Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance
Abstract Abnormal expansion of hexanucleotide GGGGCC (G4C2) in the C9ORF72 gene has been associated with multiple neurodegenerative disorders, with particularly high prevalence in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Repeat expansions of this type have been associat...
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2017
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oai:doaj.org-article:65f7f6627a3e4b1a8e9f80e21f08f7382021-12-02T16:08:24ZExpansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance10.1038/s41598-017-08857-32045-2322https://doaj.org/article/65f7f6627a3e4b1a8e9f80e21f08f7382017-08-01T00:00:00Zhttps://doi.org/10.1038/s41598-017-08857-3https://doaj.org/toc/2045-2322Abstract Abnormal expansion of hexanucleotide GGGGCC (G4C2) in the C9ORF72 gene has been associated with multiple neurodegenerative disorders, with particularly high prevalence in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Repeat expansions of this type have been associated with altered pathology, symptom rate and severity, as well as psychological changes. In this study, we enrolled twenty-five patients with ALS and fifteen neurologically healthy controls in a P300 brain-computer interface (BCI) training procedure. Four of the patients were found to possess an expanded allele, which was associated with a reduction in the quality of evoked potentials that led to reduced performance on the BCI task. Our findings warrant further exploration of the relationship between brain function and G4C2 repeat length. Such a relationship suggests that personalized assessment of suitability of BCI as a communication device in patients with ALS may be feasible.Andrew GeronimoKathryn E. SheldonJames R. BroachZachary SimmonsSteven J. SchiffNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 7, Iss 1, Pp 1-6 (2017) |
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Medicine R Science Q Andrew Geronimo Kathryn E. Sheldon James R. Broach Zachary Simmons Steven J. Schiff Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
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Abstract Abnormal expansion of hexanucleotide GGGGCC (G4C2) in the C9ORF72 gene has been associated with multiple neurodegenerative disorders, with particularly high prevalence in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Repeat expansions of this type have been associated with altered pathology, symptom rate and severity, as well as psychological changes. In this study, we enrolled twenty-five patients with ALS and fifteen neurologically healthy controls in a P300 brain-computer interface (BCI) training procedure. Four of the patients were found to possess an expanded allele, which was associated with a reduction in the quality of evoked potentials that led to reduced performance on the BCI task. Our findings warrant further exploration of the relationship between brain function and G4C2 repeat length. Such a relationship suggests that personalized assessment of suitability of BCI as a communication device in patients with ALS may be feasible. |
format |
article |
author |
Andrew Geronimo Kathryn E. Sheldon James R. Broach Zachary Simmons Steven J. Schiff |
author_facet |
Andrew Geronimo Kathryn E. Sheldon James R. Broach Zachary Simmons Steven J. Schiff |
author_sort |
Andrew Geronimo |
title |
Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
title_short |
Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
title_full |
Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
title_fullStr |
Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
title_full_unstemmed |
Expansion of C9ORF72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
title_sort |
expansion of c9orf72 in amyotrophic lateral sclerosis correlates with brain-computer interface performance |
publisher |
Nature Portfolio |
publishDate |
2017 |
url |
https://doaj.org/article/65f7f6627a3e4b1a8e9f80e21f08f738 |
work_keys_str_mv |
AT andrewgeronimo expansionofc9orf72inamyotrophiclateralsclerosiscorrelateswithbraincomputerinterfaceperformance AT kathrynesheldon expansionofc9orf72inamyotrophiclateralsclerosiscorrelateswithbraincomputerinterfaceperformance AT jamesrbroach expansionofc9orf72inamyotrophiclateralsclerosiscorrelateswithbraincomputerinterfaceperformance AT zacharysimmons expansionofc9orf72inamyotrophiclateralsclerosiscorrelateswithbraincomputerinterfaceperformance AT stevenjschiff expansionofc9orf72inamyotrophiclateralsclerosiscorrelateswithbraincomputerinterfaceperformance |
_version_ |
1718384473513394176 |