Conflict monitoring in early frontotemporal dementia

Krueger, Casey E.; Bird, A.; Growdon, Matthew E.; Jang, Jung Yun; Miller, Bruce L.; Kramer, Joel H.

doi:10.1212/wnl.0b013e3181b04b24

Cited by 40 publications

(35 citation statements)

References 39 publications

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“…Patients who showed impaired values on 2 or more scores were classified as having impaired neuropsychological performance (INP group). Similar procedures for classifying ‘high’- and ‘low’-performance bvFTD patients have previously been reported in the literature [1]. Preliminary analysis revealed that all participants originally recruited for the control group had normal neuropsychological performance, which is why they were all clustered into a unique control group (CTR).…”

Section: Methodsmentioning

confidence: 88%

“…In clinical practice, it is not uncommon to find early behavioral-variant frontotemporal dementia (bvFTD) patients who, to a considerable extent, are intellectually unimpaired [1,2], while relatives and caregivers depict a strikingly different picture: they claim that these patients show severe changes in their behavior and real-life decision-making skills. Although patients with frontal lobe damage usually maintain normal intellectual functioning, they develop difficulties in planning workday and future events, and in choosing friends, partners and activities, and their actions frequently lead to diverse losses in terms of financial status and social standing [3].…”

Section: Introductionmentioning

confidence: 99%

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Decision-Making in Frontotemporal Dementia: Clinical, Theoretical and Legal Implications

Manes¹,

Torralva²,

Ibáñez³

et al. 2011

Dement Geriatr Cogn Disord

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Background: The behavioral variant of frontotemporal dementia (bvFTD) is characterized by progressive changes in personality and social interaction, loss of empathy, disinhibition and impulsivity, most of which generally precede the onset of cognitive deficits. In this study, we investigated decision-making cognition in a group of patients with an early bvFTD diagnosis whose standard neuropsychological performance was within normal range for all variables. Methods: The Iowa Gambling Task was administered to this group of early bvFTD patients, to a group of early bvFTD patients who had shown impaired performance on the classical neuropsychological battery and to healthy controls. Results: Decision-making was impaired in both bvFTD patient groups, whether they had shown impaired or normal performance in the classical neuropsychological evaluation. Conclusions: Patients with early bvFTD may perform normally on standard cognitive tests, and yet develop severe deficits in judgment and decision-making. In many current legal systems, early bvFTD patients showing preserved cognitive functioning who commit unlawful acts run the risk of not being able to plead insane or not guilty on the grounds of diminished responsibility beyond reasonable doubt. This represents a unique legal and ethical dilemma. Our findings have important implications for medicolegal decisions relating to capacity and culpability, and regarding the philosophical concept of ‘free will’.

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Section: Methodsmentioning

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Decision-Making in Frontotemporal Dementia: Clinical, Theoretical and Legal Implications

Manes¹,

Torralva²,

Ibáñez³

et al. 2011

Dement Geriatr Cogn Disord

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“…Patients were dichotomized into those who scored within normal limits on standard tasks of executive function and those who did not, and their scores on the Flanker task were compared. Interestingly, both bvFTD patient groups showed a signi fi cantly larger congruency effect (e.g., longer latency on incongruent vs. congruent trials) compared to the normal control subjects [ 93 ] . These results suggest that even those patients who perform well on standard tasks of executive function may still have subtle, yet perceptible de fi cits in cognitive control, if measured by the appropriate method.…”

Section: Attention/executive Functions In Bvftdmentioning

confidence: 89%

“…Investigation of this domain using "traditional" tasks of executive function has led to largely con fl icting fi ndings. While some studies fi nd impairments in this domain [ 71,[91][92][93] , others do not [94][95][96] . One reason for this discrepancy likely relates to stage of disease at which patients are assessed.…”

Section: Attention/executive Functions In Bvftdmentioning

confidence: 98%

“…It is possible, however, that experimental measures of executive function may be more sensitive to subtle declines. For example, Krueger et al [ 93 ] administered traditional tasks of executive function, as well as a computerized Flanker task (measuring cognitive control) to patients with bvFTD and healthy control subjects. Patients were dichotomized into those who scored within normal limits on standard tasks of executive function and those who did not, and their scores on the Flanker task were compared.…”

Section: Attention/executive Functions In Bvftdmentioning

confidence: 99%

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Accurate Assessment of Behavioral Variant Frontotemporal Dementia

