scholarly journals Cognitive Impairment Involving Social Cognition in SPG4 Hereditary Spastic Paraplegia

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Ludivine Chamard ◽  
Sabrina Ferreira ◽  
Alexa Pijoff ◽  
Manon Silvestre ◽  
Eric Berger ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Social Cognition ◽  
Motor Skills ◽  
Disease Onset ◽  
Motor Impairment ◽  
Cognitive Disorders ◽  
Small Sample ◽  
Spastic Paraplegia ◽  
Cognition Impairment ◽  
Lateral Sclerosis

Objectives. To describe cognitive assessment including social cognition in SPG4 patients.Methods. We reported a series of nine patients with SPG4 mutation with an extensive neuropsychological examination including social cognition assessment.Results. None of our patients presented with mental retardation or dementia. All presented with mild cognitive impairment with a high frequency of attention deficit (100%), executive disorders (89%), and social cognition impairment (78%). An asymptomatic patient for motor skills presented with the same cognitive profile. No correlation was found in this small sample between cognitive impairment and motor impairment, age at disease onset, or disease duration.Conclusions. SPG4 phenotypes share some cognitive features of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Cognitive disorders including executive disorders and social cognition impairment are frequent in SPG4 patients and might sometimes occur before motor disorders. Therefore, cognitive functions including social cognition should be systematically assessed in order to improve the clinical management of this population.

Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder

2020 ◽  
Vol 91 (12) ◽  
pp. 1279-1282
Author(s):  
Patricia Lillo ◽  
Paulo Caramelli ◽  
Gada Musa ◽  
Teresa Parrao ◽  
Ricardo Hughes ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Social Cognition ◽  
Digit Span ◽  
Short Version ◽  
The Social ◽  
Significant Difference ◽  
Similar Disease ◽  
Post Hoc ◽  
Lateral Sclerosis

ObjectiveTo compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).MethodsWe included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the faux pas test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8).ResultsNo significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the faux pas (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls.DiscussionOur findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

scholarly journals Employers’ Response to Workers With Progressive Cognitive Impairment: A Systematic Literature Review

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. 717-718
Author(s):  
Josephine McMurray ◽  
AnneMarie Levy ◽  
Logan Reis ◽  
Kristina Kokorelias ◽  
Jennifer Boger ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Decline ◽  
Performance Management ◽  
Disease Onset ◽  
Cognitive Disorders ◽  
Quality Of Work Life ◽  
Aging Workforce ◽  
Early Onset Dementia ◽  
Disability Accommodation

Abstract An aging workforce increases the risk of workers experiencing cognitive decline that may lead to a diagnosis of mild cognitive impairment or early onset dementia (MCI|EOD) while still employed. This systematic review explores the use of technologies (defined as any methods, processes, software, hardware or equipment) deployed by employers to accommodate, or build sustainable workspaces for, workers diagnosed with MCI|EOD. After screening 3,860 titles/abstracts and 67 full text reviews, we identified and analyzed eight articles that met our inclusion criteria. We found that: 1) The existing literature almost exclusively focuses on employees’ perspectives on the quality of work life when diagnosed with MCI|EOD, 2) Negative workspace culture toward employees’ cognitive decline, and the variability of disease onset and progression, may account for low employer awareness, 3) Employer responses focus on mitigation of risk associated with workers’ impairment. While this review demonstrates there is scant research exploring employers’ perspectives on employees diagnosed with MCI|EOD, there is even less that explores technologies designed to specifically address employers’ needs and challenges. Technology will increasingly facilitate early identification of progressive neuro-cognitive disorders, and tools to help employers respond to an employee’s MCI|EOD disclosure as a disability accommodation rather than a terminal performance management challenge. Empathic research, that engages organizations in the process of understanding the value of affordable, employer-side technologies that help build diverse, sustainable, productive workspaces is critical to a foundational understanding of our aging workforce and accommodating workers who develop MCI|EOD while still employed.

