Abstract

Background: The behavioral variant of frontotemporal dementia (bvFTD), characterized by early behavioral abnormalities and late memory impairment, is a neurodegenerative disorder with a detrimental impact on patients and their caregivers. bvFTD is often difficult to distinguish from other neurodegenerative diseases, such as Alzheimer's disease (AD), using brief cognitive tests. Combining brief socio-cognitive and behavioral evaluations with standard cognitive testing could better discriminate bvFTD from AD patients. We sought to evaluate the diagnostic accuracy of brief socio-cognitive tests that may differentiate bvFTD and AD patients with low educational levels.Methods: A prospective study was performed on 51 individuals over the age of 50 with low educational levels, with bvFTD or AD diagnosed using published criteria, and who were receiving neurological care at a multidisciplinary neurology clinic in Lima, Peru, between July 2017 and December 2020. All patients had a comprehensive neurological evaluation, including a full neurocognitive battery and brief tests of cognition (Addenbrooke's Cognitive Examination version III, ACE-III), social cognition (Mini-social Cognition and Emotional Assessment, Mini-SEA), and behavioral assessments (Frontal Behavioral Inventory, FBI; Interpersonal Reactivity Index—Emphatic Concern, IRI-EC; IRI—Perspective Taking, IRI-PT; and Self-Monitoring Scale—revised version, r-SMS). Receiver operating characteristic (ROC) analysis to calculate the area under the curve (AUC) was performed to compare the brief screening tests individually and combined to the gold standard of bvFTD and AD diagnoses.Results: The AD group was significantly older than the bvFTD group (p < 0.001). An analysis of the discriminatory ability of the ACE-III to distinguish between patients with AD and bvFTD (AUC = 0.85) and the INECO Frontal Screening (IFS; AUC = 0.78) shows that the former has greater discriminatory ability. Social and behavioral cognition tasks were able to appropriately discriminate bvFTD from AD. The Mini-SEA had high sensitivity and high moderate specificity (83%) for discriminating bvFTD from AD, which increased when combined with the brief screening tests ACE-III and IFS. The FBI was ideal with high sensitivity (83%), as well as the IRI-EC and IRI-PT that also were adequate for distinguishing bvFTD from AD.Conclusions: Our study supports the integration of socio-behavioral measures to the standard global cognitive and social cognition measures utilized for screening for bvFTD in a population with low levels of education.

Highlights

  • The prevalence of frontotemporal dementia, a neurodegenerative disease characterized by difficulties with memory often preceded by significant behavioral changes, has been reported to range from two in 100,000 to 31 in 100,000 [1]

  • The Alzheimer’s disease (AD) group was significantly older than the behavioral variant of frontotemporal dementia (bvFTD) group (p < 0.001), but years of education (p = 0.4101), female sex (p = 0.394), and disease duration (p = 0.2407) were similar between groups

  • We observed greater disease severity in patients with bvFTD measured by the Clinical Dementia Rating Scale (CDR) sum of boxes scale; the difference in disease severity between the two groups was not statistically significant (P = 0.8461; Table 1)

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Summary

Introduction

The prevalence of frontotemporal dementia, a neurodegenerative disease characterized by difficulties with memory often preceded by significant behavioral changes, has been reported to range from two in 100,000 to 31 in 100,000 [1]. A rare neurodegenerative disorder, it can have a detrimental impact on patients and their caregivers given the significant associated early behavioral abnormalities that can impede activities of daily living, decrease the quality of life of the patient, and increase caregiver burden [2, 3]. The first syndrome, characterized by prominent abnormal behavioral symptoms, is called the behavioral variant of frontotemporal dementia (bvFTD). Given the extensive differential diagnosis for bvFTD, its rarity, and its detrimental impact on the quality of life, it is crucial to identify the disease early on in its course to offer appropriate counseling, monitoring, and prognostication to patients, families, and caregivers. The behavioral variant of frontotemporal dementia (bvFTD), characterized by early behavioral abnormalities and late memory impairment, is a neurodegenerative disorder with a detrimental impact on patients and their caregivers. We sought to evaluate the diagnostic accuracy of brief socio-cognitive tests that may differentiate bvFTD and AD patients with low educational levels

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