Cognitive Profile and Its Evolution in a Cohort of Multiple System Atrophy Patients

Introduction: Cognitive decline is not a characteristic feature of multiple system atrophy (MSA), but recent evidence suggests cognitive impairment as an integral part of the disease. We aim to describe the cognitive profile and its progression in a cohort of patients with MSA.Methods: We retrospectively selected patients referred to our department with a clinical diagnosis of MSA who were evaluated at least once a year during the course of the disease and underwent a comprehensive neuropsychological evaluation.Results: At the first evaluation (T0), 37 out of 60 patients (62%) were cognitively impaired, mainly (76%) in attention and executive functioning. Thirteen patients were impaired in one cognitive domain and 24 in more than one cognitive domain. Six out of the 24 had dementia. Twenty patients underwent a follow-up evaluation (T1) after a mean of 16.6 ± 9.3 months from the first evaluation (T0). Eight out of 20 patients were cognitively normal at both T0 and T1. Seven out of 12 patients presented with stable cognitive impairment at T1, while cognitive decline progressed in five patients. Patients with progression in cognitive decline performed significantly worse at T0 than cognitively stable patients. Education was significantly different between patients with and without cognitive impairment. No other differences in demographic and clinical variables and autonomic or sleep disturbances were found. Patients with dementia were older at disease onset and at T0 and had lower education and disease duration at T0 compared to those in other groups.Conclusions: In patients with MSA, we observed three different cognitive profiles: normal cognition, stable selective attention-executive deficits, and progressive cognitive deficits evolving to dementia. The detection of cognitive impairment in patients with suspected MSA suggests the need for comprehensive and longitudinal neuropsychological evaluation.

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Cognitive Issues in Pediatric Multiple Sclerosis

Brain Sciences ◽

10.3390/brainsci11040442 ◽

2021 ◽

Vol 11 (4) ◽

pp. 442

Author(s):

Emilio Portaccio ◽

Ermelinda De Meo ◽

Angelo Bellinvia ◽

Maria Pia Amato

Keyword(s):

Multiple Sclerosis ◽

Cognitive Impairment ◽

Cognitive Decline ◽

Pediatric Patients ◽

Leisure Activities ◽

Disease Onset ◽

Cognitive Profiles ◽

Brain Growth ◽

Network Activation ◽

Definition Of

Multiple sclerosis (MS) is one of the leading causes of disability in young adults. The onset of MS during developmental age makes pediatric patients particularly susceptible to cognitive impairment, resulting from both disease-related damage and failure of age-expected brain growth. Despite different test batteries and definitions, cognitive impairment has been consistently reported in approximately one-third of pediatric patients with MS. However, the lack of a uniform definition of cognitive impairment and the adoption of different test batteries have led to divergent results in terms of cognitive domains more frequently affected across the cohorts explored. This heterogeneity has hampered large international collaborative studies. Moreover, research aimed at the identification of risk factors (e.g., demographic, clinical, and radiological features) or protective factors (e.g., cognitive reserve, leisure activities) for cognitive decline is still scanty. Mood disorders, such as depression and anxiety, can be detected in these patients alongside cognitive decline or in isolation, and can negatively affect quality of life scores as well as academic performances. By using MRI, cognitive impairment was attributed to damage to specific brain compartments as well as to abnormal network activation patterns. However, multimodal MRI studies are still needed in order to assess the contribution of each MRI metric to cognitive impairment. Importantly, longitudinal studies have recently demonstrated failure of age-expected brain growth and of white matter (WM) and gray matter (GM) maturation plays a relevant role in determining cognitive dysfunction, in addition to MS-related direct damage. Whether these growth retardations might result in specific cognitive profiles according to the age at disease onset has not been studied, yet. A better characterization of cognitive profiles in pediatric MS patients, as well as the definition of neuroanatomical substrates of cognitive impairment and their longitudinal evolution are needed to develop efficient therapeutic strategies against cognitive impairment in this patient population.

