scholarly journals Cognitive Reserve and Brain Reserve in Prodromal Huntington's Disease

2013 ◽  
Vol 19 (7) ◽  
pp. 739-750 ◽  
Author(s):  
Aaron Bonner-Jackson ◽  
Jeffrey D. Long ◽  
Holly Westervelt ◽  
Geoffrey Tremont ◽  
Elizabeth Aylward ◽  
...  
Keyword(s):  
Cognitive Reserve ◽  
Morphological Changes ◽  
Occupational Status ◽  
Disease Onset ◽  
Brain Structures ◽  
Rate Of Change ◽  
Longitudinal Change ◽  
Brain Volumes ◽  
Cognitive Measure ◽  
Intellectual Level

AbstractHuntington disease (HD) is associated with decline in cognition and progressive morphological changes in brain structures. Cognitive reserve may represent a mechanism by which disease-related decline may be delayed or slowed. The current study examined the relationship between cognitive reserve and longitudinal change in cognitive functioning and brain volumes among prodromal (gene expansion-positive) HD individuals. Participants were genetically confirmed individuals with prodromal HD enrolled in the PREDICT-HD study. Cognitive reserve was computed as the composite of performance on a lexical task estimating premorbid intellectual level, occupational status, and years of education. Linear mixed effects regression (LMER) was used to examine longitudinal changes on four cognitive measures and three brain volumes over approximately 6 years. Higher cognitive reserve was significantly associated with a slower rate of change on one cognitive measure (Trail Making Test, Part B) and slower rate of volume loss in two brain structures (caudate, putamen) for those estimated to be closest to motor disease onset. This relationship was not observed among those estimated to be further from motor disease onset. Our findings demonstrate a relationship between cognitive reserve and both a measure of executive functioning and integrity of certain brain structures in prodromal HD individuals. (JINS, 2013, 19, 1–12).

Age of Onset as a Moderator of Cognitive Decline in Pediatric-Onset Multiple Sclerosis

2014 ◽  
Vol 20 (8) ◽  
pp. 796-804 ◽  
Author(s):  
Banafsheh Hosseini ◽  
David B. Flora ◽  
Brenda L. Banwell ◽  
Christine Till
Keyword(s):  
Multiple Sclerosis ◽  
Cognitive Decline ◽  
Social Status ◽  
Cognitive Reserve ◽  
Disease Onset ◽  
Rate Of Change ◽  
Childhood And Adolescence ◽  
Younger Age ◽  
Pediatric Onset ◽  
Cognitive Maturation

AbstractCognitive impairment is often reported in pediatric-onset multiple sclerosis (MS). Using serial cognitive data from 35 individuals with pediatric-onset MS, this study examined how age at disease-onset and proxies of cognitive reserve may impact cognitive maturation over the course of childhood and adolescence. Neuropsychological evaluations were conducted at baseline and up to four more assessments. Of the 35 participants, 7 completed only one assessment, 5 completed two assessments, 13 completed three assessments, 10 completed four or more assessments. Growth curve modeling was used to assess longitudinal trajectories on the Trail Making Test-Part B (TMT-B) and the Symbol Digit Modalities (SDMT; oral version) and to examine how age at disease onset, baseline Full Scale IQ, and social status may moderate rate of change on these measures. Mean number of evaluations completed per patient was 2.8. Younger age at disease onset was associated with a greater likelihood of cognitive decline on both the TMT-B (p=.001) and SDMT (p=.005). Baseline IQ and parental social status did not moderate any of the cognitive trajectories. Findings suggest that younger age at disease-onset increases the vulnerability for disrupted performance on measures of information processing, visual scanning, perceptual/motor speed, and working memory. Proxies of cognitive reserve did not protect against the progression of decline on these measures. Young patients with MS should be advised to seek follow-up cognitive evaluation to assess cognitive maturation and to screen for the potential late emergence of cognitive deficits. (JINS, 2014, 20, 1–9)

scholarly journals Plasma NfL levels and longitudinal change rates in C9orf72 and GRN-associated diseases: from tailored references to clinical applications

