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19 Accuracy of Self- and Informant-Reported Domain-Specific Cognitive Ratings
- Haley S Kohl, Allyson Gregoire, Jonathan Reader, Arijit Bhaumik, Bruno Giordani, Henry Paulson, Benjamin Hampstead, Annalise Rahman-Filipiak
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- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 702-703
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Objective:
Diagnostic criteria for mild cognitive impairment (MCI) include a report of cognitive decline from the patient or a close informant. It is therefore important to understand the relationship between self- and informant-rated cognition and actual patient performance. Furthermore, it is unknown whether the nature of the relationship between the patient and their informant impacts accuracy of subjective reports. This study aimed to determine the association between informant report, self-report and objective cognitive performance based on relationship factors. We predicted that informant report would be more closely associated with objective performance than self-report after controlling for demographics and mood (Geriatric Depression Scale [mean= 1.4, SD=2]), especially among those who live with the participant and those who are spouses/partners.
Participants and Methods:Participants (n = 338; age= 73.5 ±6.7) of varying diagnoses and their respective informants were drawn from the longitudinal cohort of the Michigan Alzheimer’s Disease Research Center (MADRC). The majority of informants were spouses/significant others (55.6%), followed by 23.7% being other family members and 20.7% were non- family members; 58.9% of informants live with the participant. Both respondents completed the Cognitive Change Index (CCI) to rate the patient’s cognitive status (higher scores indicating worsening cognition) across three domains: memory (12 questions), language (1 question), and attention/executive functioning (7 questions). These domains were matched to objective cognitive performance measured using the MADRC neuropsychological battery. Executive functioning and attention were assessed using Number Span Test Forward and Backward (NSF, NSB) and Trail Making Test Part B and Trail- Making Test Part A and B ratio (TMTB, TMTB: A); memory was measured using Craft Story 21 (Immediate and Delayed), Hopkins Verbal Learning Test-Revised (HVLT-R) Total Recall, Delayed Recall, and Benson Complex Figure (BCF) Delayed Recall; and Language was measured by the Controlled Oral Word Association Test (COWAT) and Animal fluency.
Results:Linear regression adjusted for sex, race, and mood indicated that both patient and informant CCI ratings were significantly (p<.05) associated with objective cognitive performance. For every one unit increase on executive CCI items, there was a significant decline in executive functioning (NSF patient and informant ß= -0.09, NSB: [ßP= -.14; ßp-0.13]) and TMTB [ßP= 3.85; ß= 3.10 [% change]). Memory performance also declined per unit increase on CCI memory items: (Craft Story 21 Immediate [ßP=-0.32; ß= -0.37] and Delayed [ßP=-.40; ßp -.47], HVLT-R Total Recall [ßP= -.31; ßI=-.37] and Delayed Recall [ßP= -.16; ß=-.20], and BCF Delayed Recall [ßP= -.18; ß= -.23]. Similarly, one unit increase on the single CCI language item was associated with a decline in COWAT (ßP= -2.27; ß= -4.61) and Animal fluency (ßP= -1.88; ß= -3.03). Effect modification by participant-informant relationship type or participant-informant cohabitation was not significant.
Conclusions:Patient and informant ratings are associated with objective measures of cognition regardless of the relationship between informant and patient or if they live together. This study was limited by a well-educated sample (mean= 16.1 years of education, SD= 2.4 years) with relatively limited diversity among participant-informant relationships. Future studies should replicate analyses across a larger and more diverse sample.
97 Evaluation of Video and Telephone-Based Administration of the Uniform Data Set Version 3 (UDS v3.0) Teleneuropsychological Measures
- Theresa F. Gierzynski, Allyson Gregoire, Jonathan M. Reader, Rebecca Pantis, Stephen Campbell, Arijit Bhaumik, Annalise Rahman-Filipiak, Judith Heidebrink, Bruno Giordani, Henry Paulson, Benjamin M. Hampstead
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- Journal:
- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 499-500
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Objective:
Telecommunication-assisted neuropsychological assessment (teleNP) has become more widespread, particularly in response to the COVID-19 pandemic. However, comparatively few studies have evaluated in-home teleNP testing and none, to our knowledge, have evaluated the National Alzheimer’s Coordinating Center’s (NACC) Uniform Data Set version 3 tele-adapted test battery (UDS v3.0 t-cog). The current study compares in-home teleNP administration of the UDS v3.0, acquired while in-person activities were suspended due to COVID-19, with a prior in-person UDS v3.0 evaluation.
