Neuropsychological and clinical heterogeneity of cognitive impairment in patients with multiple system atrophy

Barcelos, Lorena Broseghini; Saad, Flávia; Giacominelli, Carla; Saba, Roberta Arb; Aguiar, Patrícia Maria de Carvalho; Silva, Sônia Maria Cesar de Azevedo; Borges, Vanderci; Bertolucci, Paulo Henrique Ferreira; Ferraz, Henrique Ballalai

doi:10.1016/j.clineuro.2017.10.039

Cited by 30 publications

(40 citation statements)

References 36 publications

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“…Our study shows that approximately 40% of patients had a documented slight to moderate cognitive impairment in at least one domain, wherein executive functions were most often affected in line with previous findings . In detail, executive dysfunction in MSA comprised deficits in semantic and phonemic verbal fluency as well as processing speed, cognitive flexibility, and working memory, being impaired in up to 42% in this cohort. Whereas verbal memory was affected in a similar percentage, which is in agreement with previous reports, figural memory and attention span were widely preserved.…”

Section: Discussionsupporting

confidence: 89%

“…So far, few studies have addressed the possible differences between patients with predominant cerebellar or parkinsonian presentation, and the focus has been mainly on MSA‐P patients. In this study, a comparison of raw scores revealed lower performance of MSA‐C patients than MSA‐P patients in a screening of executive functions and in phonological verbal fluency.…”

Section: Discussionmentioning

confidence: 99%

“…7 Although this was beyond the scope of the following study and therefore has not been assessed, these major distinctive features are usually not present in patients with MSA. 7 So far, few studies have addressed the possible differences between patients with predominant cerebellar or parkinsonian presentation, [12][13][14][15][16][17] and the focus has been mainly on MSA-P patients. In this study, a comparison of raw scores revealed lower performance of MSA-C patients than MSA-P patients in a screening of executive functions and in phonological verbal fluency.…”

Section: Discussionmentioning

confidence: 99%

“…have been conducted but reported heterogeneous results. 8,[12][13][14][15][16][17] Kawai and colleagues observed a multidomain cognitive decline in MSA-P but not in MSA-C. 12 Another study reported a more pronounced executive and verbal memory dysfunction in MSA-C, 14 and a third found comparable cognitive profiles in both variants. 8 Cognitive deficits in MSA may be explained by underlying degeneration of basal ganglia and to secondarily disrupted striato-pallido-thalamocortical circuits.…”

Section: Introductionmentioning

confidence: 98%

“…In contrast, severe cognitive decline that significantly disrupts daily living is uncommon in MSA; hence, dementia has been regarded as a nonsupporting feature in the current diagnostic criteria . Comparative studies on cognitive impairment in both motor subtypes have been conducted but reported heterogeneous results . Kawai and colleagues observed a multidomain cognitive decline in MSA‐P but not in MSA‐C .…”

Section: Introductionmentioning

confidence: 99%

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Cognition in multiple system atrophy: a single‐center cohort study

Eschlböck

Delazer

Krismer

et al. 2020

Ann Clin Transl Neurol

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Objective Cognitive impairment in multiple system atrophy (MSA) is common, but remain poorly characterized. We evaluated cognitive and behavioral features in MSA patients and assessed between‐group differences for MSA subtypes and the effect of orthostatic hypotension (OH) on cognition. Methods This retrospective study included 54 patients with clinical diagnosis of possible and probable MSA referred to the Department of Neurology at Medical University of Innsbruck between 2000 and 2018. Neurological work‐up included comprehensive neuropsychological testing including Consortium to Establish a Registry for Alzheimer's Disease (CERAD‐plus) test battery, Frontal Assessment Battery (FAB), digit span test (DST), clock drawing task (CLOX1), and Hospital Anxiety and Depression Scale (HADS‐D). Results The mean MMSE score was 27.6 points. Overall, slight to moderate cognitive impairment was noted in up to 40% of patients, with predominant impairment of executive function and verbal memory. Patients with the cerebellar variant performed significantly worse than patients with the parkinsonian type (P < 0.05) in a screening of executive functions (FAB) and in phonemic verbal fluency. Depression and anxiety scores were elevated in 28% and 22% of MSA patients, respectively. Cognitive profile, depression, and anxiety levels were comparable between patients with and without OH. Interpretation Cognitive deficits are relatively frequent in MSA and primarily affect executive functions and verbal memory. Future comparative studies including Parkinson dementia, Lewy body disease, and MSA cases with and without OH are required to elucidate disease‐specific cognitive profiles in these synucleinopathies and to examine the influence of cardiovascular autonomic dysfunction on cognitive function in MSA.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Cognition in multiple system atrophy: a single‐center cohort study

