Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias

Ruo Yah Lai; Christian Rummey; Christian J. Amlang; Chi Ying R. Lin; Tiffany X. Chen; Susan Perlman; George Wilmot; Christopher M. Gomez; Jeremy D. Schmahmann; Henry Paulson; Sarah H. Ying; Chiadi U. Onyike; Theresa A. Zesiewicz; Khalaf O. Bushara; Michael D. Geschwind; Karla P. Figueroa; Stefan M. Pulst; Sub H. Subramony; Matthew R. Burns; Puneet Opal; Antoine Duquette; Tetsuo Ashizawa; Ali G. Hamedani; Marie Y. Davis; Sharan R. Srinivasan; Lauren R. Moore; Vikram G. Shakkottai; Liana S. Rosenthal; Sheng Han Kuo

doi:10.1002/mdc3.14006

Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias

Ruo Yah Lai, Christian Rummey, Christian J. Amlang, Chi Ying R. Lin, Tiffany X. Chen, Susan Perlman, George Wilmot, Christopher M. Gomez, Jeremy D. Schmahmann, Henry Paulson, Sarah H. Ying, Chiadi U. Onyike, Theresa A. Zesiewicz, Khalaf O. Bushara, Michael D. Geschwind, Karla P. Figueroa, Stefan M. Pulst, Sub H. Subramony, Matthew R. Burns, Puneet OpalAntoine Duquette, Tetsuo Ashizawa, Ali G. Hamedani, Marie Y. Davis, Sharan R. Srinivasan, Lauren R. Moore, Vikram G. Shakkottai, Liana S. Rosenthal, Sheng Han Kuo^*

^*Corresponding author for this work

Neurology

Research output: Contribution to journal › Article › peer-review

Abstract

Background: Fatigue is a prevalent and debilitating symptom in neurological disorders, including spinocerebellar ataxias (SCAs). However, the risk factors of fatigue in the SCAs as well as its impact have not been well investigated. Objectives: To study the prevalence of fatigue in SCAs, the factors contributing to fatigue, and the influence of fatigue on quality of life. Methods: Fatigue was assessed in 418 participants with SCA1, SCA2, SCA3, and SCA6 from the Clinical Research Consortium for the Study of Cerebellar Ataxia using the Fatigue Severity Scale. We conducted multi-variable linear regression models to examine the factors contributing to fatigue as well as the association between fatigue and quality of life. Results: Fatigue was most prevalent in SCA3 (52.6%), followed by SCA1 (36.7%), SCA6 (35.7%), and SCA2 (35.6%). SCA cases with fatigue had more severe ataxia and worse depressive symptoms. In SCA3, those with fatigue had a longer disease duration and longer pathological CAG repeat numbers. In multi-variable models, depressive symptoms, but not ataxia severity, were associated with more severe fatigue. Fatigue, independent of ataxia and depression, contributed to worse quality of life in SCA3 and SCA6 at baseline, and fatigue continued affecting quality of life throughout the disease course in all types of SCA. Conclusions: Fatigue is a common symptom in SCAs and is closely related to depression. Fatigue significantly impacts patients' quality of life. Therefore, screening for fatigue should be considered a part of standard clinical care for SCAs.

Original language	English (US)
Pages (from-to)	496-503
Number of pages	8
Journal	Movement Disorders Clinical Practice
Volume	11
Issue number	5
DOIs	https://doi.org/10.1002/mdc3.14006
State	Published - May 2024

