Prevalence and Spectrum of Non-Motor Symptoms in Patients of Spinocerebellar Ataxias in Kashmiri Population

Abstract

Background: Spinocerebellar ataxias (SCAs) are progressive neurodegenerative disorders increasingly recognized to have substantial non-motor manifestations. Data regarding these symptoms in the Kashmiri population remain limited. Objective: To assess the prevalence and pattern of non-motor symptoms among genetically confirmed SCA patients and compare them with healthy controls. Methods: This observational cross-sectional study was conducted from December 2021 to December 2023 at the Government Super Speciality Hospital, Srinagar. Twenty-five clinically and genetically confirmed SCA patients and 26 age- and sex-matched healthy controls were included. Anxiety, depression, fatigue, sleep disturbances, pain, and cognition were assessed using validated questionnaires. Disease severity was assessed using the Scale for Assessment and Rating of Ataxia (SARA). Data were analyzed using the Mann-Whitney test, with p≤0.05 considered statistically significant. Results: Among 25 patients, SCA1 was the most common subtype (52%), followed by SCA2 (40%). The median SARA score was 12 (5-37). Compared with controls, SCA patients had significantly higher median scores for anxiety, depression, fatigue, REM sleep behavior disorder, daytime sleepiness, and pain, and significantly lower MMSE scores (all p<0.05), except for restless legs syndrome. Fatigue was significantly greater in SCA2 than SCA1 (median FSS 4.0 vs. 2.7; p=0.005). Pain was reported by 19 patients. Conclusion: Non-motor symptoms are common among genetically confirmed SCA patients and represent an important component of disease burden. Routine assessment and early management of these manifestations should complement evaluation of motor symptoms.