Oculomotor abnormalities in patients with cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) reflect midline cerebellar impairment

Cerebellar oculomotor dysfunction affected mainly smooth pursuit and gaze holding function at eccentric gaze positions, but it did neither comprise spontaneous nystagmus nor saccade abnormalities. Prolonged latencies in initial pursuit acceleration might be related to the patient's cognitive decline, but normal saccade latencies point to cerebellar oculomotor neurodegeneration. Smooth pursuit impairment was not related to disease duration and vestibulopathy, contrariwise pursuit impairment became worse with larger functional impairment. Oculomotor abnormalities in CANVAS are in line with midline cerebellar impairment without evidence for extracerebellar (brainstem) brain involvement.

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Publication Details

Journal
Journal of Neurology
Published
2026-02-01
DOI
https://doi.org/10.1007/s00415-026-13678-4
Primary Topic
Genetic Neurodegenerative Diseases
Type
article
Field-Weighted Citation Impact
0.00

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article

Oculomotor abnormalities in patients with cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) reflect midline cerebellar impairment

Astrid Nümann, Christoph Helmchen, Max Borsche, Andreas Sprenger et al.
Journal of Neurology
Genetic Neurodegenerative Diseases
article

Oculomotor abnormalities in patients with cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) reflect midline cerebellar impairment

Astrid Nümann, Christoph Helmchen, Max Borsche, Andreas Sprenger, N Brüggemann, Bente Lübbers, Renana Storm, Christos Ganos, Emmelie Weiss
article en

Abstract

Cerebellar oculomotor dysfunction affected mainly smooth pursuit and gaze holding function at eccentric gaze positions, but it did neither comprise spontaneous nystagmus nor saccade abnormalities. Prolonged latencies in initial pursuit acceleration might be related to the patient's cognitive decline, but normal saccade latencies point to cerebellar oculomotor neurodegeneration. Smooth pursuit impairment was not related to disease duration and vestibulopathy, contrariwise pursuit impairment became worse with larger functional impairment. Oculomotor abnormalities in CANVAS are in line with midline cerebellar impairment without evidence for extracerebellar (brainstem) brain involvement.

Journal of NeurologyVol. 273(2)
University of Toronto (CA), Toronto Western Hospital (CA), Humboldt-Universität zu Berlin (DE), University Hospital Schleswig-Holstein (DE), Charité - Universitätsmedizin Berlin (DE), University of Lübeck (DE)
Deutsche Forschungsgemeinschaft
Openalex Percentile: Top 57%
Genetic Neurodegenerative Diseases
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Oculomotor abnormalities in patients with cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) reflect midline cerebellar impairment — Astrid Nümann, Christoph Helmchen, et al. · Journal of Neurology (2026) | TGRS Research Map | TGRS