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Published on: December 5, 2020
Cognitive changes in spinocerebellar ataxia type 2.
Martin Valis1, Jirí Masopust, Jan Bažant
1Department of Neurology, Charles University, Faculty of Medicine and University Hospital in Hradec Králové, Czech Republic. valismar@seznam.cz
Patients with spinocerebellar ataxia type 2 (SCA2) show significant cognitive deficits, performing worse on all tested tasks, especially those with increased motor difficulty. Cognitive impairment severity correlates with earlier disease onset.
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- Spinocerebellar ataxia type 2 (SCA2) affects 19-42% of patients with cognitive disorders and dementia.
- Neuropsychological testing reveals executive dysfunction, memory impairment, and speech difficulties in SCA2 patients.
Purpose of the Study:
- To evaluate cognitive performance in patients with spinocerebellar ataxia type 2 (SCA2).
- To compare cognitive abilities between SCA2 patients and healthy controls.
- To investigate the relationship between cognitive impairment and disease characteristics in SCA2.
Main Methods:
- Psychiatric and neuropsychological examinations were conducted on 12 SCA2 patients and 12 healthy volunteers.
- Motor impairment was assessed using the brief ataxia rating scale (BARS).
- Cognitive testing included executive functions, memory, attention, and psychomotor skills, with tasks categorized by motor difficulty. The cognitive abilities composite score (CACS) was calculated.
Main Results:
- SCA2 patients demonstrated significantly lower CACS compared to healthy volunteers (p=0.00005).
- Impaired performance was observed in both simple and difficult motor tasks.
- Cognitive impairment severity correlated with age at disease onset (p=0.002) but not disease duration or BARS score.
Conclusions:
- SCA2 patients exhibit widespread cognitive deficits compared to controls.
- Cognitive performance declines with increasing motor task difficulty.
- Earlier disease onset is associated with more severe cognitive impairment in SCA2.
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