Assessment of Sleep Spindle Density Among Genetically Positive Spinocerebellar Ataxias Types 1, 2, and 3 Patients

Overview

Journal Ann Neurosci

Publisher Sage Publications

Specialty Neurology

Date 2018 Aug 25

PMID 30140122

Citations 5

Authors

Doniparthi Venkata Seshagiri

Ragasudha Botta

Arun Sasidharan

Pramod Kumar Pal

Sanjeev Jain

Ravi Yadav

Bindu M Kutty

Affiliations

Soon will be listed here.

Abstract

Objective: The effect of thalamic degeneration in patients with spinocerebellar ataxias (SCA) and sleep spindle (SS) abnormalities has not been studied so far, although there is a strong association between these disorders. This study was done to evaluate and compare the SS densities (SSDs) of genetically proven autosomal dominant SCA1, SCA2 and SCA3 patients with controls.

Methods: Prospectively and genetically confirmed cases of SCA and controls were recruited. Patients were assessed clinically, were evaluated with sleep questionnaires and an overnight polysomnography was performed. SSDs were analyzed using neuroloop gain plugin of Polyman version 1.15 software.

Results: Eighteen patients of SCA1 ( = 6), SCA2 ( = 5), SCA3 ( = 7) and 6 controls were recruited in our study. The mean age of SCA1 patients was 39.2 ± 5.4, of SCA2 patients was 30.8 ± 9.5 and of SCA3 patients was 35.4 ± 6.4 years. The mean duration of illness in SCA1 was 4.7 ± 1.7 years, in SCA2 it was 4.3 ± 4.4 years and in SCA3 it was 5 ± 2.3 years. The median SSD values (percentage loop gain) during stage 2 of non-rapid eye movement sleep were 16.9% in SCA1, 0% in SCA2, 1.2% in SCA3 and 59.5% in controls. There was a significant difference in SSD values in SCA2 ( = 0.04), SCA3 ( = 0.02) patients and controls.

Conclusion: SSDs were significantly decreased in patients with SCA, which is a novel finding. This is likely due to the "thalamic switch" disruption, observed as reduced SSDs in SCA2 and SCA3. Sleep spindle deficits could act as one of the biomarkers of ongoing neurodegeneration in the thalamic circuitry of SCA patients.

Citing Articles

Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3.

Tsimpanouli M, Ghimire A, Barget A, Weston R, Paulson H, Costa M Cells. 2022; 11(19).

PMID: 36231095 PMC: 9563426. DOI: 10.3390/cells11193132.

Communication from the cerebellum to the neocortex during sleep spindles.

Xu W, De Carvalho F, Clarke A, Jackson A Prog Neurobiol. 2020; 199:101940.

PMID: 33161064 PMC: 7938225. DOI: 10.1016/j.pneurobio.2020.101940.

Pathogenesis of SCA3 and implications for other polyglutamine diseases.

McLoughlin H, Moore L, Paulson H Neurobiol Dis. 2019; 134:104635.

PMID: 31669734 PMC: 6980715. DOI: 10.1016/j.nbd.2019.104635.

Molecular Mechanisms and Therapeutics for Spinocerebellar Ataxia Type 2.

Egorova P, Bezprozvanny I Neurotherapeutics. 2019; 16(4):1050-1073.

PMID: 31435879 PMC: 6985344. DOI: 10.1007/s13311-019-00777-6.

Sleep Disorders in Hereditary Ataxias.

Huebra L, Coelho F, Rezende Filho F, Barsottini O, Pedroso J Curr Neurol Neurosci Rep. 2019; 19(8):59.

PMID: 31342187 DOI: 10.1007/s11910-019-0968-1.

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