Validity and Reliability of the Frontotemporal Dementia Rating Scale (FTD-FRS) for the Progression and Staging of Dementia in Brazilian Patients

Lima-Silva, Thaís Bento; Bahia, Valéria Santoro; Cecchini, Mário Amore; Cassimiro, Luciana; Guimarães, Henrique Cerqueira; Gambogi, Leandro Boson; Caramelli, Paulo; Balthazar, Márcio Luiz Figueredo; Damasceno, Benito; Brucki, Sônia Maria Dozzi; Souza, Leonardo Cruz de; Mioshi, Eneida; Yassuda, Mônica Sanches

doi:10.1097/wad.0000000000000246

Cited by 8 publications

(15 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Vice versa, a number of cases with a global rating of 0.5, or in a small number a rating of 1, scored 100% on the FRS (indicating zero behavioural or functional changes). In line with previous studies, 22 23 our data suggest that the CDR+NACC FTLD may be more likely to underestimate disease severity when compared with FRS scores: 41% of cases with an asymptomatic CDR+NACC FTLD global rating had a degree of disability or behavioural change according to the FRS, vs 16% of cases with an asymptomatic FRS score having some symptomatology according to the CDR+NACC FTLD. Although the scales both broadly centre around everyday functioning and behaviour, there are differences between them e.g.…”

Section: Discussionsupporting

confidence: 91%

See 1 more Smart Citation

Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort

Peakman

Russell

Convery

et al. 2021

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

BackgroundTherapeutic trials are now underway in genetic forms of frontotemporal dementia (FTD) but clinical outcome measures are limited. The two most commonly used measures, the Clinical Dementia Rating (CDR)+National Alzheimer’s Disease Coordinating Center (NACC) Frontotemporal Lobar Degeneration (FTLD) and the FTD Rating Scale (FRS), have yet to be compared in detail in the genetic forms of FTD.MethodsThe CDR+NACC FTLD and FRS were assessed cross-sectionally in 725 consecutively recruited participants from the Genetic FTD Initiative: 457 mutation carriers (77 microtubule-associated protein tau (MAPT), 187 GRN, 193 C9orf72) and 268 family members without mutations (non-carrier control group). 231 mutation carriers (51 MAPT, 92 GRN, 88 C9orf72) and 145 non-carriers had available longitudinal data at a follow-up time point.ResultsCross-sectionally, the mean FRS score was lower in all genetic groups compared with controls: GRN mutation carriers mean 83.4 (SD 27.0), MAPT mutation carriers 78.2 (28.8), C9orf72 mutation carriers 71.0 (34.0), controls 96.2 (7.7), p<0.001 for all comparisons, while the mean CDR+NACC FTLD Sum of Boxes was significantly higher in all genetic groups: GRN mutation carriers mean 2.6 (5.2), MAPT mutation carriers 3.2 (5.6), C9orf72 mutation carriers 4.2 (6.2), controls 0.2 (0.6), p<0.001 for all comparisons. Mean FRS score decreased and CDR+NACC FTLD Sum of Boxes increased with increasing disease severity within each individual genetic group. FRS and CDR+NACC FTLD Sum of Boxes scores were strongly negatively correlated across all mutation carriers (rs=−0.77, p<0.001) and within each genetic group (rs=−0.67 to −0.81, p<0.001 in each group). Nonetheless, discrepancies in disease staging were seen between the scales, and with each scale and clinician-judged symptomatic status. Longitudinally, annualised change in both FRS and CDR+NACC FTLD Sum of Boxes scores initially increased with disease severity level before decreasing in those with the most severe disease: controls −0.1 (6.0) for FRS, −0.1 (0.4) for CDR+NACC FTLD Sum of Boxes, asymptomatic mutation carriers −0.5 (8.2), 0.2 (0.9), prodromal disease −2.3 (9.9), 0.6 (2.7), mild disease −10.2 (18.6), 3.0 (4.1), moderate disease −9.6 (16.6), 4.4 (4.0), severe disease −2.7 (8.3), 1.7 (3.3). Sample sizes were calculated for a trial of prodromal mutation carriers: over 180 participants per arm would be needed to detect a moderate sized effect (30%) for both outcome measures, with sample sizes lower for the FRS.ConclusionsBoth the FRS and CDR+NACC FTLD measure disease severity in genetic FTD mutation carriers throughout the timeline of their disease, although the FRS may be preferable as an outcome measure. However, neither address a number of key symptoms in the FTD spectrum, for example, motor and neuropsychiatric deficits, which future scales will need to incorporate.

show abstract

Section: Discussionsupporting

confidence: 91%

“…21 The scale captures six levels of impairment from very mild to profound. Disease severity according to the FRS has been found to correlate with the CDR 21 22 and CDR+NACC-FTLD, 23 but a detailed evaluation of the measure across the range of presymptomatic and symptomatic FTD has not been reported.…”

Section: Neurodegenerationmentioning

confidence: 99%

Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort

Peakman

Russell

Convery

et al. 2021

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

show abstract

“…Given the need to develop specific instruments for staging typical symptoms of frontotemporal dementia variants, another scale, the Frontotemporal Dementia Rating Scale (FTD-FRS), was also developed [37]. The validity and reliability of FTD-FRS Brazilian version was recently published [38], allowing its use in future studies. Another limitation of the present study was that we could not examine the effect of informant’s distress on NPI scores because the NPI Distress (NPI-D) scale was not available for all the sample.…”