LaMarre

Kramer

2012

Handbook on the Neuropsychology of Aging and Dementia

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Behavioral variant Frontotemporal dementia (bvFTD) is a neurodegenerative syndrome characterized by profound changes in personality and behavior, including social disinhibition, loss of empathy, apathy and compulsive behaviors. While cognitive decline does occur (typically beginning with executive dysfunction), these issues tend to emerge mid-disease course, rather than early on. Onset is insidious, typically beginning between ages 45-65 and prevalence is equal to Alzheimer's disease (AD) in individuals under the age of 65. Despite signifi cant advancements in our understanding of bvFTD over the past 12 years, misdiagnosis remains common. For example, a signifi cant subset of individuals with bvFTD initially receive a diagnosis of early-onset AD, or late life psychiatric disturbance. Given their expertise in the assessment of cognition, behavior and emotion, neuropsychologists can play an important role in the differential diagnosis and management of this disease. This chapter begins with an up-to-date discussion of the clinical, neuropathological and genetic features of the disease, and then moves into a review of the neuropsychological literature. A structured discussion of key aspects to cover in a neuropsychological assessment is provided, and a case example of a 'typical' bvFTD patient is presented. Behavioral variant frontotemporal dementia (bvFTD) is one of three neurodegenerative syndromes that are collectively referred to as frontotemporal dementia (FTD). Initially thought to be rare, we now know that it is equally as common as Alzheimer's disease in individuals under the age of 65 [ 1 ] and is the third most common dementia after Alzheimer's disease (AD) and dementia with Lewy bodies (DLB) [ 2 ] . While estimates of the prevalence of FTD vary, population-based studies in both the United States and United Kingdom estimate a sporadic occurrence at around 3.3-3.5/100,000 in individuals between 45 and 65 years of age [ 3,4 ] . Age of onset is typically in the presenium, though onset ranges considerably from the 30s to 90s [ 1,5,6 ] . The usual duration of FTD ranges from 8 to 11 years [ 7 ] .Clinically, FTD is expressed as three main variants [ 8 ] . BvFTD is characterized by profound and early changes in personality and behavior [ 8 ] . This phenotype is most common and accounts for approximately 70% of the clinical expression of the disease [ 9 ] . As such, bvFTD will be the focus of this chapter. The other two variants are primary progressive aphasic syndromes. The semantic variant (svPPA) is associated with the loss of word knowledge (e.g., semantic structure of language), while the non fl uent variant (nfPPA) is characterized by early disturbances in motor speech output and loss of syntax (e.g., grammatical structure of language) [ 8,10 ] . These two variants account for approximately 15% and 10% of the phenotypic expression of the disease, respectively [ 9 ] . Gender distribution tends to vary by clinical syndrome. Many studies fi nd a male bias in bvFTD and svPPA and a female bias in nfPPA [ ...

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Reliability and Validity of Smartphone Cognitive Testing for Frontotemporal Lobar Degeneration

Staffaroni,

Clark,

Taylor

et al. 2024

JAMA Netw Open

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ImportanceFrontotemporal lobar degeneration (FTLD) is relatively rare, behavioral and motor symptoms increase travel burden, and standard neuropsychological tests are not sensitive to early-stage disease. Remote smartphone-based cognitive assessments could mitigate these barriers to trial recruitment and success, but no such tools are validated for FTLD.ObjectiveTo evaluate the reliability and validity of smartphone-based cognitive measures for remote FTLD evaluations.Design, Setting, and ParticipantsIn this cohort study conducted from January 10, 2019, to July 31, 2023, controls and participants with FTLD performed smartphone application (app)–based executive functioning tasks and an associative memory task 3 times over 2 weeks. Observational research participants were enrolled through 18 centers of a North American FTLD research consortium (ALLFTD) and were asked to complete the tests remotely using their own smartphones. Of 1163 eligible individuals (enrolled in parent studies), 360 were enrolled in the present study; 364 refused and 439 were excluded. Participants were divided into discovery (n = 258) and validation (n = 102) cohorts. Among 329 participants with data available on disease stage, 195 were asymptomatic or had preclinical FTLD (59.3%), 66 had prodromal FTLD (20.1%), and 68 had symptomatic FTLD (20.7%) with a range of clinical syndromes.ExposureParticipants completed standard in-clinic measures and remotely administered ALLFTD mobile app (app) smartphone tests.Main Outcomes and MeasuresInternal consistency, test-retest reliability, association of smartphone tests with criterion standard clinical measures, and diagnostic accuracy.ResultsIn the 360 participants (mean [SD] age, 54.0 [15.4] years; 209 [58.1%] women), smartphone tests showed moderate-to-excellent reliability (intraclass correlation coefficients, 0.77-0.95). Validity was supported by association of smartphones tests with disease severity (r range, 0.38-0.59), criterion-standard neuropsychological tests (r range, 0.40-0.66), and brain volume (standardized β range, 0.34-0.50). Smartphone tests accurately differentiated individuals with dementia from controls (area under the curve [AUC], 0.93 [95% CI, 0.90-0.96]) and were more sensitive to early symptoms (AUC, 0.82 [95% CI, 0.76-0.88]) than the Montreal Cognitive Assessment (AUC, 0.68 [95% CI, 0.59-0.78]) (z of comparison, −2.49 [95% CI, −0.19 to −0.02]; P = .01). Reliability and validity findings were highly similar in the discovery and validation cohorts. Preclinical participants who carried pathogenic variants performed significantly worse than noncarrier family controls on 3 app tasks (eg, 2-back β = −0.49 [95% CI, −0.72 to −0.25]; P &lt; .001) but not a composite of traditional neuropsychological measures (β = −0.14 [95% CI, −0.42 to 0.14]; P = .32).Conclusions and RelevanceThe findings of this cohort study suggest that smartphones could offer a feasible, reliable, valid, and scalable solution for remote evaluations of FTLD and may improve early detection. Smartphone assessments should be considered as a complementary approach to traditional in-person trial designs. Future research should validate these results in diverse populations and evaluate the utility of these tests for longitudinal monitoring.

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Conflict monitoring in early frontotemporal dementia

Abstract: Background: Despite the extensive frontal atrophy and behavioral disinhibition that characterizes

Cited by 40 publications

References 39 publications

Decision-Making in Frontotemporal Dementia: Clinical, Theoretical and Legal Implications

Decision-Making in Frontotemporal Dementia: Clinical, Theoretical and Legal Implications

Accurate Assessment of Behavioral Variant Frontotemporal Dementia

Reliability and Validity of Smartphone Cognitive Testing for Frontotemporal Lobar Degeneration

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