Funding

R.L. declares that there are no additional disclosures to report. C.R. has received research and consultancy fees from the Friedreich's Ataxia Research Alliance, Biohaven, Takeda, PTC Therapeutics, and Solid Biosciences. C.J.A. declares that there are no additional disclosures to report. C.Y.R.L. is a Medscape Neurology Decision Point: Alzheimer's disease Section Editor. T.X.C. declares that there are no additional disclosures to report. S.P. declares that there are no additional disclosures to report. G.W. declares that there are no additional disclosures to report. C.M.G. declares that there are no additional disclosures to report. J.D.S. declares that there are no additional disclosures to report. H.P. has received research funding from the National Institutes of Health (NIH). S.H.Y. declares that there are no additional disclosures to report. C.U.O. has received research funding from Biogen, Alector, and Transposon. He has served as a consultant for Acadia, Eisai, Otsuka, and Reata. T.A.Z. declares that there are no additional disclosures to report. K.O.B. declares that there are no additional disclosures to report. M.D.G. declares that there are no additional disclosures to report. KPF declares that there are no additional disclosures to report. S.M.P. declares that there are no additional disclosures to report. S.H.S. declares that there are no additional disclosures to report. M.R.B. has received grants from the National Institute on Aging and Cognito Therapeutics. P.O. has received support from the NIH (1R01NS082351 and 1R01NS127204). He has received funding from the following sources for clinical trials: Biohaven Pharmaceuticals and NIH U01NS104326 (site PI). A.D. declares that there are no additional disclosures to report. T.A. has received grants from the NIH and Biogen and has participated in Biohaven‐funded clinical trials NCT03952806 and NCT03701399. A.G.H. has received grants from the National Eye Institute and The Michael J. Fox Foundation, and clinical trial support from Biogen and Biohaven. M.Y.D. declares that there are no additional disclosures to report. S.R.S. declares that there are no additional disclosures to report. L.M. declares that there are no additional disclosures to report. V.G.S. declares that there are no additional disclosures to report. L.S.R. declares that there are no additional disclosures to report. S.H.K. is supported by the NIH: National Institute of Neurological Disorders and Stroke (NINDS) R01 NS104423 (principal investigator), NINDS R01 NS118179 (principal investigator), NINDS R01 NS124854 (principal investigator). Financial Disclosures for Previous 12 Months: R.L. has received no specific funding for this work and has no conflicts of interest relevant to this work. C.R. has received research and consultancy fees from the National Ataxia Foundation. C.J.A. has received no specific funding for this work and has no conflicts of interest relevant to this work. C.Y.R.L. has received no specific funding for this work and has no conflicts of interest relevant to this work. T.X.C. has received no specific funding for this work and has no conflicts of interest relevant to this work. S.P. has received research funding from the National Ataxia Foundation. G.W. has received research funding from the National Ataxia Foundation. C.M.G. has received research funding from the National Ataxia Foundation. J.D.S. has received research funding from the National Ataxia Foundation. H.P. has received research funding from the National Ataxia Foundation. S.H.Y. has received research funding from the National Ataxia Foundation. C.U.O. has received research funding from the National Ataxia Foundation. T.A.Z. has received research funding from the National Ataxia Foundation. K.O.B. has received research funding from the National Ataxia Foundation. M.D.G. has received research funding from the National Ataxia Foundation. K.P.F. has received research funding from the National Ataxia Foundation. S.M.P. has received research funding from the National Ataxia Foundation. S.H.S. has received research funding from the National Ataxia Foundation. M.R.B. has received research funding from the National Ataxia Foundation. P.O. has received research funding from the National Ataxia Foundation. A.D. has received research funding from the National Ataxia Foundation. T.A. has received research funding from the National Ataxia Foundation. A.G.H. has received no specific funding for this work and has no conflicts of interest relevant to this work. M.Y.D. has received no specific funding for this work and has no conflicts of interest relevant to this work. S.R.S. has received no specific funding for this work and has no conflicts of interest relevant to this work. L.M. has received research funding from the National Ataxia Foundation. V.G.S. has received research funding from the National Ataxia Foundation. L.S.R. has received research funding from the National Ataxia Foundation. S.H.K. has received research funding from the National Ataxia Foundation. Funding Sources and Conflicts of Interest:

Keywords

cerebellar ataxia
cerebellum
fatigue
quality of life
spinocerebellar ataxia

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1002/mdc3.14006

Cite this

Lai, R. Y., Rummey, C., Amlang, C. J., Lin, C. Y. R., Chen, T. X., Perlman, S., Wilmot, G., Gomez, C. M., Schmahmann, J. D., Paulson, H., Ying, S. H., Onyike, C. U., Zesiewicz, T. A., Bushara, K. O., Geschwind, M. D., Figueroa, K. P., Pulst, S. M., Subramony, S. H., Burns, M. R., ... Kuo, S. H. (2024). Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias. Movement Disorders Clinical Practice, 11(5), 496-503. https://doi.org/10.1002/mdc3.14006