Section: Discussionmentioning

confidence: 99%

Neuropsychiatric Inventory in Community-Dwelling Older Adults with Mild Cognitive Impairment and Dementia

Nunes

Schwarzer

Leite

et al. 2019

JAD

Self Cite

View full text Add to dashboard Cite

Background Behavioral and psychological symptoms (BPSD) can be a prodrome of dementia, and the Neuropsychiatric Inventory (NPI) is widely used for BPSD evaluation. Objective To compare the prevalence of BPSD according to cognitive status, and to determine NPI cutoffs that best discern individuals with mild cognitive impairment (MCI) and dementia from those without dementia. Methods We included 1,565 participants (mean age = 72.7 ± 12.2 years, 48% male). BPSD and cognitive status were assessed with the NPI and the Clinical Dementia Rating (CDR). We used multivariable logistic regression models to investigate the association of BPSD with cognitive status. The area under the curve (AUC) was used to assess model discrimination, and to determine the best NPI cutoff for MCI and dementia. Results Participants were cognitively normal (CDR = 0; n = 1,062), MCI (CDR = 0.5; n = 145), or dementia (CDR ≥ 1.0, n = 358). NPI symptoms were more frequent in dementia and MCI when compared to cognitively normal. Higher odds for delusions, hallucinations, disinhibition, and psychomotor alterations were found among participants with dementia and MCI than in those who were cognitively normal. The best NPI cutoff to discern participants with dementia from those cognitively normal was 11 (AUC = 0.755). Poor discrimination (AUC = 0.563) was found for the comparison of MCI and those cognitively normal. Conclusions We found an increase in BPSD frequencies across the continuum of cognitive impairment. BPSD severity and frequency in MCI was more similar to individuals cognitively normal than with dementia. NPI scores ≥ to 11 in individuals with no diagnosis of dementia can support the decision for further investigation of dementia.

show abstract

“…The CDR is a semi-structured interview administered to the patient and to the primary caregiver, which provides information on six specific domains (memory, orientation, judgment and problem solving, community affairs, home, hobbies, and self-care). Each domain and the scale as a whole reports values ranging from low to high severity: 0 (no impairment), 0.5 (very mild), 1 (mild), 2 (moderate), and 3 (severe) ( 252 ). However, the CDR was developed based primarily on AD symptoms, making it a less sensitive scale for other types of dementia, such as FTD ( 30 , 249 , 253 ).…”

Section: Resultsmentioning

confidence: 99%

Multidimensional Clinical Assessment in Frontotemporal Dementia and Its Spectrum in Latin America and the Caribbean: A Narrative Review and a Glance at Future Challenges

et al. 2022

View full text Add to dashboard Cite

Frontotemporal dementia (FTD) is the third most common form of dementia across all age groups and is a leading cause of early-onset dementia. The Frontotemporal dementia (FTD) includes a spectrum of diseases that are classified according to their clinical presentation and patterns of neurodegeneration. There are two main types of FTD: behavioral FTD variant (bvFTD), characterized by a deterioration in social function, behavior, and personality; and primary progressive aphasias (PPA), characterized by a deficit in language skills. There are other types of FTD-related disorders that present motor impairment and/or parkinsonism, including FTD with motor neuron disease (FTD-MND), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). The FTD and its associated disorders present great clinical heterogeneity. The diagnosis of FTD is based on the identification through clinical assessments of a specific clinical phenotype of impairments in different domains, complemented by an evaluation through instruments, i.e., tests and questionnaires, validated for the population under study, thus, achieving timely detection and treatment. While the prevalence of dementia in Latin America and the Caribbean (LAC) is increasing rapidly, there is still a lack of standardized instruments and consensus for FTD diagnosis. In this context, it is important to review the published tests and questionnaires adapted and/or validated in LAC for the assessment of cognition, behavior, functionality, and gait in FTD and its spectrum. Therefore, our paper has three main goals. First, to present a narrative review of the main tests and questionnaires published in LAC for the assessment of FTD and its spectrum in six dimensions: (i) Cognitive screening; (ii) Neuropsychological assessment divided by cognitive domain; (iii) Gait assessment; (iv) Behavioral and neuropsychiatric symptoms; (v) Functional assessment; and (vi) Global Rating Scale. Second, to propose a multidimensional clinical assessment of FTD in LAC identifying the main gaps. Lastly, it is proposed to create a LAC consortium that will discuss strategies to address the current challenges in the field.

show abstract

Validity and Reliability of the Frontotemporal Dementia Rating Scale (FTD-FRS) for the Progression and Staging of Dementia in Brazilian Patients

Cited by 8 publications

References 29 publications

Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort

Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort

Neuropsychiatric Inventory in Community-Dwelling Older Adults with Mild Cognitive Impairment and Dementia

Multidimensional Clinical Assessment in Frontotemporal Dementia and Its Spectrum in Latin America and the Caribbean: A Narrative Review and a Glance at Future Challenges

Contact Info

Product

Resources

About