bluuebunny/crossref_metadata_2025_split huggingface dataset:
A - 100 Who Is at the Door? Clinical Presentations and Predictors of Dementia Diagnosis in a Specialist Dementia Service
Authors: Stewart Martha , Pillinger Robin , Coldham Rae , Wood Lesley , Mason Damon 
Published: September 2024

Abstract

Objective
This service audit aimed to identify predictors associated with a dementia diagnosis and the diagnostic base rate in a UK-based, specialist Younger People with Dementia (YPWD) Service.

Method
All patients referred between July 2022 and 2023 to the YPWD service were included in the study. All 191 patients were under 65 years at the time of referral. 69 patients were excluded from analysis (e.g., if the patient withdrew from the service prior to assessment or if the referral was deemed inappropriate based on service guidelines). A quasi-experimental design was used, investigating the relative predictive value of factors such as demographics, presenting cognitive symptoms, comorbidities, or mental health to receiving a dementia diagnosis. Following definition, raters reviewed client case notes to establish the presence or absence of each criterion.

Results
Significant predictors of a dementia diagnosis in YPWD were functional change (p &lt; 0.001), behavior change (p &lt; 0.001), being accompanied to assessment (p &lt; 0.001), diminished insight (p &lt; 0.001), executive functioning difficulties (p &lt; 0.05), cognitive screening test score below cut-off (p &lt; 0.05) and previous radiological investigation prior to referral (p &lt; 0.001). The diagnostic rate for YPWD was 15%.

Conclusions
Memory problems alone are an insufficient predictor for a dementia diagnosis in YPWD, unless accompanied by additional factors. Overreliance on the presence of subjective memory complaints in primary care as a percipient to referral for specialist assessment contributes to a low diagnostic rate for the service The presence of insight and attending appointments alone contraindicate the presence of an organic disorder. Results indicate that factors beyond diagnostic criteria may be clinically significant.
A - 102 Neuropsychological Insights into Kearns-Sayre Syndrome: a Case Study
Authors: Jackson Daija A , Brooks Keiona L , Willis Pamelia F 
Published: September 2024

Abstract

Objective
Kearns-Sayre Syndrome (KSS) is a genetic mitochondrial and neuromuscular condition with multisystemic effects, including the eyes, heart, muscles, and cognition. Very little is known regarding the cognitive profile of patients with KSS, and existing studies have presented mixed findings. Results in one study revealed impairments in visuospatial abilities and attention, while another study found impairments in global cognition and word-finding. Interestingly, both studies revealed impairments in executive functions.

Method
This abstract presents the case of a 46-year-old right-handed, African American female diagnosed with KSS in 2021. Symptoms of KSS first appeared in 2010 with progressive vision loss and bilateral ptosis. Genetic testing revealed MT-T1 mutation consistent with KSS. Electroretinography findings revealed mild cone atrophy and mild-to-moderate concentric visual field defects. Attentional difficulties were noted in 2016 and resulted in a diagnosis of ADHD. Memory problems were first noticed in 2016 and have persisted since then. Magnetic resonance imaging results from May 2023 revealed no structural abnormalities in the brain.

Results
A comprehensive neuropsychological evaluation completed in March 2024 revealed mostly intact neurocognitive functioning. Notably, performance on memory and visuospatial measures were within normal limits. However, poor performance was revealed on one measure of executive functioning.

Conclusions
It remains unclear whether the poor performance seen on a measure of executive functioning reflects a true cognitive deficit or just chance variation. Even so, with little information regarding the cognitive profile of KSS, baseline neuropsychological testing should be considered to monitor related cognitive changes over time.
A - 104 Cardiometabolic Multimorbidity Is Associated with Greater Amyloid Burden in Cognitively Stable Adults with Down Syndrome
Authors: Bisgaard Stephanie Tuxen , Bruno Jennifer 
Published: September 2024

Abstract

Objective
Nearly everyone (&gt;90%) with Down Syndrome (DS) develops Alzheimer’s Disease (ad). Cardiometabolic conditions (CC) are known dementia risk-factors in the general population, and cardiometabolic multimorbidity (CM) increases MCI, dementia, and ad. CC are highly prevalent in DS and amyloid burden (AB) is associated with ad and elevated across lifespan in DS. However, there are no studies on the association between CM and AB in DS.

