Intermuscular Coherence in Spinocerebellar Ataxias 3 and 6: a Preliminary Study

Spinocerebellar ataxias (SCAs) are familial neurodegenerative diseases involving the cerebellum and spinocerebellar tracts. While there is variable involvement of corticospinal tracts (CST), dorsal root ganglia, and motor neurons in SCA3, SCA6 is characterized by a pure, late-onset ataxia. Abnormal...

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Veröffentlicht in:Cerebellum (London, England) England), 2024-04, Vol.23 (2), p.601-608
Hauptverfasser: Issa, Naoum P., Aydin, Serdar, Bhatnagar, Shail, Baumgartner, Nicholas W., Hill, Jacquelyn, Aluri, Sravya, Valentic, Chloe S., Polley, Eric, Gomez, Christopher M., Rezania, Kourosh
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container_issue 2
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container_title Cerebellum (London, England)
container_volume 23
creator Issa, Naoum P.
Aydin, Serdar
Bhatnagar, Shail
Baumgartner, Nicholas W.
Hill, Jacquelyn
Aluri, Sravya
Valentic, Chloe S.
Polley, Eric
Gomez, Christopher M.
Rezania, Kourosh
description Spinocerebellar ataxias (SCAs) are familial neurodegenerative diseases involving the cerebellum and spinocerebellar tracts. While there is variable involvement of corticospinal tracts (CST), dorsal root ganglia, and motor neurons in SCA3, SCA6 is characterized by a pure, late-onset ataxia. Abnormal intermuscular coherence in the beta-gamma frequency range (IMCβγ) implies a lack of integrity of CST or the afferent input from the acting muscles. We test the hypothesis that IMCβγ has the potential to be a biomarker of disease activity in SCA3 but not SCA6. Intermuscular coherence between biceps brachii and brachioradialis muscles was measured from surface EMG waveforms in SCA3 ( N = 16) and SCA6 ( N = 20) patients and in neurotypical subjects ( N = 23). IMC peak frequencies were present in the β range in SCA patients and in the γ range in neurotypical subjects. The difference between IMC amplitudes in the γ and β ranges was significant when comparing neurotypical control subjects to SCA3 ( p < 0.01) and SCA6 ( p = 0.01) patients. IMCβγ amplitude was smaller in SCA3 patients compared to neurotypical subjects ( p < 0.05), but not different between SCA3 and SCA6 patients or between SCA6 and neurotypical subjects. IMC metrics can differentiate SCA patients from normal controls.
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subjects Amyotrophic lateral sclerosis
Ataxia
Biomarkers
Biomedical and Life Sciences
Biomedicine
Cerebellum
Clinical trials
Disease
Dorsal root ganglia
Electromyography
Genetic testing
Humans
Longitudinal studies
Machado-Joseph Disease
Motor neurons
Muscles
Neurobiology
Neurodegenerative diseases
Neurology
Neurons
Neurosciences
Pyramidal tracts
Sensory neurons
Spinocerebellar ataxia
Spinocerebellar Ataxias
Transcranial magnetic stimulation
Volumetric analysis
title Intermuscular Coherence in Spinocerebellar Ataxias 3 and 6: a Preliminary Study
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