Comparison of cognitive function in siblings with neuronopathic mucopolysaccharidosis II: evaluation of early treatment with intravenous idursulfase and intrathecal idursulfase-IT

2021 ◽  
Vol 132 ◽  
pp. S142-S143
Author(s):  
Joseph Muenzer ◽  
Barbara Burton ◽  
Paul Harmatz ◽  
Luis González Gutiérrez-Solana ◽  
Matilde Ruiz-Garcia ◽  
...  
1995 ◽  
Vol 7 (4) ◽  
pp. 547-560 ◽  
Author(s):  
José L. González-Torrecillas ◽  
Julien Mendlewicz ◽  
Antonio Lobo

This study was made in an attempt to document the effects of early treatment of poststroke depression (including fluoxetine treatment) on neuropsychological rehabilitation (including cognitive function). Assessment measures used included the Schedule for Affective Disorders and Schizophrenia (SADS) and Research Diagnostic Criteria (RDC), as well as standard measures of severity of depression, functional ability, cognitive function, and neurological function. Thirty-seven patients with poststroke depression, treated with fluoxetine (n = 26) or nortriptyline (n = 11), were compared with 11 poststroke depressed patients who received no depression treatment and 82 poststroke nondepressed patients who received no depression treatment. Our findings about the prevalence of depression (37%), more frequent with anterior lesion (p = .009) and left hemisphere lesion (not statistically significant), tend to confirm previous reports. Early treatment (4th week poststroke) with either fluoxetine or nortriptyline significantly improved the depressed patients' mood, neurological function, functional ability, and cognitive ability. A close relationship between appropriate early treatment (including fluoxetine treatment) of poststroke depression and improved neuropsychological rehabilitation (including cognitive improvement) is suggested by our findings. This is the first report, to our knowledge, of the beneficial effects of early antidepressant treatment on the cognitive function of poststroke depressed patients.


2020 ◽  
Author(s):  
Mallory Owen ◽  
Rafael Romero-Garcia ◽  
Alexa McDonald ◽  
Emma Woodberry ◽  
Moataz Assem ◽  
...  

BackgroundCognitive function in patients with diffuse glioma is frequently impaired and can have a profound impact on quality of life. Accurate, reproducible and accessible tools to assess cognition are mandatory to understand the effects of the tumour and treatment. Our hypothesis was that an app-based assessment would be complementary to traditional neuropsychological testing, thereby aiding in defining cognitive profiles and trajectories during early treatment of diffuse glioma.MethodsSeventeen subjects with diffuse low-grade gliomas completed a traditional neuropsychological assessment battery before and after surgery. In addition an app-based tablet assessment (OCS-Bridge) was administered pre- and post-operatively as well as longitudinally at 3- and 12-month follow-up. Deficit rates, mean performance, and changes over time were compared using standardized z-scores between the two testing methods. Unsupervised k-means clustering was performed on individual cognitive tests in each battery.ResultsPreoperative testing showed an average of 2.88 deficits and 1.18 deficits per patient on neuropsychological testing and the tablet-based OCS-Bridge assessment, respectively. Digit span testing demonstrated agreement between testing modalities, but otherwise there was no significant correlation (Pearson’s correlation: p=0.7723, r-value = 0.0758, df = 16). Longitudinal assessment revealed dynamic changes in attention and nonverbal skills. Traditional assessment was more sensitive to memory deficits, showing 22 preoperative deficits within the cohort vs. 1 for the app-based assessment, while app-based assessment was more sensitive to nonverbal skills, showing 8 deficits preoperatively vs. none in the traditional assessment. Clustering analysis did not create clusters along the predetermined domains, indicating that certain individual tests may test more than one cognitive function.ConclusionsThese data suggest app-based assessment is reliable and complementary to data obtained from traditional neuropsychological testing. Advantages include efficiency, facilitation of longitudinal testing, and increased sensitivity in domains of non-verbal skills and attention. Patients with diffuse glioma show subtle neuropsychological impairments, unique cognitive profiles, and discrete trajectories during early treatment, therefore judicious assessment is imperative.Key PointsPatients with glioma have subtle cognitive changes requiring judicious testingApp-based assessment is effective in understanding neuropsychological functionDistinct cognitive profiles and trajectories are demonstrated during treatmentImportance of the StudyUnderstanding cognitive function over time is an essential marker of quality of life and treatment outcomes in patients with diffuse glioma. Because diffuse gliomas are associated with few impairments, careful choice of individual neurocognitive testing is paramount to ensure sufficient sensitivity. Additionally, individuals with similar presentations or seemingly innocuous tumour locations can have profound differences in cognitive outcomes (Romero-Garcia et al., 2020).Studies have demonstrated that traditional neuropsychological testing can be resource-intensive and unsuitable for administration at multiple time points. New app-based testing modalities aim to address these issues, and theoretically offer advantages in terms of novel interactive paradigms, ease of administration, and efficiency of repeated testing in longitudinal studies. However, they have not been extensively validated for use in this population, nor compared with traditional neuropsychology testing.This study compares two neurocognitive testing modalities in a single population with diffuse glioma. Our study demonstrated that patients with diffuse glioma had subtle deficits, and the two modalities were complementary in their sensitivity to deficits in different domains of cognitive function. Traditional neuropsychology testing was more sensitive to memory deficits, while app-based assessment was more sensitive nonverbal skills and attention deficits. In addition, the app-based assessment allowed identification of longitudinal cognitive trajectories, while combined analysis identified discrete cognitive profiles specific to patients with diffuse glioma. Overall these data demonstrate the importance of cognitive testing in this population and highlight the complementary role of combined testing.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Karen S. Yee ◽  
Yanyu Wu ◽  
Magdalena Harrington ◽  
Susan E. Waisbren