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Clinical Profile of Cognitive Decline in Patients with Parkinson's Disease, Progressive Supranuclear Palsy, and Multiple System Atrophy

Journal of Neurosciences in Rural Practice ◽

10.4103/jnrp.jnrp_154_17 ◽

2017 ◽

Vol 08 (04) ◽

pp. 562-568 ◽

Cited By ~ 1

Author(s):

Dipti Gupta ◽

Anjani Kumar Sharma ◽

Naveen Kumar ◽

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Cognitive Impairment ◽

Statistical Analysis ◽

Multiple System Atrophy ◽

Cognitive Decline ◽

Progressive Supranuclear Palsy ◽

Verbal Fluency ◽

Cognitive Profile ◽

Significant Difference

ABSTRACT Background: There are very less data on the comparison between the cognitive profile in Parkinson's disease (PD) and Parkinson's-plus groups, especially in India. Aims: The aim of this study is to compare the cognitive profile across PD, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA) groups and compare them using Mini–Mental State Examination (MMSE), frontal assessment battery (FAB), and verbal fluency tests. Settings and Design: This was a cross-sectional study. Materials and Methods: MMSE, FAB, and verbal fluency tests were administered in a total of 73 patients constituting 22 patients in MSA, 26 patients in PD, and 25 patients in PSP group, respectively. Twenty-six participants both age- and gender-matched were enrolled in control group. Statistical Analysis: Statistical analysis was done using SPSS Version 20.0. Descriptive statistics were done to find out the mean and standard deviation of different variables. ANOVA was done for followed by post hoc Bonferroni test to assess the cognitive function in three groups. Results: ANOVA showed that there is a significant difference for MMSE scores (P = 0.038) being worse scores for PSP and maximum for MSA. A significant difference was found for FAB scores within three groups. There is a significant difference for FAB scores (P = 0.00003) being worse scores for PSP and highest scores obtained for PD. All the subtests of FAB test differed significantly except motor programming across MSA, PSP, and PD groups. Conclusions: Our data suggest that global cognitive impairment and executive dysfunction are worst in PSP among the three groups. Patients with MSA had significant cognitive decline as opposed to previous experience. FAB scores and verbal fluency tests are good tests to assess cognitive impairment in these diseases. Subsets of FAB score have significant differences but cannot help differentiating conclusively between these three diseases.

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Cognition in a multiple system atrophy series of cases from Argentina

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20140127 ◽

2014 ◽

Vol 72 (10) ◽

pp. 773-776 ◽

Cited By ~ 3

Author(s):

Emilia Gatto ◽

Ignacio Demey ◽

Ana Sanguinetti ◽

Virginia Parisi ◽

José Luis Etcheverry ◽

...

Keyword(s):

Cognitive Impairment ◽

Informed Consent ◽

Episodic Memory ◽

Multiple System Atrophy ◽

Small Sample Size ◽

Small Sample ◽

Cognitive Domain ◽

Cognitive Tests ◽

Cognitive Profiles ◽

Visuospatial Abilities

Cognitive dysfunction may occur in 17-40% of patients with multiple system atrophy (MSA). It has been suggested a milder cognitive impairment in cerebellar (MSA-C) than in parkinsonian variant (MSA-P). However, differences in cognitive profiles remain under discussion. Objective To evaluate cognitive features in a series of patients with “probable MSA” from Argentina. Method After informed consent was obtained, an extensive cognitive tests battery was administered. Nine patients (6 MSA-P and 3 MSA-C) composed the sample. Results Depression was detected in 43% of patients. Seven patients showed at least one cognitive domain impairment. Temporospatial orientation, visuospatial abilities, executive and attentional functions, episodic memory and language were compromised in MSA-P, while MSA-C dysfunction was restricted to attentional and executive domains. Conclusion Despite the small sample size, our findings could suggest a more widespread cognitive impairment in MSA-P than MSA-C.