@article{e125eb15721848549f8b0a1e237f14c7,

title = "Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias",

abstract = "Background: Fatigue is a prevalent and debilitating symptom in neurological disorders, including spinocerebellar ataxias (SCAs). However, the risk factors of fatigue in the SCAs as well as its impact have not been well investigated. Objectives: To study the prevalence of fatigue in SCAs, the factors contributing to fatigue, and the influence of fatigue on quality of life. Methods: Fatigue was assessed in 418 participants with SCA1, SCA2, SCA3, and SCA6 from the Clinical Research Consortium for the Study of Cerebellar Ataxia using the Fatigue Severity Scale. We conducted multi-variable linear regression models to examine the factors contributing to fatigue as well as the association between fatigue and quality of life. Results: Fatigue was most prevalent in SCA3 (52.6%), followed by SCA1 (36.7%), SCA6 (35.7%), and SCA2 (35.6%). SCA cases with fatigue had more severe ataxia and worse depressive symptoms. In SCA3, those with fatigue had a longer disease duration and longer pathological CAG repeat numbers. In multi-variable models, depressive symptoms, but not ataxia severity, were associated with more severe fatigue. Fatigue, independent of ataxia and depression, contributed to worse quality of life in SCA3 and SCA6 at baseline, and fatigue continued affecting quality of life throughout the disease course in all types of SCA. Conclusions: Fatigue is a common symptom in SCAs and is closely related to depression. Fatigue significantly impacts patients' quality of life. Therefore, screening for fatigue should be considered a part of standard clinical care for SCAs.",

keywords = "cerebellar ataxia, cerebellum, fatigue, quality of life, spinocerebellar ataxia",

author = "Lai, {Ruo Yah} and Christian Rummey and Amlang, {Christian J.} and Lin, {Chi Ying R.} and Chen, {Tiffany X.} and Susan Perlman and George Wilmot and Gomez, {Christopher M.} and Schmahmann, {Jeremy D.} and Henry Paulson and Ying, {Sarah H.} and Onyike, {Chiadi U.} and Zesiewicz, {Theresa A.} and Bushara, {Khalaf O.} and Geschwind, {Michael D.} and Figueroa, {Karla P.} and Pulst, {Stefan M.} and Subramony, {Sub H.} and Burns, {Matthew R.} and Puneet Opal and Antoine Duquette and Tetsuo Ashizawa and Hamedani, {Ali G.} and Davis, {Marie Y.} and Srinivasan, {Sharan R.} and Moore, {Lauren R.} and Shakkottai, {Vikram G.} and Rosenthal, {Liana S.} and Kuo, {Sheng Han}",

note = "Publisher Copyright: {\textcopyright} 2024 International Parkinson and Movement Disorder Society.",

year = "2024",

month = may,

doi = "10.1002/mdc3.14006",

language = "English (US)",

volume = "11",

pages = "496--503",

journal = "Movement Disorders Clinical Practice",

issn = "2330-1619",

publisher = "John Wiley and Sons Ltd",

number = "5",

}

Lai, RY, Rummey, C, Amlang, CJ, Lin, CYR, Chen, TX, Perlman, S, Wilmot, G, Gomez, CM, Schmahmann, JD, Paulson, H, Ying, SH, Onyike, CU, Zesiewicz, TA, Bushara, KO, Geschwind, MD, Figueroa, KP, Pulst, SM, Subramony, SH, Burns, MR, Opal, P, Duquette, A, Ashizawa, T, Hamedani, AG, Davis, MY, Srinivasan, SR, Moore, LR, Shakkottai, VG, Rosenthal, LS & Kuo, SH 2024, 'Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias', Movement Disorders Clinical Practice, vol. 11, no. 5, pp. 496-503. https://doi.org/10.1002/mdc3.14006

TY - JOUR

T1 - Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias

AU - Lai, Ruo Yah

AU - Rummey, Christian

AU - Amlang, Christian J.

AU - Lin, Chi Ying R.

AU - Chen, Tiffany X.

AU - Perlman, Susan

AU - Wilmot, George

AU - Gomez, Christopher M.

AU - Schmahmann, Jeremy D.

