Abstract
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.
Highlights
Frontotemporal dementia (FTD) is a clinical neurodegenerative syndrome characterized by alterations in behavior, executive functions, and language [1–3]
We will present the available evidence divided into 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
We will discuss the relevance of each dimension for the assessment in the FTD diagnosis, describing the instruments generally used along with the available evidence in Latin America and Caribbean (LAC)
Summary
Frontotemporal dementia (FTD) is a clinical neurodegenerative syndrome characterized by alterations in behavior, executive functions, and language [1–3]. There are two main types of FTD: the first is the behavioral FTD variant (bvFTD), characterized by impaired social function, behavior, and personality; and the second are the language variants, namely, semantic dementia (SD), non-fluent or agrammatical aphasia (nfv-PPA), and logopenic aphasia (lv-PPA), which are characterized by progressive deficits in language skills [2, 4, 6]. There is a current controversy surrounding lv-PPA, regarding whether to maintain its inclusion as an FTD variant, given that the neuropathological studies show a stronger association with Alzheimer’s Disease (AD) pathologies [7, 8]. Some current criteria maintain it as an FTD syndrome variant [6]. Other types of FTD-related disorders present with motor symptoms and/or parkinsonism. The main disorders associated with motor difficulties are FTD with motor neuron disease (FTDMND) and FTD with atypical parkinsonism, i.e., progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) [2, 9, 10]
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