Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study
Abstract Background Cognitive decline is a common non-motor feature characterizing Spinocerebellar Ataxia type 2 (SCA2) during the prodromal stage, nevertheless a reduced number of surrogate biomarkers of these alterations have been described. Objective To provide insights into cognitive dysfunction...
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doaj-5c9648945d6443d9add71f7aa5adca552020-11-25T00:59:08ZengBMCCerebellum & Ataxias2053-88712019-03-01611910.1186/s40673-019-0097-2Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential studyRoberto Rodríguez-Labrada0Luis Velázquez-Pérez1Ricardo Ortega-Sánchez2Arnoy Peña-Acosta3Yaimeé Vázquez-Mojena4Nalia Canales-Ochoa5Jacqueline Medrano-Montero6Reidenis Torres-Vega7Yanetza González-Zaldivar8Centre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasCentre for the Research and Rehabilitation of Hereditary AtaxiasAbstract Background Cognitive decline is a common non-motor feature characterizing Spinocerebellar Ataxia type 2 (SCA2) during the prodromal stage, nevertheless a reduced number of surrogate biomarkers of these alterations have been described. Objective To provide insights into cognitive dysfunction in SCA2 patients using P300 event-related potentials (ERP) and to evaluate these measures as biomarkers of the disease. Methods A cross-sectional study was performed with 30 SCA2 patients, 20 preclinical carriers and 33 healthy controls, who underwent visual, auditory P300 ERPs, and neurological examinations and ataxia scoring. Results SCA2 patients showed significant increase in P300 latencies and decrease of P300 amplitudes for visual and auditory stimuli, whereas preclinical carriers exhibit a less severe, but significant prolongation of P300 latencies. Multiple regression analyses disclosed a significant effect of SARA score on visual P300 abnormalities in patients as well as of the time to ataxia onset on visual P300 latencies in preclinical carriers. Conclusions This paper demonstrated the role of P300 ERP for the study of attentional, discriminative and working memory abnormalities in SCA2 patients and for the search of surrogate biomarkers from prodromal to the symptomatic stages. Moreover, our findings provide psychophysiological evidences supporting the cerebellar involvement in cognitive processes and allows us to identify promising outcome measures for future trials focusing on cognitive dysfunction.http://link.springer.com/article/10.1186/s40673-019-0097-2Spinocerebellar ataxia type 2P300Event–related evoked potentialsBiomarkersCognitive decline |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Roberto Rodríguez-Labrada Luis Velázquez-Pérez Ricardo Ortega-Sánchez Arnoy Peña-Acosta Yaimeé Vázquez-Mojena Nalia Canales-Ochoa Jacqueline Medrano-Montero Reidenis Torres-Vega Yanetza González-Zaldivar |
spellingShingle |
Roberto Rodríguez-Labrada Luis Velázquez-Pérez Ricardo Ortega-Sánchez Arnoy Peña-Acosta Yaimeé Vázquez-Mojena Nalia Canales-Ochoa Jacqueline Medrano-Montero Reidenis Torres-Vega Yanetza González-Zaldivar Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study Cerebellum & Ataxias Spinocerebellar ataxia type 2 P300 Event–related evoked potentials Biomarkers Cognitive decline |
author_facet |
Roberto Rodríguez-Labrada Luis Velázquez-Pérez Ricardo Ortega-Sánchez Arnoy Peña-Acosta Yaimeé Vázquez-Mojena Nalia Canales-Ochoa Jacqueline Medrano-Montero Reidenis Torres-Vega Yanetza González-Zaldivar |
author_sort |
Roberto Rodríguez-Labrada |
title |
Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study |
title_short |
Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study |
title_full |
Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study |
title_fullStr |
Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study |
title_full_unstemmed |
Insights into cognitive decline in spinocerebellar Ataxia type 2: a P300 event-related brain potential study |
title_sort |
insights into cognitive decline in spinocerebellar ataxia type 2: a p300 event-related brain potential study |
publisher |
BMC |
series |
Cerebellum & Ataxias |
issn |
2053-8871 |
publishDate |
2019-03-01 |
description |
Abstract Background Cognitive decline is a common non-motor feature characterizing Spinocerebellar Ataxia type 2 (SCA2) during the prodromal stage, nevertheless a reduced number of surrogate biomarkers of these alterations have been described. Objective To provide insights into cognitive dysfunction in SCA2 patients using P300 event-related potentials (ERP) and to evaluate these measures as biomarkers of the disease. Methods A cross-sectional study was performed with 30 SCA2 patients, 20 preclinical carriers and 33 healthy controls, who underwent visual, auditory P300 ERPs, and neurological examinations and ataxia scoring. Results SCA2 patients showed significant increase in P300 latencies and decrease of P300 amplitudes for visual and auditory stimuli, whereas preclinical carriers exhibit a less severe, but significant prolongation of P300 latencies. Multiple regression analyses disclosed a significant effect of SARA score on visual P300 abnormalities in patients as well as of the time to ataxia onset on visual P300 latencies in preclinical carriers. Conclusions This paper demonstrated the role of P300 ERP for the study of attentional, discriminative and working memory abnormalities in SCA2 patients and for the search of surrogate biomarkers from prodromal to the symptomatic stages. Moreover, our findings provide psychophysiological evidences supporting the cerebellar involvement in cognitive processes and allows us to identify promising outcome measures for future trials focusing on cognitive dysfunction. |
topic |
Spinocerebellar ataxia type 2 P300 Event–related evoked potentials Biomarkers Cognitive decline |
url |
http://link.springer.com/article/10.1186/s40673-019-0097-2 |
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