AU - Paulson, Henry

AU - Ying, Sarah H.

AU - Onyike, Chiadi U.

AU - Zesiewicz, Theresa A.

AU - Bushara, Khalaf O.

AU - Geschwind, Michael D.

AU - Figueroa, Karla P.

AU - Pulst, Stefan M.

AU - Subramony, Sub H.

AU - Burns, Matthew R.

AU - Opal, Puneet

AU - Duquette, Antoine

AU - Ashizawa, Tetsuo

AU - Hamedani, Ali G.

AU - Davis, Marie Y.

AU - Srinivasan, Sharan R.

AU - Moore, Lauren R.

AU - Shakkottai, Vikram G.

AU - Rosenthal, Liana S.

AU - Kuo, Sheng Han

PY - 2024/5

Y1 - 2024/5

N2 - Background: Fatigue is a prevalent and debilitating symptom in neurological disorders, including spinocerebellar ataxias (SCAs). However, the risk factors of fatigue in the SCAs as well as its impact have not been well investigated. Objectives: To study the prevalence of fatigue in SCAs, the factors contributing to fatigue, and the influence of fatigue on quality of life. Methods: Fatigue was assessed in 418 participants with SCA1, SCA2, SCA3, and SCA6 from the Clinical Research Consortium for the Study of Cerebellar Ataxia using the Fatigue Severity Scale. We conducted multi-variable linear regression models to examine the factors contributing to fatigue as well as the association between fatigue and quality of life. Results: Fatigue was most prevalent in SCA3 (52.6%), followed by SCA1 (36.7%), SCA6 (35.7%), and SCA2 (35.6%). SCA cases with fatigue had more severe ataxia and worse depressive symptoms. In SCA3, those with fatigue had a longer disease duration and longer pathological CAG repeat numbers. In multi-variable models, depressive symptoms, but not ataxia severity, were associated with more severe fatigue. Fatigue, independent of ataxia and depression, contributed to worse quality of life in SCA3 and SCA6 at baseline, and fatigue continued affecting quality of life throughout the disease course in all types of SCA. Conclusions: Fatigue is a common symptom in SCAs and is closely related to depression. Fatigue significantly impacts patients' quality of life. Therefore, screening for fatigue should be considered a part of standard clinical care for SCAs.

AB - Background: Fatigue is a prevalent and debilitating symptom in neurological disorders, including spinocerebellar ataxias (SCAs). However, the risk factors of fatigue in the SCAs as well as its impact have not been well investigated. Objectives: To study the prevalence of fatigue in SCAs, the factors contributing to fatigue, and the influence of fatigue on quality of life. Methods: Fatigue was assessed in 418 participants with SCA1, SCA2, SCA3, and SCA6 from the Clinical Research Consortium for the Study of Cerebellar Ataxia using the Fatigue Severity Scale. We conducted multi-variable linear regression models to examine the factors contributing to fatigue as well as the association between fatigue and quality of life. Results: Fatigue was most prevalent in SCA3 (52.6%), followed by SCA1 (36.7%), SCA6 (35.7%), and SCA2 (35.6%). SCA cases with fatigue had more severe ataxia and worse depressive symptoms. In SCA3, those with fatigue had a longer disease duration and longer pathological CAG repeat numbers. In multi-variable models, depressive symptoms, but not ataxia severity, were associated with more severe fatigue. Fatigue, independent of ataxia and depression, contributed to worse quality of life in SCA3 and SCA6 at baseline, and fatigue continued affecting quality of life throughout the disease course in all types of SCA. Conclusions: Fatigue is a common symptom in SCAs and is closely related to depression. Fatigue significantly impacts patients' quality of life. Therefore, screening for fatigue should be considered a part of standard clinical care for SCAs.

KW - cerebellar ataxia

KW - cerebellum

KW - fatigue

KW - quality of life

KW - spinocerebellar ataxia

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U2 - 10.1002/mdc3.14006

DO - 10.1002/mdc3.14006

M3 - Article

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AN - SCOPUS:85186172225

SN - 2330-1619

VL - 11

SP - 496

EP - 503

JO - Movement Disorders Clinical Practice

JF - Movement Disorders Clinical Practice

IS - 5

ER -

Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias

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