Participants and Methods:210 participants from the Michigan Alzheimer’s Disease Research Center’s longitudinal study of memory and aging completed both an in-person UDS v3.0 and a subsequent teleNP UDS v3.0 evaluation. The teleNP UDS v3.0 was administered either via video conference (n = 131), telephone (n = 75), or hybrid format (n = 4) with approximately 16 months between evaluations (mean = 484.7 days; SD = 122.4 days; range = 320-986 days). The following clinical phenotypes were represented at the initial assessment period (i.e., the most recent in-person UDS v3.0 evaluation prior to the teleNP UDS v3.0): cognitively healthy (n = 138), mild cognitive impairment (MCI; n = 60), dementia (n = 11), and impaired not MCI (n = 1). Tests included both the in-person and teleNP UDS v3.0 measures, as well as the Hopkins Verbal Learning Test-Revised (HVLT-R) and Letter “C” Fluency.
Results:We calculated intraclass correlation coefficients (ICC) with raw scores from each time point for the entire sample. Sub-analyses were conducted for each phenotype among participants with an unchanged consensus research diagnosis: cognitively healthy (n = 122), MCI (n = 47), or cognitively impaired (i.e., MCI, dementia, and impaired not MCI) (n = 66). Test-retest reliability across modalities and clinical phenotypes was, in general, moderate. The poorest agreement was associated with the Trail Making Test (TMT) - A (ICC = 0.00; r = 0.027), TMT - B (ICC = 0.26; r = 0.44), and Number Span Backward (ICC = 0.49). The HVLT-R demonstrated moderate reliability overall (ICC = 0.51-0.68) but had notably weak reliability for cognitively healthy participants (ICC = 0.12-0.36). The most favorable reliability was observed in Craft Story 21 Recall - Delayed (ICC = 0.77), Letter Fluency (C, F, and L) (ICC = 0.74), Multilingual Naming Test (MINT) (ICC = 0.75), and Benson Complex Figure – Delayed (ICC = 0.79).
Conclusions:Even after accounting for the inherent limitations of this study (e.g., significant lapse of time between testing intervals), our findings suggest that the UDS v3.0 teleNP battery shows only modest relationships with its in-person counterpart. Particular caution should be used when interpreting measures showing questionable reliability, though we encourage further investigation of remote vs. in-person testing under more controlled conditions.
Evaluation of the Uniform Data Set version 3 teleneuropsychological measures
- Theresa F. Gierzynski, Allyson Gregoire, Jonathan M. Reader, Rebecca Pantis, Stephen Campbell, Arijit Bhaumik, Annalise Rahman-Filipiak, Judith Heidebrink, Bruno Giordani, Henry Paulson, Benjamin M. Hampstead
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- Journal:
- Journal of the International Neuropsychological Society / Volume 30 / Issue 2 / February 2024
- Published online by Cambridge University Press:
- 27 June 2023, pp. 183-193
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Objective:
Few studies have evaluated in-home teleneuropsychological (teleNP) assessment and none, to our knowledge, has evaluated the National Alzheimer’s Coordinating Center’s (NACC) Uniform Data Set version 3 tele-adapted test battery (UDS v3.0 t-cog). The current study evaluates the reliability of the in-home UDS v3.0 t-cog with a prior in-person UDS v3.0 evaluation.
Method:One hundred and eighty-one cognitively unimpaired or cognitively impaired participants from a longitudinal study of memory and aging completed an in-person UDS v3.0 and a subsequent UDS v3.0 t-cog evaluation (∼16 months apart) administered either via video conference (n = 122) or telephone (n = 59).
Results:We calculated intraclass correlation coefficients (ICCs) between each time point for the entire sample. ICCs ranged widely (0.01–0.79) but were generally indicative of “moderate” (i.e., ICCs ranging from 0.5–0.75) to “good” (i.e., ICCs ranging from 0.75–0.90) agreement. Comparable ICCs were evident when looking only at those with stable diagnoses. However, relatively stronger ICCs (Range: 0.35–0.87) were found between similarly timed in-person UDS v3.0 evaluations.