Method
We studied baseline data from 240 participants, 40–53 years (198 cognitively stable; 42 with MCI or dementia), from Alzheimer’s Biomarker Consortium-Down Syndrome (ABC-DS). Participants were grouped 0–1 or &gt; 1 CC (hyperlipidemia, hypothyroidism, hypertension, cardiovascular conditions, stroke). Participants completed amyloid PET scan with [11C]-Pittsburgh compound B ([11C]-PiB) or [18F]-AV45 (florbetapir). Regional standard uptake value-ratios (SUVRs) were calculated relative to cerebellar cortex and transformed to Centiloid global AB scores for analysis. We tested association between CM and AB scores using hierarchical linear regression, including age and apolipoprotein E (ApoE) as covariates.

Results
The regression model revealed that CM was significantly associated with greater AB after accounting for age and ApoE [F (3,194) = 57.752, p &lt; 0.001, ß = 0.228, 95% CI [0.007–0.449], p = 0.043]. Centiloid values were higher for two or more CC (Fig. 1).

Conclusion
Our results suggest that two or more CC is associated with greater AB in cognitively stable adults with DS. These findings help us understand the CM prodromal role in accelerating ad in DS and provide insights to preventative strategies. Future studies should explore how CM and related biomarkers, eg., chronic peripheral inflammation, influence ad in DS.
A - 107 The Role of Cognitive Functions in the Activities of Daily Living and Compensatory Aid Use of Older Adults
Authors: Kozuki Jordan , Baez Abril , Reyes Fabiola A , Wilkin Lily , Miller Chris H , Schmitter-Edgecombe Maureen , Woo Ellen 
Published: September 2024

Abstract

Objective
Memory and executive functions contribute to the completion of instrumental activities of daily living (IADLs). Compensatory aids may be used to support age-related changes in cognition. The current study examines the roles of memory and executive functions in IADLs and the use compensatory strategies across the cognitive spectrum.

Methods
Participants included 132 older adults (58 healthy older controls, 65 individuals with MCI, and 9 individuals with ad). Composite scores were calculated for Memory (CVLT-II Long Delay Free Recall and BVMT-R Delayed Recall) and Executive Functioning (Stroop Interference and Trails B) tests completed. The Instrumental Activities of Daily Living – Compensation (IADL-C) scale was used to assess the IADLs of money/self-management skills, home/daily living skills, travel/event memory, social skills, and total IADL skills, in addition to the use of compensatory aids.

Results
Regression analyses were conducted to examine the roles of memory and executive functions in IADLs and the use of compensatory aids, separately, across the cognitive spectrum. Results revealed that memory and executive functions predicted the ability to complete all IADLs. In contrast, cognitive skills did not predict the use of compensatory aids.

Conclusion
Overall, we found that memory and executive functions are important for completing a wide range of IADLs. Interestingly, these cognitive abilities did not play a role in the use of compensatory aids, even though these aids can support changes in cognition. This research suggests that it may be beneficial to recommend specific compensatory strategies to older adults that support memory and executive skills when performing everyday tasks.
A - 109 Neuropsychiatric Inventory Questionnaire Subfactors and Cognition in Parkinson’s Disease
Authors: Hochuli Maria , Almasri Mike , Moncrief Grant G , Roth Robert M 
Published: September 2024

Abstract

Objective
Patients with Parkinson’s disease (PD) commonly experience neuropsychiatric symptoms, often garnered through informant interview, that may be associated with poorer cognitive functioning. Recently, the Neuropsychiatric Inventory Questionnaire (NPI-Q) was found to have three factors with differential association to cognition in patients with PD. We sought to further examine this relationship in those with the condition.

Method
A sample of 42 patients with PD were seen for neuropsychological evaluation (85.7% male, 97.6% White, mean age 70.88 [SD = 7.16], mean years education 15.74 [SD = 2.99]). A knowledgeable informant completed the NPI-Q. Spearman correlations examined relationships between cognition (Digit Span, BVMT-R, CVLT-3, BNT-2, D-KEFS Color-Word Interference, COWAT, Animal Fluency, Trail Making Test), NPI-Q Total Severity, and severity for three factors (Mood [depression, anxiety, apathy, motor, nighttime behaviors, eating]; Hyperactivity [mania, aggression/agitation, disinhibition, irritability]; Psychotic Symptoms [delusions, hallucinations]).

