Prodromal and Early bvFTD: Evaluating Clinical Features and Current Biomarkers

Abstract

Despite the current diagnostic criteria, early diagnostics of behavioral variant of frontotemporal dementia (bvFTD) has remained challenging. Patients with bvFTD often present with misleading psychiatric phenotype, and, on the other hand, impairment in memory functions have increasingly been reported. However, impaired episodic memory is currently considered as an exclusion criterion for bvFTD. Single biofluid-based or imaging biomarkers do not currently provide sufficient sensitivity or specificity for early bvFTD diagnosis at single-subject level, although studies have suggested improved accuracy with different biomarker combinations. In this mini review, we evaluate the core clinical features of early bvFTD and summarize the most potential imaging and fluid biomarkers for bvFTD diagnostics.

Differential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes—Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism

“…Alzheimer’s disease should be ruled out using CSF biomarkers or amyloid PET (as in the CBS-AD described above) ( Seeley, 2019 ). Low level of neurofilament light chains (Nfl) in cerebrospinal fluid excludes neurodegeneration with a high likelihood; therefore, it may probably be useful in differentiating PSP-F with psychiatric disorders ( Vijverberg et al, 2017 ; Katisko et al, 2019 ). Genetic causes of FTLD, including MAPT, PGRN and C9orf72 genes, should be excluded; C9orf72 is particularly often related to behavioural variant of frontotemporal dementia, with the presence of motor neuron disease symptoms, which are absent in PSP-F ( Rademakers et al, 2012 ; Pan and Chen, 2013 ; Seeley, 2019 ).…”

Demencia frontotemporal – variante conductual como diagnóstico diferencial de trastornos psiquiátricos primarios

“…También hay perspectivas en relación a proteínas de neurofilamentos en el LCR pues estos son componentes del esqueleto neuronal y su presencia se incrementa en procesos neurodegenerativos. Específicamente, las cadenas ligeras de neurofilamentos parecen un promisorio marcador biológico para discriminar DFTvc de trastornos psiquiátricos primarios, no solo en LCR sino también en plasma sanguíneo (104,105).…”

“…Es importante considerar la consejería genética pre-y postest, por las implicancias que podría tener en los pacientes el tener conocimiento de un resultado positivo. No existen aún en nuestro medio la disponibilidad de estas pruebas (24,28,104). Rev Neuropsiquiatr.…”

Combining Neuropsychological Assessment with Neuroimaging to Distinguish Early-Stage Alzheimer’s Disease from Frontotemporal Lobar Degeneration in Non-Western Tonal Native Language-Speaking Individuals Living in Taiwan: A Case Series

“…As a result, for individuals younger than 65 years of age, the many overlapping signs and symptoms of MCI/AD and FTLD, such as memory impairment, language problems, and changes in individuals’ behavior, can make accurate differential diagnosis between MCI/AD, bvFTD, and PPA at their initial clinical presentations challenging. Consequently, such diagnoses are important for both research and clinical practice [ 17 , 18 , 19 ].…”