N-Acetylcysteine Attenuates the Anxiety-Like Behavior and Spatial Cognition Impairment Induced by Doxorubicin and Cyclophosphamide Combination Treatment in Rats

Pharmacology ◽  
2020 ◽  
pp. 1-8
Author(s):  
Yoshihisa Kitamura ◽  
Soichiro Ushio ◽  
Yusuke Sumiyoshi ◽  
Yudai Wada ◽  
Ikuko Miyazaki ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Cognitive Impairment ◽  
Spatial Cognition ◽  
Combination Treatment ◽  
Combined Treatment ◽  
Cognitive Disorders ◽  
The Novel ◽  
Location Recognition ◽  
Anti Oxidant ◽  
Cognition Impairment

<b><i>Background:</i></b> Cancer patients can suffer from psychological and cognitive disorders after chemotherapy, which influence quality of life. <b><i>Objective:</i></b> Oxidative stress may contribute to the psychological and cognitive disorders induced in rats by chemotherapy. In the present study, we examined the effects of N-acetylcysteine, an anti-oxidant, on anxiety-like behavior and cognitive impairment in rats treated with a combination of doxorubicin and cyclophosphamide. <b><i>Methods:</i></b> Rats were intraperitoneally injected with doxorubicin and cyclophosphamide once a week for 2 weeks. The light-dark test and the novel location recognition test were used to assess anxiety-like behavior and spatial cognition, respectively. The rats’ hippocampal levels of glutathione (GSH) and glutathione disulfide (GSSG) were measured using a GSSG/GSH quantification kit. <b><i>Results:</i></b> Combined treatment with doxorubicin and cyclophosphamide produced anxiety-like behavior and cognitive impairment in rats. N-acetylcysteine reversed the anxiety-like behavior and inhibition of novel location recognition induced by the combination treatment. Furthermore, the combination of doxorubicin and cyclophosphamide significantly reduced the rats’ hippocampal GSH/GSSG ratios. N-acetylcysteine reversed the reduction in the GSH/GSSG ratio seen in the doxorubicin and cyclophosphamide-treated rats. <b><i>Conclusion:</i></b> These results suggest that N-acetylcysteine inhibits doxorubicin and cyclophosphamide-induced anxiety-like behavior and cognitive impairment by reducing oxidative stress in the hippocampus.

scholarly journals Absence of subcerebral projection neurons delays disease onset and extends survival in a mouse model of ALS

2019 ◽  
Author(s):  
Thibaut Burg ◽  
Charlotte Bichara ◽  
Jelena Scekic-Zahirovic ◽  
Mathieu Fischer ◽  
Geoffrey Stuart-Lopez ◽  
...  
Keyword(s):  
Mouse Model ◽  
Disease Duration ◽  
Disease Onset ◽  
Motor Impairment ◽  
Projection Neurons ◽  
Direct Test ◽  
Muscle Denervation ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Motor Neuron Syndrome

AbstractAmyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease of adulthood that affects voluntary motricity and rapidly leads to full paralysis and death. ALS arises from the combined degeneration of motoneurons in the spinal cord and brain stem, responsible for muscle denervation, and corticospinal projection neurons (CSN), responsible for emergence of the upper motor neuron syndrome. Recent studies carried on ALS patients suggest that the disease may initiate in the motor cortex and spread to its projection targets. However, this “corticofugal hypothesis” of ALS has not yet been specifically challenged. Here, we provide a direct test of this hypothesis by genetically removing subcerebral projection neurons (SubCerPN), including CSN, in Sod1G86R mice, a mouse model of ALS. Ablation of the transcription factor Fezf2, leading to the complete absence of all SubCerPN, delays disease onset, reduces weight loss and motor impairment, and increases survival without modifying disease duration. Importantly absence of SubCerPN and CSN also limits pre-symptomatic hyperreflexia. Together, our results demonstrate that major corticofugal tracts are critical to ALS onset, and that SubCerPN and CSN in particular may carry detrimental signals to their downstream targets. In its whole, this study provides first experimental arguments in favour of the corticofugal hypothesis of ALS.

scholarly journals Executive dysfunction predicts social cognition impairment in amyotrophic lateral sclerosis

2015 ◽  
Vol 262 (7) ◽  
pp. 1681-1690 ◽  
Author(s):  
Tamlyn J. Watermeyer ◽  
Richard G. Brown ◽  
Katie C. L. Sidle ◽  
David J. Oliver ◽  
Christopher Allen ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Social Cognition ◽  
Executive Dysfunction ◽  
Cognition Impairment ◽  
Lateral Sclerosis

Cognitive Performance and Neuro-Metabolites in HIV using 3T Magnetic Resonance Spectroscopy: A Cross-Sectional Study from India

2020 ◽  
Vol 18 ◽  
Author(s):  
Kartik Gupta ◽  
Shivabalan ◽  
Virendra Kumar ◽  
Surabhi Vyas ◽  
RM Pandey ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Magnetic Resonance ◽  
Magnetic Resonance Spectroscopy ◽  
Motor Skills ◽  
Cross Sectional Study ◽  
Resonance Spectroscopy ◽  
Sectional Study ◽  
Cross Sectional ◽  
Total N ◽  
Simple Motor

