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Factors influencing cognitive function in patients with Huntington's disease from China: A cross-sectional clinical
Yang-Fan Cheng1, Kun-Cheng Liu1, Tian-Mi Yang1
1Department of Neurology, Laboratory of Neurodegenerative Disorders, Rare Disease Center, West China Hospital, Sichuan University, Chengdu, China.
Insights
Cognitive impairment is common in Chinese Huntington
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- Huntington's disease (HD) is an inherited neurodegenerative disorder.
- CAG repeat expansion causes HD.
- Cognitive decline impacts daily activities in manifest HD.
Purpose of the Study:
- Examine cognition in a Chinese HD cohort.
- Identify factors influencing cognitive domains in HD patients.
Main Methods:
- Neuropsychological batteries assessed 205 participants.
- Included MMSE, Stroop, SDMT, TMT, VFT, and Hopkins verbal learning test.
- Pearson correlation and multiple linear regression analyzed data.
Main Results:
- 41.46% of HD patients had normal global function initially.
- Significant differences in cognitive tests across HD stages observed.
- Motor symptom severity, functional capacity, and education independently predicted cognitive performance.
Conclusions:
- Cognitive impairment is prevalent in Chinese HD patients.
- Cognition worsens with advanced HD stages.
- Motor symptoms and functional capacity correlate with cognitive domains.
Background And Aim:
Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. Cognitive decline contributes to the loss of daily activity in manifest HD. We aimed to examine the cognition status in a Chinese HD cohort and explore factors influencing the diverse cognitive domains.
Methods:
A total of 205 participants were recruited in the study with the assessment by neuropsychological batteries, including the mini-mental state examination (MMSE), Stroop test, symbol digit modalities test (SDMT), trail making test (TMT), verbal fluency test (VFT), and Hopkins verbal learning test-revised, as well as motor and psychiatric assessment. Pearson correlation and multiple linear regression models were applied to investigate the correlation.
Results:
Only 41.46% of patients had normal global function first come to our center. There was a significantly difference in MMSE, Stroop test, SDMT, TMT, and VFT across each stage of HD patients (p < .05). Apathy of PBA-s was correlated to MMSE, animal VFT and Stroop-interference tests performance. Severity of motor symptoms, functional capacity, age, and age of motor symptom onset were correlated to all neuropsychological scores, whereas education attainment and diagnostic delay were correlated to most neuropsychological scores except TMT. Severity of motor symptoms, functional capacity, and education attainment showed independent predicting effect (p < .05) in diverse cognitive domains.
Conclusion:
Cognitive impairment was very common in Chinese HD patients at the first visit and worse in the patients in advanced phase. The severity of motor symptoms and functional capacity were correlated to the diverse cognitive domains.
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