Bidirectional Connections between Depression and Ataxia Severity in Spinocerebellar Ataxia Type 3 Patients

Lin, Min; Yang, Jiajun; Chen, Pingping; Qian, Mei-Zhen; Lin, Hui‐Xia; Chen, Xiaoping; Shang, Xianjin; Wang, Danni; Chen, Yuchao; Jiang, Bin; Chen, Yijun; Chen, Wan‐Jin; Wang, Ning; Gan, Shi‐Rui

doi:10.1159/000489398

Cited by 16 publications

(21 citation statements)

References 26 publications

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“…It indicates that a larger proportion of SCA3 patients display depression in China than in other regions (20–34%) (32, 33). Contrary to other findings, we did not find the relationship between severity of ataxia and depression (8, 31). However, we found that the HAMD score was correlated with the CAG repeat length, which was not consistent with previous studies (8).…”

Section: Discussioncontrasting

confidence: 99%

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Extra-Cerebellar Signs and Non-motor Features in Chinese Patients With Spinocerebellar Ataxia Type 3

Yuan

Hou

et al. 2019

Front. Neurol.

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Objectives: Our study attempted to systematically explore the prevalence of extra-cerebellar signs and non-motor symptoms, such as anxiety, depression, fatigue, excessive daytime sleepiness (EDS) and sleep disturbances in a cohort of Chinese patients with spinocerebellar ataxia type 3 (SCA3), and further investigated the correlations between non-motor symptoms and clinical characteristics in SCA3 patients. Methods: This study included 68 molecular-proven SCA3 patients. Extra-cerebellar signs were evaluated with the Inventory of Non-Ataxia Symptoms (INAS). The INAS count indicated the number of non-ataxia signs in each patient. The severity of ataxia, fatigue, EDS, sleep quality, anxiety, and depression were assessed using the Scale for the assessment and rating of ataxia (SARA), Fatigue Severity Scale (FSS), Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Hamilton Anxiety Rating Scale (HAMA), and the Hamilton Depression Rating Scale (HAMD) (24 items), respectively. Results: Extra-cerebellar signs were detected in 91.2% of all SCA3 patients and the mean total INAS count was 2.72 ± 1.88. Rigidity was the most frequent extra-cerebellar sign (47.1%, N = 32). Sensory symptoms (2.9%, N = 2) and chorea (5.9%, N = 4) were rare, and myoclonus (0%) was not found in this cohort. High frequencies of sleep disturbances (64.7%), fatigue (52.9%), depression (48.5%), and anxiety (42.6%) were detected in SCA3 patients. The Spearman correlation indicated that the HAMD score was associated with the CAG repeat length and HAMA score, while the PSQI score was correlated with the SARA and FSS score. In addition, multivariate linear regression analysis showed that the CAG repeat length, age of onset, sleep disturbances and depression were significant predictors of fatigue in SCA3 patients. Conclusions: Our study indicates that the vast majority of SCA3 patients display extra-cerebellar signs. Except for EDS, anxiety, depression, fatigue and impaired sleep quality are present in SCA3 patients. The CAG repeat length, age of onset, sleep disturbances and depression are predictors of fatigue in SCA3 patients.

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Section: Discussioncontrasting

confidence: 99%

“…As for psychiatric symptoms, we found a high frequency of depression (48.5%) in SCA3 patients, which was in line with another study (57.69%) conducted in a Chinese SCA3 group (31). It indicates that a larger proportion of SCA3 patients display depression in China than in other regions (20–34%) (32, 33).…”

Section: Discussionsupporting

confidence: 91%

Extra-Cerebellar Signs and Non-motor Features in Chinese Patients With Spinocerebellar Ataxia Type 3

Yuan

Hou

et al. 2019

Front. Neurol.

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“…The positive correlation between the HDRS-24 score and the severity of ataxia in patients with SCA3 detected in the current study was in line with previous cross-sectional [31] and longitudinal [32] studies, which con rmed the effects of depression on ataxia progression in patients with SCA3. In addition, the negative correlation between the MoCA score and the severity of ataxia was also in agreement with previous studies [33], indicating that the cognition status can also be an indicator of motor deterioration in patients with SCA3.…”

Section: Discussionsupporting

confidence: 92%

Abnormal Eye Movements in Spinocerebellar Ataxia Type 3

Lin¹,

Zhang²,

Cao³

et al. 2020

Preprint

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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.

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“…Interestingly, in some genetic conditions characterized by ataxia, depression appears to be an important feature. For example, a recent study found that 57% of patients with spinocerebellar ataxia type 3 (SCA3) had depression and that this seemed to have a significant impact, positively contributing to the severity of their ataxia (22). Similarly, in a study of patients with Friedreich's ataxia, 21% of participants were found to have depression in the moderate/severe range (23).…”

Section: Discussionmentioning

confidence: 99%

Balance Problems, Paralysis, and Angina as Clinical Markers for Severity in Major Depression

MacDonald

Berlim

et al. 2020

Front. Psychiatry

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Major depressive disorder (MDD) is a heterogeneous disorder. Our hypothesis is that neurological symptoms correlate with the severity of MDD symptoms. One hundred eighty-four outpatients with MDD completed a self-report questionnaire on past and present medical history. Patients were divided into three roughly equal depression severity levels based on scores from the APA Severity Measure for Depression—Adult (n = 66, 58, 60, for low, medium, high severity, respectively). We saw a significant and gradual increase in the frequency of “muscular paralysis” (1.5–5.2–16.7%) and “balance problems” (21.2–36.2–46.6%) from low to medium to high severity groups. We repeated the analysis using only the two most extreme severity categories: low severity (66 samples) vs. high severity (60 samples). High severity patients were also found to experience more “angina” symptoms than low severity patients (27.3 vs. 50%). The three significant clinical variables identified were introduced into a binary logistic regression model as the independent variables with high or low severity as the dependent variable. Both “muscular paralysis” and “balance problems” were significantly associated with increased severity of depression (odds ratio of 13.5 and 2.9, respectively), while “angina” was associated with an increase in severity with an odds ratio of 2.0, albeit not significantly. We show that neurological exam or clinical history could be useful biomarkers for depression severity. Our findings, if replicated, could lead to a simple clinical scale administered regularly for monitoring patients with MDD.

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Bidirectional Connections between Depression and Ataxia Severity in Spinocerebellar Ataxia Type 3 Patients

Cited by 16 publications

References 26 publications

Extra-Cerebellar Signs and Non-motor Features in Chinese Patients With Spinocerebellar Ataxia Type 3

Extra-Cerebellar Signs and Non-motor Features in Chinese Patients With Spinocerebellar Ataxia Type 3

Abnormal Eye Movements in Spinocerebellar Ataxia Type 3

Balance Problems, Paralysis, and Angina as Clinical Markers for Severity in Major Depression

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