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Related Experiment Video

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Persistent and Progressive Outer Retina Thinning in Frontotemporal Degeneration.

Benjamin J Kim1, Murray Grossman2, Delu Song1

  • 1Department of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.

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|April 26, 2019
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Summary

Frontotemporal degeneration (FTD) is linked to outer retina thinning, particularly in tauopathy patients. This thinning progresses over time and correlates with cognitive decline, suggesting it as a potential biomarker for disease progression.

Keywords:
frontotemporal degenerationoptical coherence tomographyprogressive supranuclear palsyretinatauopathy

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Area of Science:

  • Neuroscience
  • Ophthalmology
  • Biomarker Discovery

Background:

  • Alzheimer's disease is associated with inner retina thinning.
  • Previous cross-sectional studies indicated outer retina thinning in frontotemporal degeneration (FTD).
  • Longitudinal evaluation of outer retina thinning as a biomarker in FTD is needed.

Purpose of the Study:

  • To evaluate longitudinal changes in outer retina thickness in FTD patients.
  • To assess if outer retina thinning is specific to FTD and correlates with disease progression.
  • To investigate the potential of outer retina thinning as a biomarker for tauopathy.

Main Methods:

  • Spectral-domain optical coherence tomography (SD-OCT) measured retinal layer thicknesses at baseline and 1-2 years.
  • FTD patients were classified using clinical criteria, genetic analysis, and cerebrospinal fluid biomarkers to identify a tauopathy subgroup.
  • Retinal layer changes in FTD patients and controls were compared using generalized linear models, adjusting for covariates.

Main Results:

  • FTD patients and the tauopathy subgroup exhibited thinner outer retinas compared to controls.
  • Progressive outer retina thinning was significant in the tauopathy subgroup (-3.9 μm/year).
  • Outer retina thickness changes correlated with cognitive decline (MMSE changes) in FTD and tauopathy subgroups.

Conclusions:

  • Outer retina thinning is a persistent finding in FTD and correlates with disease progression.
  • Progressive outer retina thinning is particularly evident in probable tauopathy patients.
  • Outer retina thickness measured by SD-OCT may serve as a valuable biomarker for FTD, especially tauopathy.