scholarly journals Analysis of cognitive ability and adaptive behavior assessment tools used in an observational study of patients with mucopolysaccharidosis II

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Karen S. Yee ◽  
Yanyu Wu ◽  
Magdalena Harrington ◽  
Susan E. Waisbren
Keyword(s):  
Cognitive Function ◽  
Observational Study ◽  
Adaptive Behavior ◽  
Functional Outcomes ◽  
Early Years ◽  
Assessment Tools ◽  
School Age ◽  
Mucopolysaccharidosis Ii ◽  
Functional Changes ◽  
Mps Ii

Abstract Background Mucopolysaccharidosis II (MPS II) is a rare lysosomal storage disease characterized by cognitive impairment in most patients. This post hoc analysis evaluated changes in cognitive function, adaptive behavior and functional outcomes in patients with neuronopathic MPS II over time. Fifty-five children with MPS II were enrolled in a 24-month observational study (NCT01822184). The Differential Ability Scales, second edition (DAS-II; early years battery for ages 2 years 6 months to 6 years 11 months, school age battery for ages 7 years to 17 years 11 months), Vineland Adaptive Behavior Scales, second edition (VABS-II) and the Hunter Syndrome-Functional Outcomes for Clinical Understanding Scale (HS-FOCUS) were performed at baseline and 3-month intervals over 2 years. A subgroup of 38 children with a DAS-II General Conceptual Ability (GCA) score of 55–85 (below average–very low abilities) at any time during the study were included in this analysis. Results Mean (standard deviation [SD]) early years DAS-II GCA score decreased from 73.4 (15.7, n = 22) at baseline to 62.7 (34.9, n = 6) at month 24. For the six patients with early years GCA assessments at baseline and month 24, mean (SD) GCA scores decreased from 72.3 (21.3) at baseline to 62.7 (34.9) at month 24. School age GCA scores were stable over 2 years: mean (SD) 72.4 (11.8, n = 10) at baseline; 74.3 (12.3, n = 8) at month 24. Mean (SD) VABS-II Adaptive Behavior Composite (ABC) scores were stable throughout the study (baseline, 81.8 [11.8, n = 36]; month 24, 81.0 [10.2, n = 13]). Some associations between items and domains of HS-FOCUS (p < 0.05) and DAS-II GCA and VABS-II ABC scores were shown, but there was no clear pattern of changes in HS-FOCUS over 2 years. Conclusions The DAS-II measured changes in cognitive function over 2 years in younger patients with MPS II, whereas cognitive function in older patients remained stable. Further research is required to confirm the content validity of the DAS-II in different patient populations with MPS II. The VABS-II and HS-FOCUS were not sensitive tools for measuring behavioral and functional changes over 2 years. These findings may inform selection of appropriate cognitive and behavioral assessment tools for future studies.

scholarly journals More Than IQ: Executive Function Explains Adaptive Behavior Above and Beyond Nonverbal IQ in Youth With Autism and Lower IQ

2019 ◽  
Vol 124 (3) ◽  
pp. 191-205 ◽  
Author(s):  
Jennifer R. Bertollo ◽  
Benjamin E. Yerys
Keyword(s):  
Autism Spectrum Disorder ◽  
Executive Function ◽  
Significant Role ◽  
Adaptive Behavior ◽  
Functional Outcomes ◽  
Autism Spectrum ◽  
School Age ◽  
Measuring Outcomes ◽  
Explained Variance ◽  
Critical Metric

Abstract Adaptive behavior is a critical metric for measuring outcomes in those with autism spectrum disorder (ASD). Executive function skills predict adaptive behavior in youth with ASD with average or higher IQ; however, no study has examined this relationship in ASD with lower IQ (IQ ≤ 75). The current study evaluated whether executive function predicted adaptive behavior in school-age youth with ASD with lower IQ, above and beyond nonverbal IQ. We examined adaptive behavior and executive function through informant report on 100 youth with ASD with lower IQ. Executive function skills explained variance in adaptive social and communication domains, beyond nonverbal IQ; monitoring skills played a significant role. This research suggests that malleable skills like executive function may contribute to functional outcomes in this population.

Functional outcomes following inpatient rehabilitation of guillain-barré syndrome patients: Intravenous immunoglobulins versus plasma exchange

2021 ◽  
pp. 1-9
Author(s):  
Moshe Bondi ◽  
Einat Engel-Haber ◽  
Julie Wolff ◽  
Liza Grosman-Rimon ◽  
Ayala Bloch ◽  
...  
Keyword(s):  
Activities Of Daily Living ◽  
Plasma Exchange ◽  
Functional Outcomes ◽  
Daily Living ◽  
Functional Independence ◽  
Assessment Tools ◽  
Guillain Barre Syndrome ◽  
Disability Scale ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

BACKGROUND: Treatment with either Intravenous immunoglobulin (IVIg) or plasma exchange (PE) in patients with Guillain-Barré Syndrome (GBS) showed equivalent efficacy as attested by a commonly used disability scale. However, it has been suggested that this scale may not be sensitive enough to detect subtle functional changes between the two treatments since it mainly focuses on walking capability and respiratory function. OBJECTIVE: To evaluate functional outcomes following treatment with IVIg or PE using comprehensive scales that incorporate parameters of basic activities of daily living. METHODS: A retrospective cohort study was conducted between 2007 and 2013 in an inpatient neurologic rehabilitation department. The study group included 70 individuals with GBS: 39 were treated with PE and 31 with IVIg. A comparison of functional outcomes was performed using Functional Independence Measure (FIM), rehabilitation efficiency (REy), rehabilitation effectiveness (REs), and the GBS disability scale (GDS). RESULTS: Both treatments had a comparable effect on the various functional outcomes. Patients showed a significant increase in total FIM scores (30 points on average) during rehabilitation mainly as a result of an increase in motor sub-scores. A mean improvement of 1.23 (SD 0.9) in GDS was also observed. On average, individuals with GBS spent 20 days combined in the acute departments and 61 days in the rehabilitation department, with length of stay being similar for both treatments. CONCLUSIONS: IVIg and PE treatments have similar basic activities of daily living (ADL) functional outcomes. Nevertheless, due to the different mechanism of actions of these treatments and the multitude of GBS variants, it is possible that further comprehensive assessment tools may demonstrate differences in activity and participation of individuals with GBS.

