小脑橄榄脑桥萎缩症 3 型的眼球运动异常。

Abnormal eye movements in spinocerebellar ataxia type 3.

机构信息

Department of neurology, Laboratory of Neurodegenerative Disorders, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

出版信息

BMC Neurol. 2021 Jan 19;21(1):28. doi: 10.1186/s12883-021-02057-3.

DOI:10.1186/s12883-021-02057-3

PMID:33468086

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7814728/

Abstract

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.

摘要

背景

眼球运动异常在脊髓小脑性共济失调 3 型（SCA3）中很常见。我们开展此项研究，旨在探讨中国 SCA3 患者眼球运动异常的发生频率，比较伴有和不伴有每种类型眼球运动异常的 SCA3 患者的人口统计学和临床特征，并探讨眼球运动异常与共济失调严重程度的相关性。

方法

本横断面研究纳入了 74 例 SCA3 患者。由经验丰富的神经科医生评估 6 种类型的眼球运动异常，包括扫视运动受损、高频扫视性眼震（SWJ）增加、视动性眼震（GEN）、扫视运动速度减慢、扫视性眼跳过远或过近和核上性眼球运动障碍。采用共济失调量表（SARA）评估共济失调严重程度。

结果

中国 SCA3 患者中扫视运动受损、高频扫视性眼震增加、GEN、扫视运动速度减慢、扫视性眼跳过远或过近和核上性眼球运动障碍的发生率分别为 28.4%、13.5%、78.4%、41.9%、23.0%和 5.4%。伴有 GEN 的 SCA3 患者的国际合作共济失调评分量表（ICARS-IV）和总 ICARS 评分以及 CAG 重复序列长度均高于不伴有 GEN 的患者。伴有扫视运动速度减慢的 SCA3 患者的病程较长，ICARS-I、ICARS-II、总 ICARS 和 SARA 评分均高于不伴有扫视运动速度减慢的患者。伴有扫视性眼跳过远或过近的 SCA3 患者的 ICARS-III、ICARS-IV 和 SARA 评分均高于不伴有扫视性眼跳过远或过近的患者。伴有扫视运动受损、高频扫视性眼震增加或核上性眼球运动障碍的 SCA3 患者的人口统计学和临床特征无显著差异。多变量线性回归显示，眼球运动异常的类型（0-6 种）、病程、汉密尔顿抑郁量表-24 项（HDRS-24）评分和 CAG 重复序列长度与 SARA 评分呈正相关，而蒙特利尔认知评估（MoCA）评分与 SARA 评分呈负相关。