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The cognitive profile of behavioural variant FTD and its similarities with ALS: a systematic review and meta-analysis

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2017-317459 ◽

2018 ◽

Vol 89 (9) ◽

pp. 995-1002 ◽

Cited By ~ 21

Author(s):

Emma Beeldman ◽

Joost Raaphorst ◽

Michelle Klein Twennaar ◽

Rosanne Govaarts ◽

Yolande A L Pijnenburg ◽

...

Keyword(s):

Cognitive Impairment ◽

Social Cognition ◽

Executive Functions ◽

Verbal Memory ◽

Cognitive Profile ◽

Effect Sizes ◽

Healthy Controls ◽

Cognitive Profiles ◽

Cognitive Domains ◽

Visual Comparison

Approximately 30% of patients with amyotrophic lateral sclerosis (ALS) have cognitive impairment and 8%–14% fulfil the criteria for behavioural variant frontotemporal dementia (bv-FTD). The cognitive profiles of ALS and bv-FTD have been reported to be comparable, but this has never been systematically investigated. We aimed to determine the cognitive profile of bv-FTD and examine its similarities with that of ALS, to provide evidence for the existence of a cognitive disease continuum encompassing bv-FTD and ALS. We therefore systematically reviewed neuropsychological studies on bv-FTD patients and healthy volunteers. Neuropsychological tests were divided in 10 cognitive domains and effect sizes were calculated for all domains and compared with the cognitive profile of ALS by means of a visual comparison and a Pearson’s r correlation coefficient. We included 120 studies, totalling 2425 bv-FTD patients and 2798 healthy controls. All cognitive domains showed substantial effect sizes, indicating cognitive impairment in bv-FTD patients compared to healthy controls. The cognitive domains with the largest effect sizes were social cognition, verbal memory and fluency (1.77–1.53). The cognitive profiles of bv-FTD and ALS (10 cognitive domains, 1287 patients) showed similarities on visual comparison and a moderate correlation 0.58 (p=0.13). When social cognition, verbal memory, fluency, executive functions, language and visuoperception were considered, i.e. the cognitive profile of ALS, Pearson’s r was 0.73 (p=0.09), which raised to 0.92 (p=0.03), when language was excluded in this systematic analysis of patients with a non-language subtype of FTD. The cognitive profile of bv-FTD consists of deficits in social cognition, verbal memory, fluency and executive functions and shows similarities with the cognitive profile of ALS. These findings support a cognitive continuum encompassing ALS and bv-FTD.

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Selecting the most important self-assessed features for predicting conversion to mild cognitive impairment with random forest and permutation-based methods

Scientific Reports ◽

10.1038/s41598-020-77296-4 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Jaime Gómez-Ramírez ◽

Marina Ávila-Villanueva ◽

Miguel Ángel Fernández-Blázquez

Keyword(s):

Cognitive Impairment ◽

Mild Cognitive Impairment ◽

Random Forest ◽

Cognitive Decline ◽

Social Life ◽

Healthy Aging ◽

Personal Information ◽

Disease Onset ◽

Subjective Cognitive Decline ◽

Comorbid Condition

AbstractAlzheimer’s Disease is a complex, multifactorial, and comorbid condition. The asymptomatic behavior in the early stages makes the identification of the disease onset particularly challenging. Mild cognitive impairment (MCI) is an intermediary stage between the expected decline of normal aging and the pathological decline associated with dementia. The identification of risk factors for MCI is thus sorely needed. Self-reported personal information such as age, education, income level, sleep, diet, physical exercise, etc. is called to play a key role not only in the early identification of MCI but also in the design of personalized interventions and the promotion of patients empowerment. In this study, we leverage a large longitudinal study on healthy aging in Spain, to identify the most important self-reported features for future conversion to MCI. Using machine learning (random forest) and permutation-based methods we select the set of most important self-reported variables for MCI conversion which includes among others, subjective cognitive decline, educational level, working experience, social life, and diet. Subjective cognitive decline stands as the most important feature for future conversion to MCI across different feature selection techniques.

