Cognitive impairments in patients with spinocerebellar ataxia types 1 2 and 3 are positively correlated to the clinical severity of ataxia symptoms

摘要

Aims: This study is to assess cognitive function in patients with spinocerebellar ataxia types 1, 2 and 3 (SCA1, SCA2 and SCA3). Methods: We performed neuropsychological examinations on 8 SCA1 patients, 2 SCA2 patients, and 8 SCA3 patients, as well as 32 healthy subjects matching these patients in age, gender, nationality, and years of education. The neuropsychological examinations were focused on testing executive functions, visuo-spatial perception and verbal memory, attention, immediate and delayed recall, logical thinking function and orientation function. Results: SCA1 patients had significantly impaired executive function, visuo-spatial perception, and attention compared to healthy subjects. Cognitive disorders such as immediate and delayed recall, executive function and verbal memory were observed in SCA2 and SCA3 patients, while attention and visuo-spatial function were not affected. The severity of motor impairment was determined using the international cooperative ataxia rating scale, the scores of which ranged from 11 to 78. The number of patients with mild ataxia, moderate ataxia and severe ataxia was 3, 11, and 3, respectively, with the most severe ataxia occurring on a patient with SCA1. The scores of activities of daily living scale ranged from 20 to 66. Conclusions: Our results showed that mild executive dysfunction occurred in patients with SCA1, SCA2 and SCA3, and verbal fluency and word memory dysfunctions were detected in patients with SCA2 and SCA3. In addition, we found that the decreased logical thinking function and orientation function were observed in patients with SCA1, SCA2 and SCA3. The cognitive status was correlated with the clinical severity of ataxia symptoms rather than age, age of onset, years of education and the duration of disease.