first time these analyses describe a structure-function relationship between the retinal changes that occur in LHON patients as their disease progresses and vision worsens. The structural changes in the GCL, IPL, and RNFL preceded structural changes in the other retinal layers. This analysis suggests that the first 6 months after diagnosis define a target for therapeutic intervention, and this can inform treatment guidelines for ongoing therapeutic trials.
authors.affiliation: Neuro-Ophthalmology Department Wills Eye Hospital, Departments of Ophthalmology and Neurology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, United States.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Moster
authors.affiliation: Neuro-Ophthalmology Department Wills Eye Hospital, Departments of Ophthalmology and Neurology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, United States.
authors.forename: Mark L
authors.initials: ML
authors.lastname: Moster
authors.affiliation: Department of Public Health Sciences, Biological Sciences Division, University of Chicago, Chicago, Illinois, United States.
authors.forename: Molly
authors.initials: M
authors.lastname: Scannell Bryan
authors.affiliation: Neuro-Ophthalmology Department Wills Eye Hospital, Departments of Ophthalmology and Neurology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, United States.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Sergott
mesh_terms: D000328:Adult; D018450:Disease Progression; D006801:Humans; D008137:Longitudinal Studies; D008266:Macula Lutea / Q000473:pathology / Q000502:physiology; D009412:Nerve Fibers / Q000473:pathology / Q000502:physiology; D029242:Optic Atrophy, Hereditary, Leber / Q000473:pathology* / Q000503:physiopathology; D009929:Organ Size; D012165:Retinal Ganglion Cells / Q000473:pathology* / Q000502:physiology; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity / Q000502:physiology