This preprint is under consideration at BMC Neurology. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research article
Abnormal Eye Movements in Spinocerebellar Ataxia Type 3
Huifang Shang
Sichuan University West China Hospital
Corresponding Author
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background: Abnormal eye movements are common in spinocerebellar ataxias Type 3 (SCA3). We conducted the research to explore the frequency of abnormal eye movements in Chinese patients with SCA3, to compare the demographic and clinical characteristics between SCA3 patients with and without each type of abnormal eye movement, and to explore the correlation between abnormal eye movements and the severity of ataxia.
Methods: Seventy-four patients with SCA3 were enrolled in this cross-sectional study. Six types of abnormal eye movements including impaired smooth pursuit, increased square-wave jerks (SWJ), gaze-evoked nystagmus (GEN), slowing of saccades, saccadic hypo/hypermetria and supranuclear gaze palsy were evaluated by experienced neurologists. The severity of ataxia was evaluated by Scale for the Assessment and Rating of Ataxia (SARA).
Results: The prevalence of impaired smooth pursuit, increased SWJ, GEN, slowing of saccades, saccadic hypo/hypermetria and supranuclear gaze palsy in Chinese SCA3 patients was 28.4%, 13.5%, 78.4%, 41.9%, 23.0%, and 5.4%, respectively. SCA3 patients with GEN had higher scores of International Cooperative Ataxia Rating Scale (ICARS-IV) and total ICARS, and longer length of CAG repeat than patients without GEN. SCA3 patients with slowing of saccades had a longer disease duration, higher scores of ICARS-I, ICARS-II, total ICARS and SARA than patients without slowing of saccades. SCA3 patients with saccadic hypo/hypermetria had higher scores of ICARS-III, ICARS-IV, and SARA than patients without saccadic hypo/hypermetria. The demographic and clinical characteristics did not differ significantly between SCA3 patients with and without impaired smooth pursuit, increased SWJ, or supranuclear gaze palsy. Multivariate linear regression showed that the number of abnormal eye movements (0-6), disease duration, Hamilton Depression Rating Scale-24 (HDRS-24) score, and CAG repeat length were positively correlated with SARA score, whereas Montreal Cognitive Assessment (MoCA) score was negatively correlated with SARA score in SCA3.
Conclusions: An increased number of abnormal eye movement types correlated with the severity of ataxia in SCA3.
Keywords
Spinocerebellar Ataxia Type 3, Eye Movements, Severity
Figure 1
Figure 2
Figure 3
This is a list of supplementary files associated with this preprint. Click to download.
- Video.mp4
Segment 1. This patient demonstrates impaired smooth pursuit; Segment 2. This patient demonstrates increased square-wave jerks; Segment 3. This patient demonstrates gaze-evoked nystagmus; Segment 4. This patient demonstrates slowing of saccades; Segment 5. This patient demonstrates saccadic dysmetria; Segment 6. This patient demonstrates vertical supranuclear gaze palsy.
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 3
Posted 12 Jan, 2021
No community comments so far
Submission checks complete
On 12 Jan, 2021
Version (no preprint)
Posted 03 Jan, 2021
Editorial decision: Minor revision
On 03 Jan, 2021
Editor assigned
On 01 Jan, 2021
Submission checks complete
On 01 Jan, 2021
Editor invited
On 01 Jan, 2021
This version was not preprinted
No comments provided
Review #1 received
Received 22 Nov, 2020
Reviewer #2 agreed
On 22 Nov, 2020
Review #2 received
Received 22 Nov, 2020
Reviewers invited
Invitations sent on 10 Nov, 2020
Reviewer #1 agreed
On 10 Nov, 2020
Editor assigned
On 09 Nov, 2020
Submission checks complete
On 09 Nov, 2020
Editor invited
On 09 Nov, 2020
No comments provided
Editorial decision: Major revision
On 12 Oct, 2020
Review #1 received
Received 11 Oct, 2020
Review #2 received
Received 27 Sep, 2020
Reviewer #2 agreed
On 26 Sep, 2020
Reviewer #1 agreed
On 18 Sep, 2020
Reviewers invited
Invitations sent on 13 Sep, 2020
Editor assigned
On 07 Sep, 2020
Submission checks complete
On 06 Sep, 2020
Editor invited
On 06 Sep, 2020
First submitted
On 05 Sep, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Neurology
Learn more about our company.
