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Eye movement abnormalities are associated with brainstem atrophy in Wilson disease

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Abstract

Backgrounds

This study aims to characterize eye movement abnormalities in Wilson disease and examine their association with the degree of brainstem atrophy.

Methods

Twenty patients (10 males, mean age 46.8, SD 8.9 years) with genetically confirmed neurological WD on stable anti-copper treatment and 20 age- and sex-matched healthy subjects were examined. Eye movements, including prosaccade and antisaccade tasks, were evaluated using infrared videooculography. MRI was performed using 1.5 T system, and T2-weighted images were used for the measurement of midbrain and pontine area on mid-sagittal slices. Clinical severity was assessed using the Unified Wilson’s Disease Rating Scale (UWDRS).

Results

Compared to healthy controls, WD patients showed prolonged latencies of horizontal prosaccades and hypometry of both horizontal (p = 0.04) and vertical (p = 0.0046) prosaccades. In the antisaccade task, WD patients showed prolonged latency of both horizontal (p = 0.04) and vertical antisaccades (p = 0.047) and increased error rate of vertical antisaccades (p = 0.04). There is a significant association between midbrain area and horizontal latencies (r = −0.53; p = 0.02) and vertical maximum speed in prosaccades (r = 0.47; p = 0.04). The pons area inversely correlated with horizontal prosaccade and antisaccade latencies (p = 0.007).

Conclusions

We showed impairments of ocular saccades such as prolonged latencies, hypometry, and increased error rate in antisaccades. The strong association between prolonged latencies of prosaccades and the brainstem atrophy suggests that VOG might serve as a sensitive electrophysiological marker of brainstem dysfunction in WD.

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Acknowledgements

This work was supported by the Czech Ministry of Health (15-25602A).

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Authors and Affiliations

  1. Department of Neurology and Centre of Clinical Neuroscience, Charles University, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic

    Jaromír Hanuška, Petr Dušek, Jan Rusz, Olga Ulmanová & Evžen Růžička

  2. Departmet of Neurosurgery, Na Homolce Hospital, Prague, Czech Republic

    Jaromír Hanuška

  3. Department of Radiology, Charles University, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic

    Petr Dušek & Andrea Burgetová

  4. Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University, Prague, Czech Republic

    Jan Rusz

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  1. Jaromír Hanuška
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  2. Petr Dušek
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  4. Olga Ulmanová
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  6. Evžen Růžička
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Hanuška J., Dušek P., Rusz J., Ulmanová O., Burgetová A., and Růžička E. The first draft of the manuscript was written by Hanuška J., and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Petr Dušek.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study. 

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Hanuška, J., Dušek, P., Rusz, J. et al. Eye movement abnormalities are associated with brainstem atrophy in Wilson disease. Neurol Sci 41, 1097–1103 (2020). https://doi.org/10.1007/s10072-019-04225-3

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