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

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.

The role of brain perfusion SPECT in the diagnosis of frontotemporal dementia: A systematic review

“…SPECT should be considered for selected patients with an uncertain diagnosis despite neurological examination, laboratory analyses, cognitive assessment, and structural neuroimaging. It should be noted, however, that SPECT is the preferred nuclear imaging tool only when PET‐FDG is not available, and is recommended only for selected cases of difficult differential diagnosis, in line with latest recommendations from the National Institute for Health and Care Excellence 57 and the Brazilian Academy of Neurology 58 …”

“…It should be noted, however, that SPECT is the preferred nuclear imaging tool only when PET-FDG is not available, and is recommended only for selected cases of difficult differential diagnosis, in line with latest recommendations from the National Institute for Health and Care Excellence 57 and the Brazilian Academy of Neurology. 58 Potential limitations of this systematic review must be considered when interpreting the findings. First, there is marked variability in diagnostic criteria across studies.…”

Current clinical and research practices on frontotemporal dementia in Brazil: a national survey

“…Importantly, the Brazilian Academy of Neurology recently proposed recommendations for the diagnosis of FTD. 24 This initiative may help to standardize diagnostic procedures across the country. It should also be noted that a FTD Brazilian Research Group (http://dgp.cnpq.br/dgp/ espelhogrupo/308304) was created and formally registered at the scientific platform of the Brazilian National Council for scientific and Technological Development (CNPq).…”

Diagnóstico de la variante conductual de demencia frontotemporal basado en estadios clínicos adaptado para países con baja educación: el caso de Perú

“…Ciertos patrones de atrofia pueden sugerir DFT asociada a mutaciones genéticas, por ejemplo, una pronunciada atrofia bitemporal ocurre en pacientes con mutación de la MAPT; una marca marcada atrofia frontotemporal asimétrica es común en pacientes con mutación GRN en quienes también se puede observar extensas lesiones de sustancia blanca (LSB). Conforme progresa la enfermedad, la atrofia se torna más pronunciada en los lóbulos frontotemporales, con una relativa preservación de las regiones posteriores (39) . La IRM también permite la identificación de hiperintensidad en secuencias T2 y FLAIR, que puede sugerir demencia vascular o leucoencefalopatía.…”