This preprint is under consideration at BMC Neurology. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research article
Abnormal Eye Movements in Spinocerebellar Ataxia Type 3
Huifang Shang
Sichuan University West China Hospital
Corresponding Author
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 3
Posted 12 Jan, 2021
No community comments so far
Submission checks complete
On 12 Jan, 2021
Version (no preprint)
Posted 03 Jan, 2021
Editorial decision: Minor revision
On 03 Jan, 2021
Editor assigned
On 01 Jan, 2021
Submission checks complete
On 01 Jan, 2021
Editor invited
On 01 Jan, 2021
This version was not preprinted
No comments provided
Review #1 received
Received 22 Nov, 2020
Reviewer #2 agreed
On 22 Nov, 2020
Review #2 received
Received 22 Nov, 2020
Reviewers invited
Invitations sent on 10 Nov, 2020
Reviewer #1 agreed
On 10 Nov, 2020
Editor assigned
On 09 Nov, 2020
Submission checks complete
On 09 Nov, 2020
Editor invited
On 09 Nov, 2020
No comments provided
Editorial decision: Major revision
On 12 Oct, 2020
Review #1 received
Received 11 Oct, 2020
Review #2 received
Received 27 Sep, 2020
Reviewer #2 agreed
On 26 Sep, 2020
Reviewer #1 agreed
On 18 Sep, 2020
Reviewers invited
Invitations sent on 13 Sep, 2020
Editor assigned
On 07 Sep, 2020
Submission checks complete
On 06 Sep, 2020
Editor invited
On 06 Sep, 2020
First submitted
On 05 Sep, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Neurology
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background: Abnormal eye movements are common in spinocerebellar ataxias Type 3 (SCA3). We conducted the research to explore the frequency of abnormal eye movements in Chinese patients with SCA3, to compare the demographic and clinical characteristics between SCA3 patients with and without each type of abnormal eye movement, and to explore the correlation between abnormal eye movements and the severity of ataxia.
Methods: Seventy-four patients with SCA3 were enrolled in this cross-sectional study. Six types of abnormal eye movements including impaired smooth pursuit, increased square-wave jerks (SWJ), gaze-evoked nystagmus (GEN), slowing of saccades, saccadic hypo/hypermetria and supranuclear gaze palsy were evaluated by experienced neurologists. The severity of ataxia was evaluated by Scale for the Assessment and Rating of Ataxia (SARA).
Results: The prevalence of impaired smooth pursuit, increased SWJ, GEN, slowing of saccades, saccadic hypo/hypermetria and supranuclear gaze palsy in Chinese SCA3 patients was 28.4%, 13.5%, 78.4%, 41.9%, 23.0%, and 5.4%, respectively. SCA3 patients with GEN had higher scores of International Cooperative Ataxia Rating Scale (ICARS-IV) and total ICARS, and longer length of CAG repeat than patients without GEN. SCA3 patients with slowing of saccades had a longer disease duration, higher scores of ICARS-I, ICARS-II, total ICARS and SARA than patients without slowing of saccades. SCA3 patients with saccadic hypo/hypermetria had higher scores of ICARS-III, ICARS-IV, and SARA than patients without saccadic hypo/hypermetria. The demographic and clinical characteristics did not differ significantly between SCA3 patients with and without impaired smooth pursuit, increased SWJ, or supranuclear gaze palsy. Multivariate linear regression showed that the number of abnormal eye movements (0-6), disease duration, Hamilton Depression Rating Scale-24 (HDRS-24) score, and CAG repeat length were positively correlated with SARA score, whereas Montreal Cognitive Assessment (MoCA) score was negatively correlated with SARA score in SCA3.
Conclusions: An increased number of abnormal eye movement types correlated with the severity of ataxia in SCA3.
Figure 1
Figure 2
Figure 3