Abstract
BackgroundStudies on cognition in multiple system atrophy (MSA) patients are limited.MethodsA total of 110 MSA patients were evaluated using Addenbrooke's Cognitive Examination-Revised (ACE-R), Frontal Assessment Battery (FAB), Frontal Behavioral Inventory (FBI), and Unified MSA Rating Scale (UMSARS) tests. Fifty-five age-, sex-, education- and domicile-matched healthy controls were recruited to perform the FAB and ACE-R scales.ResultsApproximately 32.7% of the patients had global cognitive deficits with the most impaired domain being verbal fluency and visuospatial ability (26.4%), followed by memory (24.5%), language (20%) and orientation/attention (20%) based on a cut-off score of ACE-R ≤ 70. A total of 41.6% of the patients had frontal lobe dysfunction, with inhibitory control (60.9%) as the most impaired domain based on a cut-off score of FAB ≤14. Most patients (57.2%) showed moderate frontal behavior changes (FBI score 4–15), with incontinence (64.5%) as the most impaired domain. The binary logistic regression model revealed that an education level < 9 years (OR:13.312, 95% CI:2.931–60.469, P = 0.001) and UMSARS ≥ 40 (OR: 2.444, 95%CI: 1.002–5.962, P< 0.049) were potential determinants of abnormal ACE-R, while MSA-C (OR: 4.326, 95%CI: 1.631–11.477, P = 0.003), an education level < 9 years (OR:2.809 95% CI:1.060–7.444, P = 0.038) and UMSARS ≥ 40 (OR:5.396, 95%CI: 2.103–13.846, P < 0.0001) were potential determinants of abnormal FAB.ConclusionsCognitive impairment is common in Chinese MSA patients. MSA-C patients with low education levels and severe motor symptoms are likely to experience frontal lobe dysfunction, while MSA patients with low education levels and severe motor symptoms are likely to experience global cognitive deficits. These findings strongly suggest that cognitive impairment should not be an exclusion criterion for the diagnosis of MSA.
Highlights
Multiple system atrophy (MSA) is a sporadic adult-onset neurodegenerative disease characterized by any combination of parkinsonism, cerebellar ataxia, autonomic dysfunction and pyramidal tract dysfunction [1]
The binary logistic regression model revealed that an education level < 9 years (OR:13.312, 95% CI:2.931–60.469, P = 0.001) and Unified MSA Rating Scale (UMSARS) 40 (OR: 2.444, 95%CI: 1.002–5.962, P< 0.049) were potential determinants of abnormal Addenbrooke’s Cognitive Examination-Revised (ACE-R), while MSA-C (OR: 4.326, 95%CI: 1.631–11.477, P = 0.003), an education level < 9 years (OR:2.809 95% CI:1.060–7.444, P = 0.038) and UMSARS 40 (OR:5.396, 95%CI: 2.103– 13.846, P < 0.0001) were potential determinants of abnormal Frontal Assessment Battery (FAB)
Cognitive impairment is common in Chinese MSA patients
Summary
Multiple system atrophy (MSA) is a sporadic adult-onset neurodegenerative disease characterized by any combination of parkinsonism, cerebellar ataxia, autonomic dysfunction and pyramidal tract dysfunction [1]. The Addenbrooke’s Cognitive Examination-Revised (ACE-R) was developed as a brief cognitive assessment instrument incorporating elements of the Mini-Mental State Examination (MMSE). This multidimensional test facilitates the overall assessment of cognition and provides an assessment of the profile of different cognitive domains. The validation of different ACE-R versions has been conducted in several countries[11,12,13,14], and this test has been demonstrated as superior to the MMSE in the detection of cognitive dysfunction in patients with neurodegenerative diseases, such as Parkinson’s disease (PD)[15], MSA[16,17], progressive supranuclear palsy (PSP)[16], corticobasal degeneration (CBD)[16], Alzheimer’s disease (AD)[18], Huntington’s disease (HD)[17] and amyotrophic lateral sclerosis (ALS)[19]. Studies on cognition in multiple system atrophy (MSA) patients are limited
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