The frequency of non-motor symptoms in SCA3 and their association with disease severity and lifestyle factors.

Hengel, Holger; Klockgether, Thomas; de Vries, Jeroen; Onyike, Chiadi; Reetz, Kathrin; Garcia-Moreno, Hector; Santana, Magda M; Silva, Patrick; Bushara, Khalaf; Initiative, European Spinocerebellar Ataxia Type-3/Machado-Joseph Disease; Hübener-Schmid, Jeannette; Infante, Jon; Schöls, Ludger; Pires, Paula; Solanky, Nita; Faber, Jennifer; de Almeida, Luís Pereira; Jacobi, Heike; Timmann, Dagmar; Schmahmann, Jeremy D; Martus, Peter; Cunha, Maria Ines; Synofzik, Matthis; Giunit, Paola; van de Warrenburg, Bart P; Lima, Manuela

doi:10.1007/s00415-022-11441-z

Journal Article

DZNE-2022-01668

The frequency of non-motor symptoms in SCA3 and their association with disease severity and lifestyle factors.

Hengel, H. (First author)DZNE* ; Martus, P. ; Faber, J.DZNE* ; Giunit, P. ; Garcia-Moreno, H. ; Solanky, N. ; Klockgether, T.DZNE* ; Reetz, K. ; van de Warrenburg, B. P. ; Santana, M. M. ; Silva, P. ; Cunha, M. I.Extern* ; de Almeida, L. P. ; Timmann, D. ; Infante, J. ; de Vries, J. ; Lima, M. ; Pires, P. ; Bushara, K. ; Jacobi, H.Extern* ; Onyike, C. ; Schmahmann, J. D. ; Hübener-Schmid, J. ; Synofzik, M.DZNE* ; Initiative, E. S. A. T. D. (Collaboration Author) ; Schöls, L. (Last author)DZNE*

2023
Springer Berlin

Journal of neurology 270(2), 944-952 (2022) [10.1007/s00415-022-11441-z]

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Please use a persistent id in citations: doi:10.1007/s00415-022-11441-z

Abstract: Non-motor symptoms (NMS) are a substantial burden for patients with SCA3. There are limited data on their frequency, and their relation with disease severity and activities of daily living is not clear. In addition, lifestyle may either influence or be affected by the occurrence of NMS.To characterize NMS in SCA3 and investigate possible associations with disease severity and lifestyle factors.In a prospective cohort study, we performed a cross-sectional analysis of NMS in 227 SCA3 patients, 42 pre-ataxic mutation carriers, and 112 controls and tested for associations with SARA score, activities of daily living, and the lifestyle factors alcohol consumption, smoking and physical activity.Sleep disturbance, restless legs syndrome, mild cognitive impairment, depression, bladder dysfunction and pallhypesthesia were frequent among SCA3 patients, while mainly absent in pre-ataxic mutation carriers. Except for restless legs syndrome, NMS correlated significantly with disease severity and activities of daily living. Alcohol abstinence was associated with bladder dysfunction. Patients with higher physical activity showed less cognitive impairment and fewer depressive symptoms, but these differences were not significant.This study revealed a clear association between disease severity and NMS, likely driven by the progression of the widespread neurodegenerative process. Associations between lifestyle and NMS can probably be attributed to the influence of NMS on lifestyle.

Keyword(s): Humans (MeSH) ; Restless Legs Syndrome: epidemiology (MeSH) ; Prospective Studies (MeSH) ; Cross-Sectional Studies (MeSH) ; Activities of Daily Living (MeSH) ; Patient Acuity (MeSH) ; Life Style (MeSH) ; Lifestyle ; Non-motor symptoms ; Physical activity ; SCA3

Classification:

ddc:610

Note: ISSN 1432-1459 not unique: **2 hits**.

Contributing Institute(s):

Research Program(s):

353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2022

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Medline

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