2021 ◽  
pp. jnnp-2021-326914
Author(s):  
Dario Saracino ◽  
Karim Dorgham ◽  
Agnès Camuzat ◽  
Daisy Rinaldi ◽  
Armelle Rametti-Lacroux ◽  
...  
Keyword(s):  
Single Molecule ◽  
Light Chain ◽  
Rate Of Change ◽  
Longitudinal Change ◽  
Youden Index ◽  
Clinical Genetic ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Link Type ◽  
Therapeutic Trials

ObjectiveNeurofilament light chain (NfL) is a promising biomarker in genetic frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). We evaluated plasma neurofilament light chain (pNfL) levels in controls, and their longitudinal trajectories in C9orf72 and GRN cohorts from presymptomatic to clinical stages.MethodsWe analysed pNfL using Single Molecule Array (SiMoA) in 668 samples (352 baseline and 316 follow-up) of C9orf72 and GRN patients, presymptomatic carriers (PS) and controls aged between 21 and 83. They were longitudinally evaluated over a period of >2 years, during which four PS became prodromal/symptomatic. Associations between pNfL and clinical–genetic variables, and longitudinal NfL changes, were investigated using generalised and linear mixed-effects models. Optimal cut-offs were determined using the Youden Index.ResultspNfL levels increased with age in controls, from ~5 to~18 pg/mL (p<0.0001), progressing over time (mean annualised rate of change (ARC): +3.9%/year, p<0.0001). Patients displayed higher levels and greater longitudinal progression (ARC: +26.7%, p<0.0001), with gene-specific trajectories. GRN patients had higher levels than C9orf72 (86.21 vs 39.49 pg/mL, p=0.014), and greater progression rates (ARC:+29.3% vs +24.7%; p=0.016). In C9orf72 patients, levels were associated with the phenotype (ALS: 71.76 pg/mL, FTD: 37.16, psychiatric: 15.3; p=0.003) and remarkably lower in slowly progressive patients (24.11, ARC: +2.5%; p=0.05). Mean ARC was +3.2% in PS and +7.3% in prodromal carriers. We proposed gene-specific cut-offs differentiating patients from controls by decades.ConclusionsThis study highlights the importance of gene-specific and age-specific references for clinical and therapeutic trials in genetic FTD/ALS. It supports the usefulness of repeating pNfL measurements and considering ARC as a prognostic marker of disease progression.Trial registration numbersNCT02590276 and NCT04014673.

scholarly journals Cognitive reserve in amyotrophic lateral sclerosis (ALS): a population-based longitudinal study

2021 ◽  
pp. jnnp-2020-324992
Author(s):  
Emmet Costello ◽  
James Rooney ◽  
Marta Pinto-Grau ◽  
Tom Burke ◽  
Marwa Elamin ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Impairment ◽  
Cognitive Decline ◽  
Cognitive Reserve ◽  
Population Based ◽  
Protective Role ◽  
Drop Out ◽  
Longitudinal Change ◽  
Activity Data ◽  
Lateral Sclerosis

BackgroundAmyotrophic lateral sclerosis (ALS) is often associated with cognitive and/or behavioural impairment. Cognitive reserve (CR) may play a protective role in offsetting cognitive impairment. This study examined the relationship between CR and longitudinal change in cognition in an Irish ALS cohort.MethodsLongitudinal neuropsychological assessment was carried out on 189 patients over 16 months using the Edinburgh cognitive and behavioural ALS screen (ECAS) and an additional battery of neuropsychological tests. CR was measured by combining education, occupation and physical activity data. Joint longitudinal and time-to-event models were fitted to investigate the associations between CR, performance at baseline and decline over time while controlling for non-random drop-out.ResultsCR was a significant predictor of baseline neuropsychological performance, with high CR patients performing better than those with medium or low CR. Better cognitive performance in high CR individuals was maintained longitudinally for ECAS, social cognition, executive functioning and confrontational naming. Patients displayed little cognitive decline over the course of the study, despite controlling for non-random drop-out.ConclusionsThese findings suggest that CR plays a role in the presentation of cognitive impairment at diagnosis but is not protective against cognitive decline. However, further research is needed to examine the interaction between CR and other objective correlates of cognitive impairment in ALS.