Results
NPI-Q total severity was generally low (mean = 3.90, SD = 5.12). Total severity and severity of Mood and Hyperactivity were unrelated to cognition. More severe Psychotic Symptoms were related to worse Digit Span (r = −0.41, p = 0.008), BVMT-R Learning (r = −0.33, p = 0.03) and Delayed Recall (r = −0.34, p = 0.03), and D-KEFS Inhibition Time (r = 0.44, p = 0.006).

Conclusions
In a modest sample of patients with PD, severity of psychotic symptoms was related to worse auditory attention/working memory, visual learning and memory, and inhibition. No other NPI-Q score was associated with cognitive functioning. Findings support the utility of the NPI-Q in PD and the importance of considering specific neuropsychiatric factors and their implications for cognitive functioning.
A - 110 Characterization of Mild Cognitive Impairment in Multiple System Atrophy
Authors: Muscat Whitney , Assuras Stephanie 
Published: September 2024

Abstract

Objective
Multiple system atrophy (MSA) is a rare neurodegenerative disorder that primarily impacts movement and balance, as well as the autonomic nervous system. While traditionally not associated with cognitive decline, recent evidence indicates cognitive impairment is part of the disease process, typically manifesting as attentional and executive function deficits, alongside learning/memory impairments. This case contributes to MSA literature by describing mild cognitive changes in a patient with predominant autonomic dysfunction.

Method
A 68-year-old Caucasian man exhibited progressive cognitive changes over 2–3 years, with concerns emerging 5–10 years prior to neurological consultation (e.g., forgetfulness, word-finding difficulties, decline in ability to follow complex instructions). Predominant sensorimotor/physiological changes included chronic constipation, vasovagal episodes, hearing loss, orthostatic hypotension, transient vision loss, and mild dysphagia. Neuroimaging showed nonspecific mild pontine T2/FLAIR hyperintensity. Significant family history of dementia.

Results
Evaluation showed mostly normal cognitive function with some decline in executive functioning (slowed processing, reduced problem-solving, set loss, task rigidity) and mild memory decline. Despite scores within normal limits, performance likely reflects cognitive change consistent with his subjective experience, possibly masked by strong cognitive reserve (high educational and occupational attainment, research interests, social engagement).

Conclusions
Although lacking frank impairment, the patient’s history suggests a neurological process (i.e., synucleinopathy such as MSA) including history of REM sleep behavior disorder, autonomic symptoms/conditions, and neuroimaging findings suggestive of brain stem involvement. Neuropsychological evaluation is critical in differentiating MSA from other Parkinsons’s plus syndromes, as it typically presents with fewer cognitive impairments.
A - 111 Neuropsychological Functioning in those with Chronic Traumatic Encephalopathy
Authors: Iyengar Niyenth , Schaffert Jeff , Lobue Christian , Hart John , Chiang Hsueh-Sheng , Cullum C Munro 
Published: September 2024

Abstract

Objective
To differentiate neurocognitive performance between those with chronic traumatic encephalopathy neuropathologic change (CTE-NC) and matched CTE-negative individuals.

Methods
Data were obtained from the National Alzheimer’s Coordinating Center (NACC). Participants with mild cognitive impairment (MCI) or dementia and CTE-NC (n = 22) were matched approximately 1:3 to those with MCI/dementia but without CTE-NC (n = 64) on demographics (age of initial cognitive diagnosis, education, sex) and postmortem Alzheimer’s and Lewy body neuropathology. ANCOVAs (covarying for symptom duration and cognitive diagnosis) compared neurocognitive measures between groups (MMSE and neuropsychological battery composites of Attention, Executive Function, and Speed [AEFS], Memory, and Language).

