Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

Souza, Leonardo Cruz de; Hosogi, Mirna Lie; Machado, Thais Helena; Carthery-Goulart, Maria Teresa; Yassuda, Mônica Sanches; Smid, Jerusa; Barbosa, Breno José Alencar Pires; Schilling, Lucas Porcello; Balthazar, Marcio Luiz Figueredo; Frota, Norberto Anízio Ferreira; Vale, Francisco Assis Carvalho; Caramelli, Paulo; Bertolucci, Paulo Henrique Ferreira; Chaves, Márcia Lorena Fagundes; Brucki, Sonia Maria Dozzi; Nitrini, Ricardo; Bahia, Valéria Santoro; Takada, Leonel Tadao

doi:10.1590/1980-5764-DN-2022-S103EN

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Dementia & Neuropsychologia

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Sumário

Consensus • Dement. neuropsychol. 16 (3 suppl 1) • Sept 2022 • https://doi.org/10.1590/1980-5764-DN-2022-S103EN copy

Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

“Frontotemporal dementia” (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.

Keywords:
Frontotemporal Dementia; Aphasia, Primary Progressive

Frontotemporal dementia: behavioral variant
Progressive deterioration in behavior and/or cognition, evidenced by observation or clinical history (requires an informant).
Possible diagnosis
Must meet at least 3 of the 6 criteria listed below:
(I) behavioral disinhibition (socially inappropriate behavior; impulsiveness; loss of social rules or decorum);
(II) apathy or inertia;
(III) loss of empathy/sympathy (decreased affective resonance to the needs and feelings of others; diminished social interest, reduced interpersonal “heat”);
(IV) perseverative, stereotyped, or compulsive/ritualistic behaviors;
(V) hyperorality and dietary changes (alteration of food preference, increased consumption of alcohol or cigarettes, oral exploration of objects);
(VI) neuropsychological profile with executive deficits and relative preservation of episodic memory and visuospatial functions.
Probable diagnosis
Must meet criteria for possible FTD and have:
(I) Significant functional decline, demonstrated in specific inventories; and
(II) Evidence of typical FTD alterations on neuroimaging examination (atrophy or hypometabolism or hypoperfusion in frontotemporal regions).
Definite diagnosis
Must meet criteria for possible or probable FTD and have:
(I) Pathological confirmation of frontotemporal lobe degeneration; or
(II) Evidence of causative genetic mutation.

Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revistadementia@abneuro.org.br | demneuropsy@uol.com.br

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[1] Correspondence: Leonardo Cruz de Souza; Email: leocruzsouza@ufmg.br.