Early corticospinal tract damage in prodromal SCA2 revealed by EEG-EMG and EMG-EMG coherence

Publication date: December 2017
Source:Clinical Neurophysiology, Volume 128, Issue 12
Author(s): Luis Velázquez-Pérez, Johannes Tünnerhoff, Roberto Rodríguez-Labrada, Reidenis Torres-Vega, Yusely Ruiz-Gonzalez, Paolo Belardinelli, Jacqueline Medrano-Montero, Nalia Canales-Ochoa, Yanetza González-Zaldivar, Yaimeé Vazquez-Mojena, Georg Auburger, Ulf Ziemann
ObjectiveClinical data suggest early involvement of the corticospinal tract (CST) in spinocerebellar ataxia type 2 (SCA2). Here we tested if early CST degeneration can be detected in prodromal SCA2 mutation carriers by electrophysiological markers of CST integrity.MethodsCST integrity was tested in 15 prodromal SCA2 mutation carriers, 19 SCA2 patients and 25 age-matched healthy controls, using corticomuscular (EEG-EMG) and intermuscular (EMG-EMG) coherence measures in upper and lower limb muscles.ResultsSignificant reductions of EEG-EMG and EMG-EMG coherences were observed in the SCA2 patients, and to a similar extent in the prodromal SCA2 mutation carriers. In prodromal SCA2, EEG-EMG and EMG-EMG coherences correlated with the predicted time to ataxia onset.ConclusionsFindings indicate early CST neurodegeneration in SCA2. EEG-EMG and EMG-EMG coherence may serve as biomarkers of early CST neurodegeneration in prodromal SCA2 mutation carriers.SignificanceFindings are important for developing preclinical disease markers in the context of currently emerging disease-modifying therapies of neurodegenerative disorders.



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