Results
Participants were all male, 89.3% White, and averaged 16.3 years of education. They were, on average, 74.8 years of age (SD = 7.3) when diagnosed with MCI/dementia and had 4.2 years (SD = 4.2) of cognitive symptoms prior to MCI/dementia diagnosis. Rates of comorbid moderate/severe Alzheimer’s or Lewy body pathology were high (n = 13, 54%) in the CTE-NC group. At time of cognitive diagnosis, individuals with CTE-NC performed slightly better on the MMSE (F = 4.510, p = 0.037, CTE-NC+ M = 26.03 [1.05] vs. CTE-NC- M = 23.44 [0.58]). No differences in AEFS [F = 0.280, p = 0.599], Memory [F = 2.265, p = 0.137], or Language [F = 3.424, p = 0.069] were observed between groups.

Conclusions
Those with CTE-NC did not greatly differ in neuropsychological domains, but had relatively higher MMSE scores compared to those without CTE-NC. This is the largest clinicopathological investigation utilizing in-vivo neurocognitive data, though NACC lacks CTE-NC severity/distribution data for sensitivity analyses. Nonetheless, results have implications for CTE clinical criteria frameworks and larger clinicopathological studies are needed to determine if CTE-NC has distinct neurocognitive features.
A - 114 Exploring Alzheimer’s Disease: the Interplay of Depression, Substance Use, and Neurocognition
Authors: Venkataramanan Vaibhavi , Heath Alesha , McNerney Margaret W 
Published: September 2024

Abstract

Objective
Depression and substance use (SU) can significantly impact one’s neurocognition and are potential risk factors for Alzheimer’s Disease (ad). The project aimed to explore whether a history of depression and/or SU could predict baseline performance on neuropsychological assessment among individuals with and without ad.

Method
Data from the National Alzheimer’s Coordinating Center (NACC) was analyzed. N = 9114 participants (41.5% Male, 58.5% Female; 56.7% Normal Cognition, 43.2% ad) at their first visit were selected. A series of linear regression models, separated by presence of ad diagnosis, were run to determine if a history of depression, and/or SU, age, race, sex, education level, and APOE status would predict performance on selected neuropsychological assessments measuring facets of executive functioning (EF) (Trail Making, Digit Span, and Wechsler Adult Intelligence Scale (WAIS)).

Results
Depression was a significant predictor of performance on the WAIS in the normal cognition (β = −0.12,p &lt; 0.001) and ad (β = −0.14,p &lt; 0.001) groups, on Number of Correct Digit Span Backward Trials in the normal cognition (β = 0.09,p &lt; 0.01) and ad (β = −0.10,p &lt; 0.01) groups, and on Number of Correct Digit Span Forward Trials in the ad group (β = −0.11,p &lt; 0.001). SU was a significant predictor of performance on Number of Correct Digit Span Backward Trials in the ad group (β = 0.20,p &lt; 0.01). Demographic variables were also significant.

Conclusions
Depression predicted performance on multiple neuropsychological assessments across diagnostic groups compared to SU. EF facets measured across assessments may have been further impacted by ad and mental health history compared to those with normal cognition endorsing mental health history.
A - 115 Cognitive Predictors of Phosphorylated Skin Nerve α-Synuclein Deposition in Patients with Suspected Synucleinopathy
Authors: Rennie Katie , Kolessar Michael , Gillen Lindsey , Townley Ryan , Pleen Joseph , Parks Adam 
Published: September 2024

Abstract

Objective
Synucleinopathies are a group of neurodegenerative diseases caused by abnormal α-Synuclein aggregates and characterized by motor, autonomic, and cognitive changes, including Parkinson’s disease, Lewy Body disease, and Multiple System Atrophy. Due to variability in presentation, clinical phenotyping is challenging for diagnosis and treatment planning. Phosphorylated α-Synuclein (P-syn) detected in nerve fibers using skin biopsy shows promise in clinical phenotyping of synucleinopathies. However, cognitive predictors of P-syn deposition in synucleinopathies have not been fully explored. This study aimed to examine the utility of cognitive testing in predicting P-syn deposition in a clinical sample.

Method
A chart review of 52 older adults (Mean age = 72.8 years) referred to an outpatient neurology clinic for a diagnostic dementia workup was conducted. Clinical variables were collected, including skin biopsy (Syn-One) and cognitive test scores (Short Test of Mental Status [STMS]). P-syn was detected in 22 patients (42%) and at 68%, 41%, and 55% of central (neck) and distal (thigh and calf) biopsy sites, respectively.