Background: Cognitive impairment in patients with human immunodeficiency virus (HIV) is associated with higher morbidity. The prevalence of and the metabolite changes in the brain associated with cognitive impairment in anti-retroviral therapy naïve patients with HIV is unknown. Objective: To estimate the prevalence of, and the neurometabolites associated with cognitive impairment in antiretroviral therapy (ART) naïve patients with HIV. Methods: We conducted a cross-sectional study among ART naïve patients with HIV aged 18-50 years in a tertiary care center in India. Cognition was tested using the Post Graduate Institute battery of brain dysfunction across five domains; memory, attentioninformation processing, abstraction executive, complex perceptual, and simple motor skills. We assessed the total N-acetyl aspartyl (tNAA), creatine (tCr) and glutamate + glutamine (Glx) using 3T magnetic resonance spectroscopy. Cognitive impairment was defined as an impairment in ≥2 domains. Results: Among 43 patients eligible for this study, the median age was 32 years (IQR 29, 40) and 30% were women. Median CD4 count and viral load were 317 cells/µL (IQR 157, 456) and 9.3 copies/ µL (IQR 1.4, 38), respectively. Impairment in at least one cognitive domain was present in 32 patients (74.4%). Impairment in simple motor skills and memory was present in 46.5% and 44% of patients, respectively. Cognitive impairment, defined by impairment in ≥2 domains, was found in 22 (51.2%) patients. There was a trend towards higher concentration of tNAA (7.3 vs. 7.0 mmol/kg), tGlx (9.1 vs. 8.2 mmol/kg), and tCr (5.5 vs. 5.2 mmol/kg) in the frontal lobe of patients with cognitive impairment vs. without cognitive impairment but it did not reach statistical significance (p>0.05 for all). There was no difference in the concentration of these metabolites in the two groups in the basal ganglia. Conclusions: There is a high prevalence of cognitive impairment in ART naïve patients with HIV. There is no difference in metabolites in patients with or without cognitive impairment. Further studies, with longitudinal follow-up, are required to understand the underlying pathophysiological mechanisms.

MHealth Applications for People with Cognitive Impairment and Their Caregivers: A Scoping Review (Preprint)

2020 ◽  
Author(s):  
Qing Zhao ◽  
Pei Chen ◽  
Yu Zhang ◽  
Haining Liu ◽  
Xianwen Li
Keyword(s):  
Cognitive Impairment ◽  
Sample Size ◽  
Research Design ◽  
Scoping Review ◽  
Life Support ◽  
Small Sample ◽  
Mobile Technologies ◽  
Control Group ◽  
Caregiver Support ◽  
The Impact

BACKGROUND Mobile health application has become an important tool for healthcare systems. One such tool is the delivery of assisting in people with cognitive impairment and their caregivers. OBJECTIVE This scoping review aims to explore and evaluate the existing evidence and challenges on the use of mHealth applications that assisting in people with cognitive impairment and their caregivers. METHODS Nine databases, including PubMed, EMBASE, Cochrane, PsycARTICLES, CINAHL, Web of Science, Applied Science & Technology Source, IEEE Xplore and the ACM Digital Library were searched from inception through June 2020 for the studies of mHealth applications on people with cognitive impairment and their caregivers. Two reviewers independently extracted, checked synthesized data independently. RESULTS Of the 6101 studies retrieved, 64 studies met the inclusion criteria. Three categories emerged from this scoping review. These categories are ‘application functionality’, ‘evaluation strategies’, ‘barriers and challenges’. All the included studies were categorized into 7 groups based on functionality: (1) cognitive assessment; (2) cognitive training; (3) life support; (4) caregiver support; (5) symptom management; (6) reminiscence therapy; (7) exercise intervention. The included studies were broadly categorized into four types: (1) Usability testing; (2) Pilot and feasibility studies; (3) Validation studies; and (4) Efficacy or Effectiveness design. These studies had many defects in research design such as: (1) small sample size; (2) deficiency in active control group; (3) deficiency in analyzing the effectiveness of intervention components; (4) lack of adverse reactions and economic evaluation; (5) lack of consideration about the education level, electronic health literacy and smartphone proficiency of the participants; (6) deficiency in assessment tool; (7) lack of rating the quality of mHealth application. Some progress should be improved in the design of smartphone application functionality, such as: (1) the design of cognitive measurements and training game need to be differentiated; (2) reduce the impact of the learning effect. Besides this, few studies used health behavior theory and performed with standardized reporting. CONCLUSIONS Preliminary results show that mobile technologies facilitate the assistance in people with cognitive impairment and their caregivers. The majority of mHealth application interventions incorporated usability outcome and health outcomes. However, these studies have many defects in research design that limit the extrapolation of research. The content of mHealth application is urgently improved to adapt to demonstrate the real effect. In addition, further research with strong methodological rigor and adequate sample size are needed to examine the feasibility, effectiveness, and cost-effectiveness of mHealth applications for people with cognitive impairment and their caregivers.

