Abstract

Nonmotor symptoms in patients with multiple system atrophy (MSA) have received an increasing amount of attention in recent years, but no research on MSA patients' cognitive characteristics has been conducted in China. To evaluate the cognitive function of MSA patients in China. Using a case–control study design, 256 MSA patients and 64 controls were evaluated by the Montreal cognitive assessment (MoCA) scale to characterize their cognitive function. Like the controls, 60.5% of the patients with MSA had cognitive impairment, but the characteristics of cognitive impairment between the two groups were different. The cognitive impairment in MSA patients was prominent in the cognitive domains of visuospatial/executive functions, naming, attention, and orientation; particularly, the visuospatial/executive functions were the most significantly impaired, while impairment in language function was mainly seen in the controls. Besides, impairments in visuospatial/executive functions, attention, language, and orientation were more prominent in MSA-P (MSA with predominant Parkinsonism) patients than in MSA-C (MSA with predominant cerebellar ataxia). The cognitive impairments were more severe in patients with probable MSA than in patients with possible MSA. In addition, the results showed that the level of cognitive function was negatively correlated with the severity of MSA. This study, which characterized the cognitive function of MSA patients with the largest sample size known so far in China, found that patients with MSA do have cognitive impairment and display specific characteristics. Therefore, the cognitive impairment of MSA should be paid more attention.The study has been registered in the Chinese Clinical Trial Registry (ChiCTR) (Registration No: ChiCTR1900022462).

Highlights

  • Multiple system atrophy (MSA), a delayed sporadic progressive neurodegenerative disease, is a type of atypical Parkinsonian syndrome characterized by autonomic nervous system dysfunction, Parkinson-like symptoms that respond poorly to chronic levodopa therapy, and cerebellar ataxia and pyramidal symptoms

  • The exclusion criteria were (1) a similar family history, (2) MSA caused by systemic disease or other confirmed causes, (3) hallucinations unrelated to drugs, (4) neurologically confirmed dementia according to the DSM-IV diagnostic criteria, (5) remarkably slow vertical saccades or vertical supranuclear gaze palsy, (6) local cerebral cortical lesions such as aphasia, body integrity identity disorder (BIID), and parietal dysfunction, or (7) pregnant patients or patients who could not complete the scale test and related examinations due to severe heart, liver, or kidney diseases

  • There were no significant differences in Montreal cognitive assessment (MoCA) scores between MSA patients and controls

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Summary

Introduction

Multiple system atrophy (MSA), a delayed sporadic progressive neurodegenerative disease, is a type of atypical Parkinsonian syndrome characterized by autonomic nervous system dysfunction, Parkinson-like symptoms that respond poorly to chronic levodopa therapy, and cerebellar ataxia and pyramidal symptoms. The motor symptoms are remarkable in MSA and have received a significant amount of attention. As research has advanced in recent years, an increasing amount of evidence has shown that the cognitive impairment of patients with MSA cannot be ignored. More and more studies have shown that MSA patients have different degrees of cognitive i­mpairment[3,4,5,6,7]. A study conducted by the neuropsychological working group of the MDS-MSA research group found that MSA patients’ cognitive impairments were most prominent in executive functions and verbal fluency, followed by attention, memory, and visuospatial. This study used the largest sample size known to characterize the cognitive function in Chinese MSA patients. This study emphasizes that the cognitive impairment of patients with MSA should be taken seriously

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