scholarly journals Short-Term Memory Deficits in the SLEEP Inbred Panel

2019 ◽  
Vol 1 (4) ◽  
pp. 471-488 ◽  
Author(s):  
Shailesh Kumar ◽  
Kirklin R. Smith ◽  
Yazmin L. Serrano Negron ◽  
Susan T. Harbison
Keyword(s):  
Learning And Memory ◽  
Sleep Duration ◽  
Genetic Architecture ◽  
Short Term Memory ◽  
Morphological Changes ◽  
Brain Structures ◽  
Social Conditions ◽  
Short Term ◽  
The Relationship ◽  
The Brain

Although sleep is heritable and conserved across species, sleep duration varies from individual to individual. A shared genetic architecture between sleep duration and other evolutionarily important traits could explain this variability. Learning and memory are critical traits sharing a genetic architecture with sleep. We wanted to know whether learning and memory would be altered in extreme long or short sleepers. We therefore assessed the short-term learning and memory ability of flies from the Sleep Inbred Panel (SIP), a collection of 39 extreme long- and short-sleeping inbred lines of Drosophila. Neither long nor short sleepers had appreciable learning, in contrast to a moderate-sleeping control. We also examined the response of long and short sleepers to enriched social conditions, a paradigm previously shown to induce morphological changes in the brain. While moderate-sleeping control flies had increased daytime sleep and quantifiable increases in brain structures under enriched social conditions, flies of the Sleep Inbred Panel did not display these changes. The SIP thus emerges as an important model for the relationship between sleep and learning and memory.

scholarly journals Relationship Between Verbal Fluency Performance, Fight Exposure, and Subcortical Region Brain Volumes in Professional Fighters

2019 ◽  
Vol 34 (5) ◽  
pp. 735-735
Author(s):  
L Bennett ◽  
C Bernick ◽  
S Banks
Keyword(s):  
Verbal Fluency ◽  
Brain Structures ◽  
Brain Regions ◽  
Health Study ◽  
Semantic Fluency ◽  
Cognitive Changes ◽  
Brain Volumes ◽  
Subcortical Brain ◽  
Subcortical Region ◽  
Subcortical Brain Structures

Abstract Purpose Verbal fluency performance has been shown to be sensitive to preclinical cognitive changes in neurodegenerative diseases and may detect early, trauma-related cognitive and volumetric changes amongst professional fighters. Baseline verbal fluency performance and volumes of relevant subcortical brain structures were expected to decline as number of professional fights (NoPF) increased, while controlling for education. Methods Baseline letter and semantic fluency performance, NoPF, and structural brain imaging from 548 active and retired fighters who participated in the Professional Fighters Brain Health Study were considered. ANCOVAs were conducted to assess differences in verbal fluency performance by NOPF, while controlling for years of education. Number of professional fights were stratified into low (0-20 fights), medium (21-40 fights), and high (41 or more fights). Results Semantic fluency performance differed across the three levels of NoPF (F(2, 542)=4.56; p<.02). In addition, significant positive correlations between semantic fluency performance and volumes in the following regions were observed: left thalamus, left putamen, left pallidum, bilateral caudates, bilateral amygdalae, bilateral hippocampi, and bilateral accumbens (all p’s<.05). In contrast, letter fluency performance was not significantly associated with NoPF or volumes of relevant subcortical brain structures (all p’s>.05). Conclusion Semantic fluency may be low-cost, easy-to-administer harbinger of emerging cognitive dysfunction and lower volumes in related subcortical brain regions. Additional assessment of clinical utility is necessary.

scholarly journals Telemedicine assessment of long-term cognitive and functional status in anti-leucine-rich, glioma-inactivated 1 encephalitis

