Abstract
BackgroundCognitive dysfunctions have been reported in multiple system atrophy (MSA). However the underlying mechanisms remain to be elucidated. This study aimed to explore the possible cerebral metabolism associated with domain-specific cognitive performances in MSA.MethodsA total of 84 patients were diagnosed as probable or possible MSA, comprised of 27 patients as MSA with predominant parkinsonism (MSA-P) and 57 patients as MSA with predominant cerebellar ataxia (MSA-C). The comprehensive neuropsychological tests and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) imaging were performed. Z-score was calculated to non-dimensionalize and unify indicators of different tests in the domains of executive function, attention, language, memory, and visuospatial function. Correlations between specific Z-score and cerebral 18F-FDG uptake were analyzed using statistical parametric mapping. The cognition-related metabolic differences between patients with MSA-P and MSA-C were analyzed using the post-hoc test.ResultsZ-scores of the domains including attention, executive function, and language correlated positively with the metabolism in the superior/inferior frontal gyrus and cerebellum, but negatively with that in the insula and fusiform gyrus (p < 0.001). No significant differences in neuropsychological performances and frontal metabolism were found between patients with MSA-P and MSA-C. Only lower metabolism in the cerebellum was observed in MSA-C.ConclusionMetabolic changes in the frontal lobe and cerebellum may participate in the cognitive impairments of patients with MSA. Nevertheless, cognitive and corresponding metabolic differences between the two subtypes of MSA still need more exploration.
Highlights
Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disorder, with the classical clinical presence of progressive autonomic failure, parkinsonism, and/or cerebellar ataxia (Wenning et al, 1997; Stefanova et al, 2009; Fanciulli and Wenning, 2015)
Patients with MSA were divided into two subtypes according to their predominant clinical symptoms, including 27 patients with MSA-P and 57 patients with MSA-C
No group differences were found for age at onset and education, but differences were observed in age, sex, disease duration, and UPDRS III score (Table 1). Both subtypes had considerable and extensive cognitive impairments in each domain represented by number and percentage of impairment as follows: attention, MSA-P, 18(67%), MSA-C, 30(53%); executive function, MSA-P, 13(48%), MSA-C, 32(56%); memory, MSA-P, 18(67%), MSA-C, 25(44%); visuospatial function, MSA-P, 12(44%), MSA-C, 22(39%); language, MSA-P, 10(37%), MSA-C, 21(37%)
Summary
Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disorder, with the classical clinical presence of progressive autonomic failure, parkinsonism, and/or cerebellar ataxia (Wenning et al, 1997; Stefanova et al, 2009; Fanciulli and Wenning, 2015). Subsequent studies have compared the pathological burden degrees of glial cytoplasmic inclusions (GCIs) and neuronal cytoplasmic inclusions (NCIs) between MSA with normal cognition (MSA-NC) and MSA with cognitive impairment (MSA-CI; Cykowski et al, 2015; Homma et al, 2016; Koga et al, 2017; Jellinger, 2020; Miki et al, 2020). These results were variable and uncertain, which might be partially attributed to the small sample size. This study aimed to explore the possible cerebral metabolism associated with domain-specific cognitive performances in MSA
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