DIAGNOSIS OF CHILDREN WITH SANFILIPPO DISEASE – PSYCHOLOGICAL, SOCIAL AND MOTOR ASSESSMENT

2020 ◽  
Vol 18 (4) ◽  
pp. 525-535
Author(s):  
Paulina Anikiej-Wiczenbach ◽  
Agata Rudnik ◽  
Monika Limanówka ◽  
Jolanta Wierzba ◽  
Arkadiusz Mański
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Functioning ◽  
Somatic Symptoms ◽  
Motor Dysfunction ◽  
Daily Functioning ◽  
Following Instructions ◽  
Neurological Effects ◽  
Sanfilippo Disease ◽  
Mps Iiia ◽  
Independent Person

Sanfilippo disease (mucopolysaccharidosis, MPS IIIA) is one of the types of mucopolysaccharidosis associated with extensive neurological effects and somatic symptoms. The consequences of neurodegeneration and cognitive impairment are manifested in challenges with the daily functioning of patients who experience problems with communication and following instructions. The aim of this study was to assess the cognitive functioning of three patients with MPS IIIA and to find patterns of neurodegeneration and to make their environment more friendly. Three boys (from 5.5 to 7 years) with MPS IIIA participated in the study. Each participant attended two meetings, and his functioning was assessed by three independent person (using two-way mirror). We used Bayley’s Scale III with some modifications. Interviews with parents were also included. The communication of patients was limited to some vocalizations. Patients presented instrumental use of items, but not all of them were able to repeat actions after diagnostician or presented object permanence. The results showed that the cognitive functioning of participants was significantly hindered by problems related to motor dysfunction, hyperactivity, and ataxia. The psychological data was collated with medical results. This study allows indicating new sources giving the possibility of child phenotype variability and to create specific interventions in the field of psychological therapy for patients with MPS IIIA and their families.

Constipation is Associated with Development of Cognitive Impairment in de novo Parkinson’s Disease: A Longitudinal Analysis of Two International Cohorts

2021 ◽  
pp. 1-11
Author(s):  
Valentina Leta ◽  
Daniele Urso ◽  
Lucia Batzu ◽  
Daniel Weintraub ◽  
Nataliya Titova ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Cognitive Impairment ◽  
De Novo ◽  
Motor Dysfunction ◽  
Group Differences ◽  
Motor Symptoms ◽  
Kaplan Meier ◽  
Patients At Risk ◽  
Non Motor Symptoms

Background: Constipation is regarded as one of the prodromal features of Parkinson’s disease (PD) and there is emerging evidence linking gastrointestinal dysfunction and cognitive impairment (CI) in PD. Objective: We explored whether constipation is associated with development of CI in two independent cohorts of de novo PD patients (n = 196 from the Non-motor International Longitudinal Study [NILS] and n = 423 from the Parkinson’s Progression Markers Initiative [PPMI] study). Methods: Constipation was clinically defined using the Non-Motor Symptoms Scale (NMSS) item-21 [NILS] and Scales for Outcomes in PD-Autonomic (SCOPA-AUT) item-5 [PPMI]. We assessed baseline group differences (PD with or without constipation) in CI, global non-motor symptoms burden, motor dysfunction, and striatal dopaminergic denervation. Kaplan-Meier method estimated group differences in cumulative proportion of patients with incident CI over three years. In PPMI, we subsequently performed univariate and multivariate Cox survival analyses to evaluate whether constipation predicts incident mild cognitive impairment or dementia over a 6-year period, including constipation and other known predictors of CI as covariates. Results: Patients with constipation had greater motor and global non-motor burden in both cohorts at baseline (p <  0.05). Kaplan-Meier plots showed faster conversion to CI in patients with constipation in both cohorts (p <  0.05). In PPMI, 37 subjects developed dementia during a mean follow-up of 4.9 years, and constipation was an independent predictor of dementia onset (hazard ratio = 2.311; p = 0.02). Conclusion: Constipation in de novo PD patients is associated with development of cognitive decline and may serve as a clinical biomarker for identification of patients at risk for cognitive impairment.

scholarly journals The Association between Physical Activity and Selected Parameters of Psychological Status and Dementia in Older Women

2021 ◽  
Vol 18 (14) ◽  
pp. 7549
Author(s):  
Angelika Cisek-Woźniak ◽  
Kinga Mruczyk ◽  
Rafał W. Wójciak
Keyword(s):  
Mental Health ◽  
Physical Activity ◽  
Cognitive Impairment ◽  
Cognitive Functioning ◽  
Cognitive Functions ◽  
Protective Factor ◽  
The Elderly ◽  
Regular Physical Activity ◽  
Psychological Status ◽  
The Impact