Eschlböck

Delazer

Krismer

et al. 2020

Ann Clin Transl Neurol

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Early autonomic and cognitive dysfunction in PD, DLB and MSA: blurring the boundaries between α-synucleinopathies

et al. 2020

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Differential diagnosis between Parkinson's disease, dementia with Lewy bodies and multiple system atrophy can be difficult, especially because in early phase they might present with overlapping clinical features. Notably, orthostatic hypotension and cognitive dysfunction are common nonmotor aspects of parkinsonian syndromes and can be both present from the earliest stages of all α-synucleinopathies, indicating a common neurobiological basis in their strong relationship. In view of the increasing awareness about the prevalence of mild cognitive dysfunction in multiple system atrophy, the relevance of autonomic dysfunction in demented parkinsonian patients, the critical role of non-motor symptoms in clustering Parkinson's disease patients and the shift to studying patients in the prodromal phase, we will discuss some intrinsic limitations of current clinical diagnostic criteria, even when applied by movement disorder specialists. In particular, we will focus on the early coexistence of autonomic and cognitive dysfunction in the setting of overt or latent parkinsonism as pitfalls in the differential diagnosis of α-synucleinopathies. As early and accurate diagnosis remains of outmost importance for counselling of patients and timely enrolment into disease-modifying clinical trials, a continuous effort of research community is ongoing to further improve the clinical diagnostic accuracy of α-synucleinopathies.

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Neurological update: non-motor symptoms in atypical parkinsonian syndromes

Ananthavarathan,

Patel,

Peeros

et al. 2023

J Neurol

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Among people with Parkinson’s disease (PD), non-motor symptoms (NMS) are a well-recognised cause of significant morbidity and poor quality of life. Yet, it is only more recently that NMS have been recognised to affect the lives of patients with atypical parkinsonian syndromes in a similar fashion. The aim of this article is to highlight and compare the relative prevalence of NMS among patients with atypical parkinsonian syndromes in the published literature, which largely remain underreported and unaddressed in routine clinical practice. All NMS that are recognised to occur in PD are also found to commonly occur in atypical parkinsonian syndromes. In particular, excessive daytime sleepiness is more prevalent among atypical parkinsonian syndromes (94.3%) compared to PD (33.9%) or normal controls (10.5%) (p < 0.001). Urinary dysfunction (not limited to urinary incontinence) is not only found to occur in MSA (79.7%) and PD (79.9%), but has also been reported in nearly half of the patients with PSP (49.3%), DLB (42%) and CBD (53.8%) (p < 0.001). Apathy is significantly more common among the atypical parkinsonian syndromes [PSP (56%), MSA (48%), DLB (44%), CBD (43%)] compared to PD (35%) (p = 0.029). Early recognition and addressing of NMS among atypical parkinsonian syndromes may help improve the holistic patient care provided and may encompass a range of conservative and pharmacotherapeutic treatments to address these symptoms.

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Neuropsychological and clinical heterogeneity of cognitive impairment in patients with multiple system atrophy

Cited by 30 publications

References 36 publications

Cognition in multiple system atrophy: a single‐center cohort study

Cognition in multiple system atrophy: a single‐center cohort study

Early autonomic and cognitive dysfunction in PD, DLB and MSA: blurring the boundaries between α-synucleinopathies

Neurological update: non-motor symptoms in atypical parkinsonian syndromes

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