Results
Overall cognition (STMS Total) did not significantly differ between patients with (Mean = 30.5) and without (Mean = 29.6) P-syn (p = 0.51). Within the P-syn positive group, STMS Registration-Trials and Calculation scores significantly predicted distally deposited P-syn (F(1,18) = 7.54, p = 0.01). This relationship was more pronounced in the thigh compared to the calf biopsy sites.

Conclusion
STMS Registration-Trials (immediate recall) and Calculation scores were significantly related to distally deposited P-syn. These findings highlight the importance of how cognitive test performance can potentially impact clinical phenotyping in synucleinopathies. Future research will examine the relationship between cognition, clinical features, and other neurodegenerative disease biomarkers.
A - 116 A Neuropsychological Testing Protocol for Assessing Lecanemab Eligibility for Alzheimer’s Disease: the First Fifty Patients
Authors: Parks Adam , Brunette Amanda , Reinhart Clare , Kreszyn Katelynn , Townley Ryan , Burns Jeffrey 
Published: September 2024

Abstract

Objective
Since the FDA approved lecanemab for Alzheimer’s disease (ad) treatment in 2023, neuropsychology’s role in the treatment eligibility determination process has become increasingly important. Not only is objective evidence of cognitive impairment required for the diagnosis of mild cognitive impairment (MCI) and mild dementia, but testing can be used to measure treatment efficacy and monitor for adverse effects. Our center developed a specialized protocol to assess cognitive and behavioral functioning for assisting in the eligibility determination process.

Method
Fifty adults (Mean Age = 73.8 years) underwent neuropsychological testing, including the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), as a part of their eligibility workup. Caregiver questionnaires were used to assess behavioral functioning. Test scores and patient outcomes were explored.

Results
Thirteen patients (26%) were prescribed lecanemab. Another 13 patients were deemed ineligible (five due to their cognitive results), eight patients (16%) were undecided, and 16 patients (32%) had not yet completed their workup. Of those who initiated lecanemab, eight patients (62%) had a diagnosis of MCI and six were diagnosed with mild dementia. Of these patients, eight (82%) showed an amnestic pattern on testing (RBANS Delayed Recall Standard Score Mean = 46.8). Additional analyses involving ad biomarkers, neuroimaging, and APOE status, were conducted.

Conclusion
Over one quarter of patients referred for neuropsychological testing as a part of their eligibility determination initiated lecanemab treatment for ad. Cognitive testing was directly related to determining ineligibility in 10% of patients. This review of an initial lecanemab treatment cohort highlights the importance of neuropsychology in the eligibility determination process.
A - 117 Double Trouble: a Case Study on Cardiac Amyloidosis and Alzheimer’s Disease
Authors: Ismail Hajar , Lacy Maureen 
Published: September 2024

Abstract

Objective
Transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure amongst older adults. The median age upon diagnosis is 75 with 90% of patients reported as men. The amyloidogenic protein is transthyretin (TTR), which is primarily produced in the liver, but is also produced in the choroid plexus and neurons under distress. There are two types of ATTR: an autosomal dominant and a wild-type (wt) variant. ATTRwt is the amyloid precursor in the cardiac depositions found in older adults. We present the first neuropsychological evaluation of a patient with ATTRwt.

Method
An 87-year-old right-handed White woman reported a 2 year history of slowly progressive short term memory decline. Her family noted rapid cognitive decline and paranoia approximately 6 months ago. Medical history was notable for heart failure, along with lumbar stenosis, carpal tunnel syndrome, arthritis, scoliosis, and hyperlipidemia. ATTRwt diagnosis was made during cardiac clearance for lumbar surgery. Neuroimaging documented age-related cerebral atrophy and chronic microvascular ischemic disease.

Results
Inspection of data revealed an amnestic presentation, with impaired confrontational naming, spatial judgment, and processing speed performances. She denied depressogenic and anxiolytic symptoms while family endorsed symptoms of apathy and executive dysfunction.

Conclusions
The relationship between cardiac and nervous systems has garnished increasing attention in the literature and in clinical planning. Overall, the relationship between cardiac TTR amyloid and brain amyloid beta deposition in humans is unclear. Neuropsychologists should be aware of this heart condition that is increasingly recognized in older adults and the intriguing findings implicated around the cardiac-brain axis.