Abstract

Background/ObjectiveParkinson's disease (PD) and the atypical parkinsonian syndromes multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are movement disorders associated with degeneration of the central nervous system. Degeneration of the retina has not been systematically compared in these diseases.MethodsThis cross-sectional study used spectral-domain optical coherence tomography with manual segmentation to measure the peripapillar nerve fiber layer, the macular thickness, and the thickness of all retinal layers in foveal scans of 40 patients with PD, 19 with MSA, 10 with CBS, 15 with PSP, and 35 age- and sex-matched controls.ResultsThe mean paramacular thickness and volume were reduced in PSP while the mean RNFL did not differ significantly between groups. In PSP patients, the complex of retinal ganglion cell- and inner plexiform layer and the outer nuclear layer was reduced. In PD, the inner nuclear layer was thicker than in controls, MSA and PSP. Using the ratio between the outer nuclear layer and the outer plexiform layer with a cut-off at 3.1 and the additional constraint that the inner nuclear layer be under 46 µm, we were able to differentiate PSP from PD in our patient sample with a sensitivity of 96% and a specificity of 70%.ConclusionDifferent parkinsonian syndromes are associated with distinct changes in retinal morphology. These findings may serve to facilitate the differential diagnosis of parkinsonian syndromes and give insight into the degenerative processes of patients with atypical parkinsonian syndromes.

Highlights

  • Parkinson’s disease (PD) and the atypical parkinsonian syndromes multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are movement disorders associated with degeneration of the central nervous system

  • Disease duration was longer in PD, but did not differ between the other groups

  • Routine Parameters retinal nerve fiber layer (RNFL) and macular thickness The peripapillar RNFL, paramacular thickness and volume, and the thickness of the different retinal layers were measured as illustrated in figure 1A, the results of all retinal parameters for the

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Summary

Introduction

Parkinson’s disease (PD) and the atypical parkinsonian syndromes multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are movement disorders associated with degeneration of the central nervous system. In PD, prolonged latencies of visually evoked potentials (VEP) [5] and deficits in contrast sensitivity and color vision [6,7] have been reported. In a patient with MSA, optic disc pallor and negative ERGs were observed [12]. Visual event related potentials using a visual oddball paradigm were reported to be abnormal in all four diseases [13]. The often-observed blurry vision of PSP patients is usually attributed to the pronounced impairment of ocular movements and diplopia [14], but might be associated with retinal changes. A post-mortem study using high performance liquid chromatography suggested a reduction in the dopamine content of retinas of PD patients [16]

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