Conclusions:Our findings suggest that most tests on the UDS v3.0 t-cog battery may serve as a viable alternative to its in-person counterpart, though reliability may be attenuated relative to the traditional in-person format. More tightly controlled studies are needed to better establish the reliability of these measures.
Cognitive Indicators of Preclinical Behavioral Variant Frontotemporal Dementia in MAPT Carriers
- Gayathri Cheran, Liwen Wu, Seonjoo Lee, Masood Manoochehri, Sarah Cines, Emer Fallon, Timothy Lynch, Judith Heidebrink, Henry Paulson, Jill Goldman, Edward Huey, Stephanie Cosentino
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- Journal:
- Journal of the International Neuropsychological Society / Volume 25 / Issue 2 / February 2019
- Published online by Cambridge University Press:
- 21 November 2018, pp. 184-194
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Objectives: The cognitive indicators of preclinical behavioral variant Frontotemporal Dementia (bvFTD) have not been identified. To investigate these indicators, we compared cross-sectional performance on a range of cognitive measures in 12 carriers of pathogenic MAPT mutations not meeting diagnostic criteria for bvFTD (i.e., preclinical) versus 32 demographically-matched familial non-carriers (n = 44). Studying preclinical carriers offers a rare glimpse into emergent disease, environmentally and genetically contextualized through comparison to familial controls. Methods: Evaluating personnel blinded to carrier status administered a standardized neuropsychological battery assessing attention, speed, executive function, language, memory, spatial ability, and social cognition. Results from mixed effect modeling were corrected for multiplicity of comparison by the false discovery rate method, and results were considered significant at p < .05. To control for potential interfamilial variation arising from enrollment of six families, family was treated as a random effect, while carrier status, age, gender, and education were treated as fixed effects. Results: Group differences were detected in 17 of 31 cognitive scores and spanned all domains except spatial ability. As hypothesized, carriers performed worse on specific measures of executive function, and social cognition, but also on measures of attention, speed, semantic processing, and memory storage and retrieval. Conclusions: Most notably, group differences arose on measures of memory storage, challenging long-standing ideas about the absence of amnestic features on neuropsychological testing in early bvFTD. Current findings provide important and clinically relevant information about specific measures that may be sensitive to early bvFTD, and advance understanding of neurocognitive changes that occur early in the disease. (JINS, 2019, 25, 184–194)
47 - Autosomal dominant cerebellar ataxia
- from Part VIII - Cerebellar degenerations
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- By Henry L. Paulson, Department of Neurology, University of Iowa Hospitals and Clinics, IA, USA
- M. Flint Beal, Cornell University, New York, Anthony E. Lang, University of Toronto, Albert C. Ludolph, Universität Ulm, Germany
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- Book:
- Neurodegenerative Diseases
- Published online:
- 04 August 2010
- Print publication:
- 02 June 2005, pp 709-718
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Summary
This review discusses clinical and genetic features of dominantly inherited ataxia. In addition, potential pathogenic mechanisms are discussed with a special focus on the polyglutamine disorders, since most is known about this group of diseases.
Classification of ADCA
Most adult onset hereditary ataxias are dominantly inherited. Harding (1993) clinically divided autosomal dominant cerebellar ataxia (ADCA) into four types, I through IV. Type I ADCA represents cerebellar disease accompanied by brainstem signs. ADCA type II is similar to type I but also includes retinopathy. ADCA type III represents later onset, “pure” cerebellar disease and ADCA type IV represents episodic ataxia.
A newer, favored classification for ADCA reflects the growing number of identified genetic loci, each of which is designated a specific spinocerebellar ataxia or SCA (Table 47.1). As SCAs are mapped to loci, they are assigned numbers – SCA1, SCA2 and so forth, currently up to 21 at the time of this writing. Most SCAs fall within Harding's ADCA type I classification. There is considerable clinical overlap among the ADCA type I group, even within families. In contrast, the sole genetic cause of ADCA type II appears to be SCA7, the only dominant ataxia routinely accompanied by retinal degeneration. Most patients with SCA6 (and several rarer SCAs for which the gene defects have not been identified) manifest as a pure cerebellar syndrome and thus fall within ADCA III. Finally, ADCA type IV currently consists of two genetically identified forms of episodic ataxia (EA), EA-1 and EA-2, caused by mutations in a potassium and calcium channel respectively.