scholarly journals The Rationale for Monitoring Cognitive Function in Multiple Sclerosis: Practical Issues for Clinicians

2018 ◽  
Vol 12 (1) ◽  
pp. 31-40 ◽  
Author(s):  
Christos Bakirtzis ◽  
Panagiotis Ioannidis ◽  
Lambros Messinis ◽  
Grigorios Nasios ◽  
Elina Konstantinopoulou ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Clinical Practice ◽  
Cognitive Function ◽  
Cognitive Rehabilitation ◽  
Assessment Tools ◽  
Systematic Evaluation ◽  
Presenting Symptoms ◽  
Increased Risk ◽  
Medication Side ◽  
Multiple Sclerosis Patients

About half of patients with multiple sclerosis exhibit cognitive impairment which negatively affects their quality of life. The assessment of cognitive function in routine clinical practice is still undervalued, although various tools have been proposed for this reason. In this article, we describe the potential benefits of implementing cognitive assessment tools in routine follow -ups of MS patients. Early detection of changes in cognitive performance may provide evidence of disease activity, could unmask depression or medication side-effects and provide suitable candidates for cognitive rehabilitation. Since apathy and cognitive deficiencies are common presenting symptoms in Progressive Multifocal Leukoencephalopathy, we discuss the utility of frequent monitoring of mental status in multiple sclerosis patients at increased risk. In addition, we propose a relevant algorithm aiming to incorporate a systematic evaluation of cognitive function in every day clinical practice in multiple sclerosis.

scholarly journals The Effects of Intermittent Fasting on Brain and Cognitive Function

2021 ◽  
Author(s):  
Jip Gudden ◽  
Alejandro Arias Vasquez ◽  
Mirjam Bloemendaal
Keyword(s):  
Cognitive Function ◽  
Animal Studies ◽  
Randomized Clinical Trials ◽  
Caloric Intake ◽  
Autism Spectrum ◽  
Future Research ◽  
Intermittent Fasting ◽  
Long Term Effects ◽  
Functional Changes ◽  
Positive Effects

The importance of diet and the gut-brain axis for brain health and cognitive function is increasingly acknowledged. Dietary interventions are tested for their potential to prevent and/or treat brain disorders. Intermittent fasting (IF), the abstinence or strong limitation of calories for 12 to 48 hours, alternated with periods of regular food intake, has shown promising results on neurobiological health in animal models. In this review article, we discuss the potential benefits of IF on cognitive function and the possible effects on the prevention and progress of brain-related disorders in animals and humans. We do so by summarizing the effects of IF which - through metabolic, cellular and circadian mechanisms - lead to anatomical and functional changes in the brain. Our review shows that there is no clear evidence of a positive short-term effect of IF on cognition in healthy subjects. Clinical studies show benefits of IF for epilepsy, Alzheimer&rsquo;s disease and multiple sclerosis on disease symptoms and progress. Findings from animal studies show mechanisms by which Parkinson&rsquo;s disease, ischaemic stroke, autism spectrum disorder and mood- and anxiety disorders could benefit from IF. Future research should disentangle whether positive effects of IF hold true regardless of age or the presence of obesity. Also, variations in fasting patterns, total caloric intake and intake of specific nutrients may be relevant components of IF success. Longitudinal studies and Randomized Clinical Trials (RCTs) will provide a window into the long-term effects of IF on the development and progress of brain-related diseases.

Recovery from stunting and cognitive outcomes in young children: evidence from the South African Birth to Twenty Cohort Study

2015 ◽  
Vol 7 (2) ◽  
pp. 163-171 ◽  
Author(s):  
D. Casale ◽  
C. Desmond
Keyword(s):  
Cohort Study ◽  
Cognitive Function ◽  
Young Children ◽  
School Readiness ◽  
Economic Status ◽  
Cognitive Outcomes ◽  
Early Years ◽  
Cognitive Tests ◽  
Data Set ◽  
Urban South

In this study we analyse the implications for cognitive function of recovery from stunting in early childhood. More specifically, we test whether children who met the definition for stunted at age 2, but not at age 5, perform better in cognitive tests than children who remain stunted over this period. The sample is drawn from the Birth to Twenty Cohort Study, a prospective data set of children born in 1990 in urban South Africa. The measure of cognitive function that we use is based on the Revised Denver Prescreening Developmental Questionnaire implemented when the children were age 5. We employ multivariate regression in the analysis to control for child-specific characteristics, socio-economic status, the home environment and caregiver inputs. We find that recovery from stunting is not uncommon among young children in our sample. However, children who recover from stunting by age 5 still perform significantly worse on cognitive tests than children who do not experience early malnutrition, and almost as poorly as children who remain stunted. These findings suggest that the timing of nutritional inputs in the early years is key in a child’s cognitive development, with implications for school readiness and achievement.

Sign in / Sign up

Export Citation Format

Share Document