The Oxford Handbook of Adult Cognitive Disorders

2019 ◽  
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Function ◽  
Neurodevelopmental Disorders ◽  
Brain Aging ◽  
Cognitive Disorders ◽  
Diverse Range ◽  
Medical Specialties ◽  
Psychiatric Illnesses ◽  
Medical Disorders ◽  
The Impact

The prevalence of cognitive impairment caused by neurodegenerative diseases and other neurologic disorders associated with aging is expected to rise dramatically between now and year 2050, when the population of Americans aged 65 or older will nearly double. Cognitive impairment also commonly occurs in other neurologic conditions, as well as in non-neurologic medical disorders (and their treatments), idiopathic psychiatric illnesses, and adult neurodevelopmental disorders. Cognitive impairment can thus infiltrate all aspects of healthcare, making it necessary for clinicians and clinical researchers to have an integrated knowledge of the spectrum of adult cognitive disorders. The Oxford Handbook of Adult Cognitive Disorders is meant to serve as an up-to-date, scholarly, and comprehensive volume covering most diseases, conditions, and injuries resulting in impairments in cognitive function in adults. Topics covered include normal cognitive and brain aging, the impact of medical disorders (e.g., cardiovascular, liver, pulmonary) and psychiatric illnesses (e.g., depression and bipolar disorder) on cognitive function, adult neurodevelopmental disorders (e.g., Down Syndrome, Attention Deficit/Hyperactivity Disorder), as well as the various neurological conditions (e.g., Alzheimer’s disease, chronic traumatic encephalopathy, concussion). A section of the Handbook is also dedicated to unique perspectives and special considerations for the clinicians and clinical researchers, covering topics such as cognitive reserve, genetics, diversity, and neuroethics. The target audience of this Handbook includes: (1) clinicians, particularly psychologists, neuropsychologists, neurologists (including behavioral and cognitive neurologists), geriatricians, and psychiatrists (including neuropsychiatrists), who provide clinical care and management for adults with a diverse range of cognitive disorders; (2) clinical researchers who investigate cognitive outcomes and functioning in adult populations; and (3) graduate level students and post-doctoral trainees studying psychology, clinical neuroscience, and various medical specialties.

scholarly journals Lipidomics study of plasma from patients suggest that ALS and PLS are part of a continuum of motor neuron disorders

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Estela Area-Gomez ◽  
D. Larrea ◽  
T. Yun ◽  
Y. Xu ◽  
J. Hupf ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neurons ◽  
Cell Structure ◽  
Disease Onset ◽  
Point Of View ◽  
Motor Dysfunction ◽  
Cellular Processes ◽  
Progressive Muscle Weakness ◽  
Human Pathology ◽  
Lateral Sclerosis

AbstractMotor neuron disorders (MND) include a group of pathologies that affect upper and/or lower motor neurons. Among them, amyotrophic lateral sclerosis (ALS) is characterized by progressive muscle weakness, with fatal outcomes only in a few years after diagnosis. On the other hand, primary lateral sclerosis (PLS), a more benign form of MND that only affects upper motor neurons, results in life-long progressive motor dysfunction. Although the outcomes are quite different, ALS and PLS present with similar symptoms at disease onset, to the degree that both disorders could be considered part of a continuum. These similarities and the lack of reliable biomarkers often result in delays in accurate diagnosis and/or treatment. In the nervous system, lipids exert a wide variety of functions, including roles in cell structure, synaptic transmission, and multiple metabolic processes. Thus, the study of the absolute and relative concentrations of a subset of lipids in human pathology can shed light into these cellular processes and unravel alterations in one or more pathways. In here, we report the lipid composition of longitudinal plasma samples from ALS and PLS patients initially, and after 2 years following enrollment in a clinical study. Our analysis revealed common aspects of these pathologies suggesting that, from the lipidomics point of view, PLS and ALS behave as part of a continuum of motor neuron disorders.

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