Physical activity has an unquestionable impact on broadly understood human health. One interesting issue related to this is the importance of movement on mental health and cognitive functioning. Research shows that regular physical activity improves the cognitive functioning of adults and people with mental disorders. Regular physical activity can be an important and powerful protective factor in cognitive impairment and dementia in the elderly, and exercise is an important non-pharmacological treatment for mild cognitive impairment or neurodegenerative diseases. This study aims to present the impact of physical activity on selected cognitive functions in physically active women over 60 years of age. The research was carried out in a group of 110 generally healthy women from the area of western Poland over 60 years of age, who were divided into four groups based on the intensity of their physical activity. A pedometer (sport watch) and a physical activity diary were used to measure physical activity. Body Mass Index was assessed. Selected cognitive functions were assessed using the MMSE test, motor and psychomotor skills were measured, and Luria’s auditory memory test and recall test, a clock drawing test, and a GDS test were performed. There were statistically significant relationships between the level of physical activity and the effectiveness of cognitive processes. These results show that about 5000 steps a day is enough to see a positive effect on the mental health and cognitive functioning of this group of the elderly population. The women had an average BMI of 28.1 ± 4.7. BMI, indicating an overweight condition (over 30 kg/m2), was observed in 31% of women. The results of this study lead the authors to conclude that physical activity positively influences cognitive function and can be recommended for all seniors who do not have other serious comorbidities that would prevent them from playing sports.

scholarly journals Motor Dysfunction in Mild Cognitive Impairment and the Risk of Incident Alzheimer Disease

2006 ◽  
Vol 63 (12) ◽  
pp. 1763 ◽  
Author(s):  
Neelum T. Aggarwal ◽  
Robert S. Wilson ◽  
Todd L. Beck ◽  
Julia L. Bienias ◽  
David A. Bennett
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Alzheimer Disease ◽  
Motor Dysfunction

Cognitive Functioning in Acute and Remitted Psychiatric Patients

1967 ◽  
Vol 21 (1) ◽  
pp. 24-26 ◽  
Author(s):  
Robert H. Goldstein ◽  
Leonard F. Salzman
Keyword(s):  
Cognitive Impairment ◽  
Hospital Admission ◽  
Cognitive Functioning ◽  
Clinical Remission ◽  
Psychiatric Patients ◽  
Discharge Home ◽  
Schizophrenic Patients

Tests of vocabulary and abstraction administered to groups of schizophrenic and non-schizophrenic patients at time of hospital admission and at time of clinical remission and discharge home revealed some evidence of general cognitive impairment in both groups, with significant improvement by time of discharge, as well as evidence of continuing specific conceptual impairment among schizophrenics.

scholarly journals The Association Between Depressive Symptoms and Cognitive Functioning in Older Hispanic/Latino Adults Enrolled in an Exercise Intervention: Results From the “¡Caminemos!” Study

2017 ◽  
Vol 30 (6) ◽  
pp. 843-862 ◽  
Author(s):  
Rosalba Hernandez ◽  
Elaine Cheung ◽  
Minli Liao ◽  
Seth W. Boughton ◽  
Lisett G. Tito ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Depressive Symptoms ◽  
Cognitive Function ◽  
Cognitive Functioning ◽  
Chronic Conditions ◽  
Exercise Intervention ◽  
Longitudinal Analyses ◽  
Symptoms Of Depression ◽  
Intervention Method

Objective: We examined the association between depressive symptoms and cognitive functioning in older Hispanics/Latinos enrolled in an exercise intervention. Method: We analyzed baseline, 1-year, and 2-year in-person interview data collected from Hispanics/Latinos aged ≥60 years participating in an exercise intervention across 27 senior centers ( N = 572). Results: Mean age was 73.13 years; 77% female. At baseline, older adults screening positive for depression were 1.58 times more likely to experience cognitive impairment ( p = .04); controlling for demographics and comorbid chronic conditions. Compared to peers with little to no depressive symptoms, lower cognitive functioning scores were evident at each follow-up assessment point where elevated depressive symptoms were present, but baseline depression was not associated with cognitive function in longitudinal analyses. Discussion: In older Hispanics/Latinos enrolled in an exercise intervention, though baseline depression did not predict cognitive function over time, elevated symptoms of depression were associated with greater cognitive impairment at every point in this study.

scholarly journals Predictors of subjective versus objective cognitive functioning in patients with stable grades II and III glioma

2015 ◽  
Vol 2 (1) ◽  
pp. 20-31 ◽  
Author(s):  
Karin Gehring ◽  
Martin J.B. Taphoorn ◽  
Margriet M. Sitskoorn ◽  
Neil K. Aaronson
Keyword(s):  
Mental Health ◽  
Cognitive Function ◽  
Cognitive Functioning ◽  
Well Being ◽  
Low Grade Glioma ◽  
Motor Dysfunction ◽  
Low Grade ◽  
Physical And Mental Health ◽  
Subjective Cognitive Function ◽  
Mental Health Factors

Abstract Background Studies in cancer and noncancer populations demonstrate lower than expected correlations between subjective cognitive symptoms and cognitive functioning as determined by standardized neuropsychological tests. This paper systematically examines the association between subjective and objective cognitive functioning in patients with low-grade glioma and the associations of these indicators of cognitive function with clusters of sociodemographic, clinical, and self-reported physical and mental health factors. Methods Multiple regression analyses with the subjective and 2 objective indicators of cognitive functioning as dependent variables and 4 clusters of predictor variables were conducted in 169 patients with predominantly low-grade glioma. Results Correlations between the subjective and the 2 objective cognitive indicators were negligible (0.04) to low (0.24). Objective cognitive deficits were predominantly associated with sociodemographic (older age, lower education, male sex) and clinical (left hemisphere tumor) variables, while lower ratings of subjective cognitive function were more closely related to self-reported mental health symptoms (fatigue, lower mental well-being), physical (motor) dysfunction and female sex. Self-reported communication deficits were associated significantly with both subjective and objective dysfunction. Conclusions We recommend that both subjective and objective measures of cognitive functioning, together with a measure of psychological distress, be used for comprehensive neuropsychological assessments of patients with glioma to determine which areas are most affected and which specific intervention strategies are most appropriate.

