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Decreased retinal sensitivity and loss of retinal nerve fibers in multiple system atrophy

  • Neuro-ophthalmology
  • Published:
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Abstract

Background and aim

In a previous study, retinal nerve fiber layer thickness (RNFLT) loss was shown as part of the neurodegenerative process in multiple system atrophy (MSA). Here, we investigate in a larger cohort of MSA patients whether the RNFLT loss translates into respective visual field defects.

Methods

Spectral domain optical coherence tomography was performed in 20 MSA patients (parkinsonian subtype = 12, cerebellar subtype = 8) to quantify peripapillary RNFLT. Visual field (90°) was analyzed by automated static perimetry to investigate retinal structure/function relationship. Eight data sets did not meet stringent quality criteria, and only 12 data sets were further analyzed.

Results

Compared to healthy controls, MSA patients demonstrated a significant reduction of RNFLT in the nasal sectors (p nasal-superior  = 0.02, p nasal  = 0.03, p nasal-inferior  < 0.01), while changes in temporal RNFLT measures (p temporal-superior  = 0.42, p temporal  = 0.34, p temporal-inferior  = 0.25) were not statistically significant compared to healthy controls (ANOVA). MSA patients featured a significant global mean deviation (2.74 dB; p < 0.01) without predominant peripheral visual field defects. Statistical analysis of mean defect in the central (0–30°), peripheral (30–90°) or global (0–90°) visual field revealed no significant correlation (r 2 central = 0.11, r 2 peripheral = 0.04, r 2 global = 0.07) with nasal RNFLT in MSA patients.

Conclusion

MSA patients feature significant reduction in nasal RNFLT and global mean deviation when compared to healthy controls, consistent with the multi-systemic nature of this neurodegenerative disorder. This finding provides first evidence for two independent deteriorations of the visual system in MSA.

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Disclosure

Ulrich Schiefer is Consultant of Haag–Streit, Koenitz, Switzerland. He developed several perimetric strategies, and holds patents in this field. The other authors report no conflicts of interest. The authors also have full control of all primary data, and agree to allow the journal to review the data on request.

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Author notes

  1. M. Dominik Fischer

    Present address: Nuffield Laboratory of Ophthalmology, University of Oxford, Oxford, UK

Authors and Affiliations

  1. University Eye Clinic, Centre for Ophthalmology, Tübingen, Germany

    M. Dominik Fischer, Christoph Kernstock, Janko Dietzsch, Robert Heidlauf & Ulrich Schiefer

  2. Department of Neurodegeneration, Hertie Institute for Clinical Brain Research and German Center of Neurodegenerative Diseases (DZNE), University of Tübingen, 72076, Tübingen, Germany

    Matthis Synofzik, Julia Schicks, Karin Srulijes, Sarah Wiethoff, Daniela Berg & Ludger Schöls

  3. Klinikum Stuttgart, Bürgerhospital, Neurologische Klinik, Stuttgart, Germany

    Oliver Menn

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  1. M. Dominik Fischer
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Corresponding author

Correspondence to Ludger Schöls.

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Supplemental Fig. 1

Example of the visual field test protocol with results from an individual MSA patient. White-on-white perimetry was used with Goldmann III stimulus size at 200 ms stimulus duration and 1200 ms response time on a background-illumination set to 10 cd/m2. One hundred and nine stimuli were distributed in a polar pattern, with 58 stimuli within the central 30°. Interpolation data of responses are presented in areas of grey values, which indicate level of localized sensitivity defects (JPEG 34 kb)

High Resolution Image 1

(TIFF 3526 kb)

Supplemental Fig. 2

Pearson’s correlation analyses of global (left panels) and nasal (right panels) retinal nerve fiber layer thickness (RNFLT) with a-b disease duration in years, or c-d severity of disease as quantified by the united multiple system atrophy rating scale (UMSARS). No correlation reached statistical significance (p > 0.05); correlation coefficient r 2 is displayed in the upper left corner of each scatter graph (JPEG 43 kb)

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Fischer, M.D., Synofzik, M., Kernstock, C. et al. Decreased retinal sensitivity and loss of retinal nerve fibers in multiple system atrophy. Graefes Arch Clin Exp Ophthalmol 251, 235–241 (2013). https://doi.org/10.1007/s00417-012-2118-1

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  • DOI: https://doi.org/10.1007/s00417-012-2118-1

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