TY - JOUR
T1 - Cortical network dysfunction revealed by magnetoencephalography in carriers of spinocerebellar ataxia 1 or 2 mutation
AU - Visani, Elisa
AU - Mariotti, Caterina
AU - Nanetti, Lorenzo
AU - Mongelli, Alessia
AU - Castaldo, Anna
AU - Panzica, Ferruccio
AU - Rossi Sebastiano, Davide
AU - Nigri, Anna
AU - Grisoli, Marina
AU - Franceschetti, Silvana
AU - Canafoglia, Laura
N1 - Funding Information:
This study was partially supported by the Italian Ministry of Health, Grant RF-2011-02347420 (to CM and LC).
Publisher Copyright:
© 2020 International Federation of Clinical Neurophysiology
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/7
Y1 - 2020/7
N2 - Objective: In patients with spinocerebellar ataxia type 1 or 2 (SCA1 or SCA2) and in their asymptomatic gene-positive relatives (AsyRs) we investigated the event-related desynchronization and synchronisation (ERD/ERS) on magnetoencephalographic signals to assess the changes occurring before manifest ataxia, by comparing the results obtained in AsyRs and in their gene-negative healthy relatives (HRs). Methods: Twenty-four patients (12 SCA1, 12 SCA2), 24 AsyRs (13 SCA1, 11 SCA2) and 17 HRs performed a visually cued Go/No-go task. We evaluated the ERD/ERS in regions of interest corresponding to the frontal, central and parietal cortices. Results: In the SCA patients the main findings were a loss of side predominance for alpha and beta ERD and significantly weakened beta ERS. In AsyRs the main finding was a significantly enhanced alpha ERD, namely in those who were approaching the estimated time of symptom onset. Conclusions: In ataxic patients, the loss of ERD lateralisation and the significantly reduction of beta ERS suggest defective bilateral processes that are involved in ending the movement. In AsyRs, enhanced alpha ERD proposes the presence of preclinical marker closely preceding symptom onset. Significance: Movement-related ERD/ERS can detect the defective sensorimotor integration in ataxic patients, and reveals possible compensatory mechanisms in their AsyRs.
AB - Objective: In patients with spinocerebellar ataxia type 1 or 2 (SCA1 or SCA2) and in their asymptomatic gene-positive relatives (AsyRs) we investigated the event-related desynchronization and synchronisation (ERD/ERS) on magnetoencephalographic signals to assess the changes occurring before manifest ataxia, by comparing the results obtained in AsyRs and in their gene-negative healthy relatives (HRs). Methods: Twenty-four patients (12 SCA1, 12 SCA2), 24 AsyRs (13 SCA1, 11 SCA2) and 17 HRs performed a visually cued Go/No-go task. We evaluated the ERD/ERS in regions of interest corresponding to the frontal, central and parietal cortices. Results: In the SCA patients the main findings were a loss of side predominance for alpha and beta ERD and significantly weakened beta ERS. In AsyRs the main finding was a significantly enhanced alpha ERD, namely in those who were approaching the estimated time of symptom onset. Conclusions: In ataxic patients, the loss of ERD lateralisation and the significantly reduction of beta ERS suggest defective bilateral processes that are involved in ending the movement. In AsyRs, enhanced alpha ERD proposes the presence of preclinical marker closely preceding symptom onset. Significance: Movement-related ERD/ERS can detect the defective sensorimotor integration in ataxic patients, and reveals possible compensatory mechanisms in their AsyRs.
KW - Asymptomatic gene-positive relatives
KW - ERD/ERS
KW - MEG
KW - Spinocerebellar ataxia
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U2 - 10.1016/j.clinph.2020.03.036
DO - 10.1016/j.clinph.2020.03.036
M3 - Article
C2 - 32408088
AN - SCOPUS:85084368230
VL - 131
SP - 1548
EP - 1555
JO - Clinical Neurophysiology
JF - Clinical Neurophysiology
SN - 1388-2457
IS - 7
ER -