Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10.
<h4>Background</h4>Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant cerebellar ataxia, characterized by epilepsy, ataxic symptoms, and cognitive impairments linked to Cerebellar Cognitive Affective Syndrome (CCAS). The Cerebellar Cognitive Affective Syndrome Scale (CCAS-S)...
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Public Library of Science (PLoS)
2025-01-01
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| Online Access: | https://doi.org/10.1371/journal.pone.0319505 |
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| author | Angel Omar Romero-Molina Gabriel Ramirez-Garcia Amanda Chirino-Perez Gustavo Padron-Rivera Carlos Roberto Hernandez-Castillo Maria Guadalupe Garcia-Gomar Diana Laura Torres-Vences Juan Fernandez-Ruiz |
| author_facet | Angel Omar Romero-Molina Gabriel Ramirez-Garcia Amanda Chirino-Perez Gustavo Padron-Rivera Carlos Roberto Hernandez-Castillo Maria Guadalupe Garcia-Gomar Diana Laura Torres-Vences Juan Fernandez-Ruiz |
| author_sort | Angel Omar Romero-Molina |
| collection | DOAJ |
| description | <h4>Background</h4>Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant cerebellar ataxia, characterized by epilepsy, ataxic symptoms, and cognitive impairments linked to Cerebellar Cognitive Affective Syndrome (CCAS). The Cerebellar Cognitive Affective Syndrome Scale (CCAS-S) has been developed to identify CCAS across various cerebellar pathologies.<h4>Objective</h4>To determine whether patients with SCA10 exhibit CCAS using the CCAS-S, and to compare its effectiveness with the Montreal Cognitive Assessment (MoCA). A secondary objective was to evaluate the effect of demographic and clinical data on CCAS-S performance.<h4>Method</h4>Fifteen patients with SCA10 and fifteen matched controls underwent assessments using the CCAS-S, the MoCA, the Scale for the Assessment and Rating of Ataxia (SARA), and the Center for Epidemiologic Studies Depression Scale (CES-D). Diagnostic accuracy was analyzed using ROC curve analysis, comparing total and subcategory scores between groups. Demographic and clinical data were examined for relations with CCAS-S scores.<h4>Results</h4>The CCAS-S effectively distinguished cognitive impairments in SCA10 patients, showing satisfactory sensitivity and specificity (AUC of 0.83). Although no significant differences were found in the AUCs between CCAS-S and MoCA (p = 0.45), the CCAS-S demonstrated a significantly larger effect size in the comparison between patients and control group (d = 2.33). Cognitive performance was poorer in patients than in controls (p = < 0.001), with depressive symptoms and age having a significant impact on CCAS-S outcomes.<h4>Conclusions</h4>Patients with the SCA10 mutation exhibit CCAS. Besides the significant cognitive impairment, also detected by MoCA, the CCAS-S score was significantly affected by indicators of depressive mood and age, highlighting the importance of considering these variables during outcome analyses. |
| format | Article |
| id | doaj-art-e1d5def3f42f4b70a5a7bfc5574cba63 |
| institution | DOAJ |
| issn | 1932-6203 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Public Library of Science (PLoS) |
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| series | PLoS ONE |
| spelling | doaj-art-e1d5def3f42f4b70a5a7bfc5574cba632025-08-20T02:57:41ZengPublic Library of Science (PLoS)PLoS ONE1932-62032025-01-01203e031950510.1371/journal.pone.0319505Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10.Angel Omar Romero-MolinaGabriel Ramirez-GarciaAmanda Chirino-PerezGustavo Padron-RiveraCarlos Roberto Hernandez-CastilloMaria Guadalupe Garcia-GomarDiana Laura Torres-VencesJuan Fernandez-Ruiz<h4>Background</h4>Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant cerebellar ataxia, characterized by epilepsy, ataxic symptoms, and cognitive impairments linked to Cerebellar Cognitive Affective Syndrome (CCAS). The Cerebellar Cognitive Affective Syndrome Scale (CCAS-S) has been developed to identify CCAS across various cerebellar pathologies.<h4>Objective</h4>To determine whether patients with SCA10 exhibit CCAS using the CCAS-S, and to compare its effectiveness with the Montreal Cognitive Assessment (MoCA). A secondary objective was to evaluate the effect of demographic and clinical data on CCAS-S performance.<h4>Method</h4>Fifteen patients with SCA10 and fifteen matched controls underwent assessments using the CCAS-S, the MoCA, the Scale for the Assessment and Rating of Ataxia (SARA), and the Center for Epidemiologic Studies Depression Scale (CES-D). Diagnostic accuracy was analyzed using ROC curve analysis, comparing total and subcategory scores between groups. Demographic and clinical data were examined for relations with CCAS-S scores.<h4>Results</h4>The CCAS-S effectively distinguished cognitive impairments in SCA10 patients, showing satisfactory sensitivity and specificity (AUC of 0.83). Although no significant differences were found in the AUCs between CCAS-S and MoCA (p = 0.45), the CCAS-S demonstrated a significantly larger effect size in the comparison between patients and control group (d = 2.33). Cognitive performance was poorer in patients than in controls (p = < 0.001), with depressive symptoms and age having a significant impact on CCAS-S outcomes.<h4>Conclusions</h4>Patients with the SCA10 mutation exhibit CCAS. Besides the significant cognitive impairment, also detected by MoCA, the CCAS-S score was significantly affected by indicators of depressive mood and age, highlighting the importance of considering these variables during outcome analyses.https://doi.org/10.1371/journal.pone.0319505 |
| spellingShingle | Angel Omar Romero-Molina Gabriel Ramirez-Garcia Amanda Chirino-Perez Gustavo Padron-Rivera Carlos Roberto Hernandez-Castillo Maria Guadalupe Garcia-Gomar Diana Laura Torres-Vences Juan Fernandez-Ruiz Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. PLoS ONE |
| title | Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. |
| title_full | Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. |
| title_fullStr | Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. |
| title_full_unstemmed | Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. |
| title_short | Cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10. |
| title_sort | cerebellar cognitive affective syndrome in patients with spinocerebellar ataxia type 10 |
| url | https://doi.org/10.1371/journal.pone.0319505 |
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