2019 ◽  
Vol 7 (2) ◽  
pp. e652 ◽  
Author(s):  
Nuria Sola-Valls ◽  
Helena Ariño ◽  
Domingo Escudero ◽  
Elisabeth Solana ◽  
Albert Lladó ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Functional Status ◽  
Verbal Memory ◽  
Cognitive Reserve ◽  
Disease Onset ◽  
Cognitive Status ◽  
Functional Activities ◽  
Telephone Interviews ◽  
Status Assessment

ObjectiveTo assess the feasibility of a structured telephone interview examining the long-term cognitive and functional status in anti–leucine-rich, glioma-inactivated 1 (LGI1) encephalitis.MethodsTelephone interviews were conducted with 37 patients after a median follow-up of 87 months from disease onset and 23 healthy controls matched for age and sex. Cognitive status was assessed with the telephone Mini-Mental State Examination (t-MMSE) and 3 tests exploring verbal memory, fluency, and executive function. Functional status was evaluated with the Functional Activities Questionnaire and the modified Rankin Scale (mRS). Patients were classified as normal, with mild cognitive impairment (MCI), or with dementia based on cognitive and functional status. Assessment of the cognitive reserve was performed with a structured questionnaire. Logistic regression analysis was applied to identify predictors of cognitive impairment.ResultsTelephone interviews were successful in 36/37 (97%) patients. Cognitive impairment was detected in 27 (75%) including 17 with MCI and 10 with dementia. Eight (29%) patients would have been misclassified using only the t-MMSE. Twenty-six (72%) patients were functionally independent according to the mRS, but only 9 (35%) were cognitively normal. Independent predictors for long-term cognitive impairment were a low cognitive reserve (OR = 1.36, 95% CI: 1.05–1.76; p = 0.02) and bilateral hippocampal hyperintensity at initial MRI (OR = 27.03, 95% CI: 1.87–390; p = 0.02).ConclusionsTelemedicine is a feasible tool to assess the cognitive and functional outcome in patients with anti-LGI1 encephalitis. Cognitive impairment is often missed if only functional scales are used. Premorbid cognitive reserve and MRI with bilateral hippocampal hyperintensity were predictors for long-term cognitive impairment.

scholarly journals Delaying Onset of Dementia: Are Two Languages Enough?

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Morris Freedman ◽  
Suvarna Alladi ◽  
Howard Chertkow ◽  
Ellen Bialystok ◽  
Fergus I. M. Craik ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Protective Effect ◽  
Executive Control ◽  
Cognitive Reserve ◽  
Language Use ◽  
Occupational Status ◽  
Protective Effects ◽  
Significant Protective Effect ◽  
Multicultural Perspective

There is an emerging literature suggesting that speaking two or more languages may significantly delay the onset of dementia. Although the mechanisms are unknown, it has been suggested that these may involve cognitive reserve, a concept that has been associated with factors such as higher levels of education, occupational status, social networks, and physical exercise. In the case of bilingualism, cognitive reserve may involve reorganization and strengthening of neural networks that enhance executive control. We review evidence for protective effects of bilingualism from a multicultural perspective involving studies in Toronto and Montreal, Canada, and Hyderabad, India. Reports from Toronto and Hyderabad showed a significant effect of speaking two or more languages in delaying onset of Alzheimer’s disease by up to 5 years, whereas the Montreal study showed a significant protective effect of speaking at least four languages and a protective effect of speaking at least two languages in immigrants. Although there were differences in results across studies, a common theme was the significant effect of language use history as one of the factors in determining the onset of Alzheimer’s disease. Moreover, the Hyderabad study extended the findings to frontotemporal dementia and vascular dementia.

Longitudinal change in brain volumes and cognitive function in MPS IIIA

2013 ◽  
Vol 108 (2) ◽  
pp. S69-S70
Author(s):  
Igor Nestrasil ◽  
Kathleen Delaney ◽  
Brianna Yund ◽  
Alia Ahmed ◽  
Kyle Rudser ◽  
...  
Keyword(s):  
Cognitive Function ◽  
Longitudinal Change ◽  
Brain Volumes ◽  
Mps Iiia
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