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BDNF serum levels are not related to cognitive functioning in older depressed patients and controls

International Psychogeriatrics ◽

10.1017/s1041610214002622 ◽

2014 ◽

Vol 27 (4) ◽

pp. 649-656 ◽

Cited By ~ 7

Author(s):

Annemiek Dols ◽

Carisha S. Thesing ◽

Filip Bouckaert ◽

Richard C. Oude Voshaar ◽

Hannie C. Comijs ◽

...

Keyword(s):

Cognitive Impairment ◽

Cognitive Decline ◽

Cognitive Functioning ◽

Older Persons ◽

Serum Levels ◽

Analysis Of Covariance ◽

Cognitive Domain ◽

Depressed Patients ◽

Serum Bdnf ◽

Low Serum

ABSTRACTBackground:Depression and cognitive decline are highly prevalent in older persons and both are associated with low serum brain derived neurotrophic factor (BDNF). Mutual pathways of depression and cognitive decline in older persons may explain the overlap in symptoms and low serum BDNF. We hypothesized that serum BDNF levels are lower in depressed elderly with poor cognitive performance (global or specifically in working memory, speed of information processing, and episodic memory) compared to depressed elderly without cognitive impairment or non-depressed controls.Methods:BDNF Serum levels and cognitive functioning were examined in 378 depressed persons and 132 non-depressed controls from a large prospective study on late-life depression. The association between BDNF levels and each cognitive domain among the depressed patients was tested by four separate linear regression models adjusted for relevant covariates. An analysis of covariance (ANCOVA) was performed to compare BDNF serum levels in three groups (depression with cognitive impairment, depression without cognitive impairment, and non-depressed controls), when adjusted for potential confounders.Results:No significant linear association was found between BDNF and any of the four cognitive domains tested. There are no differences in BDNF levels between controls and depressed patients with or without cognitive impairment global or in specific domains after controlling for confounders.Conclusions:BDNF serum levels in this cohort of older depressed patients and controls are not related to cognitive functioning. As BDNF is essential for the survival and functioning of neurons, its levels may remain normal in stages of disease where remission is achievable.

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INFLUENCE OF SLEEP DISTURBANCES ON COGNITIVE DECLINE IN PATIENTS WITH PARKINSON’S DISEASE

Ukrainian Scientific Medical Youth Journal ◽

10.32345/usmyj.3(117).2020.58-67 ◽

2020 ◽

Vol 117 (3) ◽

pp. 58-67

Author(s):

Anastasiia Shkodina ◽

Kateryna Tarianyk ◽

Dmytro Boiko

Keyword(s):

Parkinson’S Disease ◽

Parkinson's Disease ◽

Cognitive Impairment ◽

Cognitive Decline ◽

Sleep Disorders ◽

Sleep Disturbances ◽

Rating Scale ◽

Early Stages ◽

The Impact

The article summarizes the arguments and counter-arguments within the scientific discussion on the impact of sleep disorders on the development of cognitive decline in patients with Parkinson's disease. The main purpose of the study is to study the possibility of predicting the development of cognitive decline by assessing the severity of sleep disorders and their differences in the presence of cognitive impairment. Systematization of literature sources and approaches to solving the problem showed that sleep disorders develop in the early stages of Parkinson's disease and are often accompanied by cognitive impairment. Cognitive decline is manifested throughout Parkinson's disease and ranges from moderate in the early stages to dementia in the late stages. The relevance of the study of the relationship between sleep disorders and cognitive functions lies in the possibility of further improving the prediction of the development of cognitive decline in order to effectively correct it. Treatment of sleep disorders can be accompanied by improved memory and even morphological changes in the brain. Therefore, the question arises about the possibility of correcting cognitive decline by influencing sleep disorders. The methodology of the study included assessment of the overall status of patients on a unified scale of Parkinson's disease, Montreal cognitive rating scale and sleep scale in Parkinson's disease. The duration of the study was 8 months. Patients with Parkinson's disease were selected as the study. The article presents the results of a survey of patients who show that patients with Parkinson's disease and cognitive decline showed a predominance of motor disorders, sleep disorders and the overall score on the sleep scale in Parkinson's disease. In the presence of cognitive decline more pronounced disorders of motor functions in everyday life, which can lead to sleep disorders and its quality. The study empirically confirms and theoretically proves that the assessment of sleep disorders can be used to predict the risk of developing cognitive impairment in patients with Parkinson's disease. The results of this study may be useful for improving the early diagnosis and prevention of cognitive impairment in patients with Parkinson's disease, which, in turn, leads to improved quality of treatment of these patients. Such changes can directly affect the choice of therapeutic tactics and improve the quality of life of patients with Parkinson's disease. The question of the features of various sleep disorders and their prognostic value in relation to cognitive decline in patients with various forms of Parkinson's disease remains open.

