Abstract
Fixation nystagmus refers to the nystagmus that appears or markedly increases with fixation. While relatively common in infantile (congenital) nystagmus, acquired fixation nystagmus is unusual and has been ascribed to lesions involving the cerebellar nuclei or the fibers projecting from the cerebellum to the brainstem. We aimed to report the clinical features of patients with acquired fixation nystagmus and discuss possible mechanisms using a model simulation and diagnostic significance. We describe four patients with acquired fixation nystagmus that appears or markedly increases with visual fixation. All patients had lesions involving the cerebellum or dorsal medulla. All patients showed direction-changing gaze-evoked nystagmus, impaired smooth pursuit, and decreased vestibular responses on head-impulse tests. The clinical implication of fixation nystagmus is that it may occur in central lesions that impair both smooth pursuit and the vestibulo-ocular reflex (VOR) but without creating a spontaneous nystagmus in the dark. We develop a mathematical model that hypothesizes that fixation nystagmus reflects a central tone imbalance due to abnormal function in cerebellar circuits that normally optimize the interaction between visual following (pursuit) and VOR during attempted fixation. Patients with fixation nystagmus have central lesions involving the cerebellar circuits that are involved in visual–vestibular interactions and normally eliminate biases that cause a spontaneous nystagmus.
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Change history
28 April 2022
A Correction to this paper has been published: https://doi.org/10.1007/s00415-022-11157-0
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Funding
This study was supported by the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health and Welfare (HI14C3477), and the Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education, Science and Technology (no. NRF-2016R1D1A1B04935568).
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Dr. Lee wrote the manuscript and analyzed and interpreted the data. Drs. H.J. Kim J.Y. Choi and J.H. Choi, and Zee analyzed and interpreted the data, and revised the manuscript. Dr. J.S. Kim designed and conceptualized the study, interpreted the data, and revised the manuscript.
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Drs. Lee, H.J. Kim, J.Y. Choi, and J.H. Choi report no disclosures. Dr. JS Kim serves as an Associate Editor of Frontiers in Neuro-otology and on the editorial boards of the Journal of Clinical Neurology, Frontiers in Neuro-ophthalmology, Journal of Neuro-ophthalmology, Journal of Vestibular Research, Journal of Neurology, and Medicine. Dr. Zee serves on the editorial board for Frontiers in Neuro-otology, The cerebellum, and the Journal of the Italian Neurology Society. The authors thank Sung-Jin Jo for the illustration.
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This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of Institutional Review Board of Seoul National University Bundang Hospital (B-1910/570-107) and Pusan National University Yangsan Hospital (05-2019-170).
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Lee, SU., Kim, HJ., Choi, JY. et al. Nystagmus only with fixation in the light: a rare central sign due to cerebellar malfunction. J Neurol 269, 3879–3890 (2022). https://doi.org/10.1007/s00415-022-11108-9
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DOI: https://doi.org/10.1007/s00415-022-11108-9