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Optical coherence tomography identifies outer retina thinning in frontotemporal degeneration.

ABSTRACT: OBJECTIVE:Whereas Alzheimer disease (AD) is associated with inner retina thinning visualized by spectral-domain optical coherence tomography (SD-OCT), we sought to determine if the retina has a distinguishing biomarker for frontotemporal degeneration (FTD). METHODS:Using a cross-sectional design, we examined retinal structure in 38 consecutively enrolled patients with FTD and 44 controls using a standard SD-OCT protocol. Retinal layers were segmented with the Iowa Reference Algorithm. Subgroups of highly predictive molecular pathology (tauopathy, TAR DNA-binding protein 43, unknown) were determined by clinical criteria, genetic markers, and a CSF biomarker (total tau: ?-amyloid) to exclude presumed AD. We excluded eyes with poor image quality or confounding diseases. SD-OCT measures of patients (n = 46 eyes) and controls (n = 69 eyes) were compared using a generalized linear model accounting for intereye correlation, and correlations between retinal layer thicknesses and Mini-Mental State Examination (MMSE) were evaluated. RESULTS:Adjusting for age, sex, and race, patients with FTD had a thinner outer retina than controls (132 vs 142 ?m, p = 0.004). Patients with FTD also had a thinner outer nuclear layer (ONL) (88.5 vs 97.9 ?m, p = 0.003) and ellipsoid zone (EZ) (14.5 vs 15.1 ?m, p = 0.009) than controls, but had similar thicknesses for inner retinal layers. The outer retina thickness of patients correlated with MMSE (Spearman r = 0.44, p = 0.03). The highly predictive tauopathy subgroup (n = 31 eyes) also had a thinner ONL (88.7 vs 97.4 ?m, p = 0.01) and EZ (14.4 vs 15.1 ?m, p = 0.01) than controls. CONCLUSIONS:FTD is associated with outer retina thinning, and this thinning correlates with disease severity.

SUBMITTER: Kim BJ

PROVIDER: S-EPMC5634666 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature

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Optical coherence tomography identifies outer retina thinning in frontotemporal degeneration.

Kim Benjamin J BJ Irwin David J DJ Song Delu D Daniel Ebenezer E Leveque Jennifer D JD Raquib Aaishah R AR Pan Wei W Ying Gui-Shuang GS Aleman Tomas S TS Dunaief Joshua L JL Grossman Murray M

Neurology 20170908 15

<h4>Objective</h4>Whereas Alzheimer disease (AD) is associated with inner retina thinning visualized by spectral-domain optical coherence tomography (SD-OCT), we sought to determine if the retina has a distinguishing biomarker for frontotemporal degeneration (FTD).<h4>Methods</h4>Using a cross-sectional design, we examined retinal structure in 38 consecutively enrolled patients with FTD and 44 controls using a standard SD-OCT protocol. Retinal layers were segmented with the Iowa Reference Algori ...[more]

PMID: 28887373

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Project description:BackgroundRecently, a transgenic rabbit with rhodopsin Pro 347 Leu mutation was generated as a model of retinitis pigmentosa (RP), which is characterized by a gradual loss of vision due to photoreceptor degeneration. The purpose of the current study is to noninvasively visualize and assess time-dependent changes in the retinal structures of a rabbit model of retinal degeneration by using speckle noise-reduced spectral-domain optical coherence tomography (SD-OCT).Methodology/principal findingsWild type (WT) and RP rabbits (aged 4-20 weeks) were investigated using SD-OCT. The total retinal thickness in RP rabbits decreased with age. The thickness of the outer nuclear layer (ONL) and between the external limiting membrane and Bruch's membrane (ELM-BM) were reduced in RP rabbits around the visual streak, compared to WT rabbits even at 4 weeks of age, and the differences increased with age. However, inner nuclear layer (INL) thickness in RP rabbits did not differ from that of WT during the observation period. The ganglion cell complex (GCC) thickness in RP rabbits increased near the optic nerve head but not around the visual streak in the later stages of the observation period. Hyper-reflective change was widely observed in the inner segments (IS) and outer segments (OS) of the photoreceptors in the OCT images of RP rabbits. Ultrastructural findings in RP retinas included the appearance of small rhodopsin-containing vesicles scattered in the extracellular space around the photoreceptors.Conclusions/significanceIn the current study, SD-OCT provided the pattern of photoreceptor degeneration in RP rabbits and the longitudinal changes in each retinal layer through the evaluation of identical areas over time. The time-dependent changes in the retinal structure of RP rabbits showed regional and time-stage variations. In vivo imaging of RP rabbit retinas by using SD-OCT is a powerful method for characterizing disease dynamics and for assessing the therapeutic effects of experimental interventions.

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Optical coherence tomography identifies outer retina thinning in frontotemporal degeneration.

Publications

Optical coherence tomography identifies outer retina thinning in frontotemporal degeneration.

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