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Variable course of Unverricht-Lundborg disease

Neurology ◽

10.1212/wnl.0000000000004518 ◽

2017 ◽

Vol 89 (16) ◽

pp. 1691-1697 ◽

Cited By ~ 8

Author(s):

Laura Canafoglia ◽

Edoardo Ferlazzo ◽

Roberto Michelucci ◽

Pasquale Striano ◽

Adriana Magaudda ◽

...

Keyword(s):

Risk Factors ◽

Cognitive Impairment ◽

Cognitive Decline ◽

Time Course ◽

Proportional Hazards ◽

Cell Damage ◽

Age At Onset ◽

Disease Onset ◽

Rank Test ◽

Genetically Determined

Objective:To explore the course of Unverricht-Lundborg disease (EPM1) and identify the risk factors for severity, we investigated the time course of symptoms and prognostic factors already detectable near to disease onset.Methods:We retrospectively evaluated the features of 59 Italian patients carrying the CSTB expansion mutation, and coded the information every 5 years after the disease onset in order to describe the cumulative time-dependent probability of reaching disabling myoclonus, relevant cognitive impairment, and inability to work, and evaluated the influence of early factors using the log-rank test. The risk factors were included in a Cox multivariate proportional hazards regression model.Results:Disabling myoclonus occurred an average of 32 years after disease onset, whereas cognitive impairment occurred a little later. An age at onset of less than 12 years, the severity of myoclonus at the time of first assessment, and seizure persistence more than 10 years after onset affected the timing of disabling myoclonus and cognitive decline. Most patients became unable to work years before the appearance of disabling myoclonus or cognitive decline.Conclusions:A younger age at onset, early severe myoclonus, and seizure persistence are predictors of a more severe outcome. All of these factors may be genetically determined, but the greater hyperexcitability underlying more severe seizures and myoclonus at onset may also play a role by increasing cell damage due to reduced cystatin B activity.

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Self-reported sleep disturbances are associated with poorer cognitive performance in older adults with hypertension: a multi-parameter risk factor investigation

International Psychogeriatrics ◽

10.1017/s1041610219001492 ◽

2019 ◽

Vol 32 (7) ◽

pp. 815-825 ◽

Cited By ~ 2

Author(s):

Jordan N. Kohn ◽

Emily Troyer ◽

Robert N. Guay-Ross ◽

Kathleen Wilson ◽

Amanda Walker ◽

...

Keyword(s):

