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

Mov Disord Clin Pract. 2024 Feb 29. doi: 10.1002/mdc3.14006. Online ahead of print.

Authors

Ruo-Yah Lai^{1

2}, Christian Rummey³, Christian J Amlang^{1

2

4}, Chi-Ying R Lin⁵, Tiffany X Chen^{1

2

6}, Susan Perlman⁷, George Wilmot⁸, Christopher M Gomez⁹, Jeremy D Schmahmann¹⁰, Henry Paulson¹¹, Sarah H Ying¹², Chiadi U Onyike¹², Theresa A Zesiewicz¹³, Khalaf O Bushara¹⁴, Michael D Geschwind¹⁵, Karla P Figueroa¹⁶, Stefan M Pulst¹⁶, Sub H Subramony¹⁷, Matthew R Burns¹⁷, Puneet Opal¹⁸, Antoine Duquette¹⁹, Tetsuo Ashizawa²⁰, Ali G Hamedani²¹, Marie Y Davis^{22

23}, Sharan R Srinivasan¹¹, Lauren R Moore²⁴, Vikram G Shakkottai²⁵, Liana S Rosenthal²⁶, Sheng-Han Kuo^{1

2}

Affiliations

¹ Department of Neurology, Columbia University Medical Center, New York, New York, USA.
² Initiative of Columbia Ataxia and Tremor, Columbia University Irving Medical Center, New York, New York, USA.
³ Clinical Data Science, Basel, Switzerland.
⁴ Department of Neurology, SUNY Downstate Health Sciences University, Brooklyn, New York, New York, USA.
⁵ Alzheimer's Disease and Parkinson's Disease Centers, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
⁶ Department of Biomedical Engineering, Whiting School of Engineering, Johns Hopkins University, Baltimore, Maryland, USA.
⁷ Department of Neurology, University of California Los Angeles, Los Angeles, California, USA.
⁸ Department of Neurology, Emory University, Atlanta, Georgia, USA.
⁹ Department of Neurology, University of Chicago, Chicago, Illinois, USA.
¹⁰ Ataxia Center, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Cognitive Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
¹¹ Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA.
¹² Department of Psychiatry and Behavioral Sciences, Johns Hopkins University, Baltimore, Maryland, USA.
¹³ Department of Neurology, University of South Florida, Tampa, Florida, USA.
¹⁴ Department of Neurology, University of Minnesota, Minneapolis, Minnesota, USA.
¹⁵ Department of Neurology, University of California San Francisco, San Francisco, California, USA.
¹⁶ Department of Neurology, University of Utah, Salt Lake City, Utah, USA.
¹⁷ Department of Neurology, McKnight Brain Institute, University of Florida, Gainesville, Florida, USA.
¹⁸ Department of Neurology, Northwestern University, Chicago, Illinois, USA.
¹⁹ Centre Hospitalier de l'Université de Montréal, University of Montreal, Montreal, Quebec, Canada.
²⁰ Houston Methodist Research Institute, Houston, Texas, USA.
²¹ Departments of Neurology, Ophthalmology, and Epidemiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
²² Department of Neurology, University of Washington, Seattle, Washington, USA.
²³ Neurology Division, VA Puget Sound Health Care System, Seattle, WA, United States.
²⁴ National Ataxia Foundation, Minneapolis, Minnesota, USA.
²⁵ Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
²⁶ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.

PMID: 38419568
DOI: 10.1002/mdc3.14006

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.

Abstract

Grants and funding