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.


2009 ◽  
Vol 42 (20) ◽  
pp. 1-2
Author(s):  
MITCHEL L. ZOLER
Keyword(s):  

GeroPsych ◽  
2011 ◽  
Vol 24 (2) ◽  
pp. 83-92 ◽  
Author(s):  
Valentina A. Tesky ◽  
Christian Thiel ◽  
Winfried Banzer ◽  
Johannes Pantel

To investigate the effects of leisure activities on cognitive performance of healthy older subjects, an innovative intervention program was developed. Frequent participation in cognitively stimulating activities (i.e., reading, playing chess, or playing music) is associated with reduced risk of dementia. AKTIVA (active cognitive stimulation – prevention in the elderly) is an intervention program designed to enhance cognitive stimulation in everyday life by increasing cognitive stimulating leisure activities. The present study determines the effects of AKTIVA on cognitive function, mood and attitude toward aging in a sample of older participants from the general population. Several measurement instruments were used including the Alzheimer’s Disease Assessment Scale (ADAS-Cog), the Trail-Making Test (TMT), and the Memory Complaint Questionnaire (MAC-Q). Initially, the sample consisted of 307 older persons (170 female, 72 ± 7 years). The intervention was evaluated with a randomized, controlled pre-post follow-up design. Participants were randomly assigned to one of three conditions: AKTIVA intervention (n = 126), AKTIVA intervention plus nutrition and exercise counseling (n = 84), no-intervention control group (n = 97). The AKTIVA intervention consisted of 8 weekly sessions and two booster sessions after a break of 4 months. Participation in the group program resulted in positive effects on cognitive function and attitude toward aging for subassembly groups. Older persons (≥ 75 years) showed enhanced speed of information processing (by TMT Version A) (F = 4.17*, p < .05); younger participants (< 75 years) showed an improvement in subjective memory decline (by MAC-Q) (F = 2.55*, p < .05). Additionally, AKTIVA enhanced the frequency of activities for leisure activities for subassembly groups. The results of this study suggest that the AKTIVA program can be used to increase cognitively stimulating leisure activities in the elderly. Further research is necessary to identify the long-term effects of this intervention particularly with respect to the prevention of dementia.


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