Risk Factors ◽

Older Adults ◽

Logistic Regression ◽

Cognitive Impairment ◽

Cognitive Performance ◽

Sleep Disturbances ◽

Depressive Symptomatology ◽

Cognitive Outcomes ◽

Cognitive Domain ◽

Increased Risk

ABSTRACTObjectives:Given the evidence of multi-parameter risk factors in shaping cognitive outcomes in aging, including sleep, inflammation, cardiometabolism, and mood disorders, multidimensional investigations of their impact on cognition are warranted. We sought to determine the extent to which self-reported sleep disturbances, metabolic syndrome (MetS) factors, cellular inflammation, depressive symptomatology, and diminished physical mobility were associated with cognitive impairment and poorer cognitive performance.Design:This is a cross-sectional study.Setting:Participants with elevated, well-controlled blood pressure were recruited from the local community for a Tai Chi and healthy-aging intervention study.Participants:One hundred forty-five older adults (72.7 ± 7.9 years old; 66% female), 54 (37%) with evidence of cognitive impairment (CI) based on Montreal Cognitive Assessment (MoCA) score ≤24, underwent medical, psychological, and mood assessments.Measurements:CI and cognitive domain performance were assessed using the MoCA. Univariate correlations were computed to determine relationships between risk factors and cognitive outcomes. Bootstrapped logistic regression was used to determine significant predictors of CI risk and linear regression to explore cognitive domains affected by risk factors.Results:The CI group were slower on the mobility task, satisfied more MetS criteria, and reported poorer sleep than normocognitive individuals (all p < 0.05). Multivariate logistic regression indicated that sleep disturbances, but no other risk factors, predicted increased risk of evidence of CI (OR = 2.00, 95% CI: 1.26–4.87, 99% CI: 1.08–7.48). Further examination of MoCA cognitive subdomains revealed that sleep disturbances predicted poorer executive function (β = –0.26, 95% CI: –0.51 to –0.06, 99% CI: –0.61 to –0.02), with lesser effects on visuospatial performance (β = –0.20, 95% CI: –0.35 to –0.02, 99% CI: –0.39 to 0.03), and memory (β = –0.29, 95% CI: –0.66 to –0.01, 99% CI: –0.76 to 0.08).Conclusions:Our results indicate that the deleterious impact of self-reported sleep disturbances on cognitive performance was prominent over other risk factors and illustrate the importance of clinician evaluation of sleep in patients with or at risk of diminished cognitive performance. Future, longitudinal studies implementing a comprehensive neuropsychological battery and objective sleep measurement are warranted to further explore these associations.

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Challenges in Predicting Cognitive Decline in Dementia with Lewy Bodies

Dementia and Geriatric Cognitive Disorders ◽

10.1159/000515008 ◽

2021 ◽

pp. 1-8

Author(s):

Konstantinos Tsamakis ◽

Christoph Mueller

Keyword(s):

Cognitive Impairment ◽

Clinical Practice ◽

Cognitive Decline ◽

Dementia With Lewy Bodies ◽

Sleep Disturbances ◽

Neuropsychiatric Symptoms ◽

Lewy Bodies ◽

Assessment Tools ◽

Disease Course ◽

The Impact

Despite being the second most common form of neurodegenerative dementia, dementia with Lewy bodies (DLB) is under-recognized and carries a worse prognosis than other subtypes of the condition. Cognitive impairment is a cardinal feature of all types of dementia and DLB presents with a distinct profile with deficits in attention, executive function, and visuoperceptual abilities. This difference from Alzheimer’s disease and the common presence of neuropsychiatric symptoms may lead to challenges in predicting cognitive decline in this patient population. Firstly, the diagnosis of DLB is often delayed in clinical practice leading to variability from which time point in the disease course cognitive decline is measured. Secondly, the most frequently used measurement tools for cognitive difficulties focus on memory and naming rather than the domains affected by DLB. While there is now largely a consensus which tools are useful in diagnosing DLB, their validity in assessing deteriorating cognition is less clear. Thirdly, the presence of fluctuating cognition, the propensity to develop delirium episodes, as well as difficulties in distinguishing the two entities in clinical practice make it difficult to predict the disease course. Sleep disturbances are likely to influence cognitive decline but require further study in patients within established DLB. Fourthly, as in most cases of dementia, neuropathological comorbidities are frequently present in DLB. While the influence of Alzheimer’s pathology on cognitive decline in DLB is comparatively well understood, the impact of other pathologies remains unclear. The recent definition of research criteria for mild cognitive impairment in DLB could facilitate earlier diagnosis and more structured follow-up. Assessment tools measuring cognitive domains predominantly affected in DLB need to be more consistently used in longitudinal studies and clinical practice, as well as concurrent measures of fluctuations in cognition. Greater availability of biomarkers and digital healthcare solutions can play an important role in enabling more accurate monitoring and prediction of cognitive decline in DLB.

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