Assessment of cognitive impairment with the cognitive assessment interview (CAI) was useful for identifying poor psychosocial functioning outcome in patients with psychosis

2016 ◽  
Vol 33 (S1) ◽  
pp. S106-S107
Author(s):  
A. Sánchez-Torres ◽  
M.R. Elosúa ◽  
R. Lorente-Omeñaca ◽  
L. Moreno-Izco ◽  
V. Peralta ◽  
...  
Keyword(s):  
Social Cognition ◽  
Cognitive Functioning ◽  
Verbal Memory ◽  
Psychosocial Functioning ◽  
Cognitive Assessment ◽  
Daily Functioning ◽  
Everyday Functioning ◽  
Cognitive Domains ◽  
Assessment Interview ◽  
Competing Interest

IntroductionCognitive impairments clearly impact the daily functioning of patients with psychosis.ObjectivesTo assess cross-sectionally whether there are differences in the cognitive domains assessed with the CAI, for considering the real-world functioning of a sample of patients with psychosis.MethodsThe sample consisted of 76 patients with a DSM-IV psychotic disorder. Patients were assessed with the cognitive assessment interview (CAI), which is an interview-based measure of cognitive functioning that is intermediate between cognitive functioning and daily functioning, and three subscales of the specific levels of functioning (SLOF), an informant-rated measure of functioning. The CAI was used to assess the patient and an informant, and these scores were integrated into a rater composite score. We divided the sample by a median-split procedure for each of the three functional domains, and then applied ANOVAs to compare the two groups (impaired/not impaired) in the six cognitive domains of the CAI: working memory, attention, verbal memory, problem solving, processing speed, and social cognition.ResultsWe found significant differences between the impaired vs. non-impaired groups in most of the cognitive domains assessed with the CAI (Fig. 1).ConclusionsInterview-based assessment of cognition with the CAI allows for the prediction of everyday functioning in patients with psychosis. Impairment in almost all CAI cognitive domains, except for social cognition, was associated with poorer psychosocial functioning.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Abstract TP437: Asymptomatic Carotid Stenosis is Associated With Cognitive Impairment in a Chinese Community Population

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Jiaokun Jia ◽  
Xingquan Zhao
Keyword(s):  
Risk Factors ◽  
Cognitive Impairment ◽  
Alcohol Consumption ◽  
Confidence Interval ◽  
Cognitive Functioning ◽  
Color Doppler ◽  
Chinese Community ◽  
Current Smoking ◽  
Community Population ◽  
Language Abstraction

Purpose: This study aimed to investigate the effects of aCAS on cognitive functioning in a Chinese community population who were over 40 year-old and to find the domains of cognitive impairment which were mainly affected. Methods: Information was collected on the presence of CAS, which was assessed by Color Doppler Ultrasound, from the Asymptomatic Polyvascular Abnormalities Community study, and cognitive functioning was assessed by the score of the Montreal Cognitive Assessment (MoCA). Multivariate logistic regression was used to assess the relationships between aCAS and cognitive impairment. Results: A total of 812 (61.2% men, 55% of 40-60y) patients were included in this study. After adjusting for potential confounding factors, the associations between the aCAS and cognitive impairment remained significant [odds ratio(OR)(95% confidence interval, 95%CI)=1.812(1.049-3.127)]. Also, there were significant relationships between the aCAS and the domains of cognitive function like attention, language, abstraction and recall [OR(95%CI) 2.740(1.494-5.023), 2.005(1.184-3.396), 2.841(1.594-5.065), 4.674(2.478-8.818) respectively, P < 0.05). In addition, there were no interaction effects of age and other possible risk factors on the association. Conclusion: In the over 40 year-old Chinese community population, aCAS was an independent indicator of cognitive impairment, especially affecting the function of attention, language, abstraction and recall. Table 1 Baseline demographic characteristics and cardiovascular risk factors between groups with/without cognitive impairment Values are median (interquartile range) or number (percent). Table 2 Unadjusted and Multivariate-adjusted OR (95% CI) for total and subtests of MoCA, according to groups with vs. without CAS. *P<0.05;**P<0.001 OR: odd ratio; CI: confidence interval Model 1: adjusted for age and gender. Model 2: adjusted for age, gender, BMI, education, current smoking and alcohol consumption. Model 3:adjusted for age, gender, BMI, education, current smoking, alcohol consumption hypertension, diabetes, hyperlipidemia, hs-CRP and Hcy.

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