Disease trajectories in behavioural variant frontotemporal dementia, primary psychiatric and other neurodegenerative disorders presenting with behavioural change

Abstract

Behavioural variant frontotemporal dementia (bvFTD) is characterized by behavioural and social cognitive disturbances, while various psychiatric and neurodegenerative disorders may have similar clinical symptoms. Since neurodegenerative disorders are eventually progressive, whereas primary psychiatric disorders are not, this study aimed to investigate whether the change in clinical symptoms over time differed between groups and which biomarkers predicted rate of decline. Disease trajectories (median follow-up = 3 years) of frontal and stereotyped behaviour, general and frontal cognitive functioning, and social cognition were examined in bvFTD (n = 34), other neurodegenerative (n = 28) and primary psychiatric disorders (n = 43), all presenting with late-onset frontal lobe syndrome (45-75 years), using linear mixed models. To gain more insight in underlying pathological processes driving disease progression, we studied the association of baseline cerebrospinal fluid (CSF) (neurofilament light (NfL) and YKL-40 levels, phosphotau to total tau ratio) and neuroimaging markers with disease trajectories. Frontal behavioural symptoms (e.g., disinhibition, apathy) worsened over time in bvFTD, whereas they improved in psychiatric disorders and remained stable in other neurodegenerative disorders. General and frontal cognitive decline was observed in bvFTD and other neurodegenerative disorders, but not in psychiatric disorders. None of the groups showed change in stereotypy and social cognition. For all diagnostic groups, higher CSF NfL levels were associated with faster frontal cognitive decline. A modest association was observed between caudate volume and stereotyped behaviour. Tracking frontal behavioural symptoms and cognition has potential to distinguish bvFTD from other disorders. CSF NfL levels seem to be associated with decline in frontal cognitive functioning.

Understanding and predicting the longitudinal course of dementia

“…Another study found that patients with a behavioural variant frontotemporal dementia worsened in frontal symptoms such as disinhibition and apathy, whereas these symptoms were rather stable in other neurodegenerative disorders and even improved in primary psychiatric disorders [25]. In contrast, a comparison of Alzheimer's dementia and behavioural variant frontotemporal dementia patients showed strongly overlapping longitudinal trajectories in executive functioning, memory and orientation measures [26].…”

A case of bipolar disorder developing into atypical parkinsonism and presenting with frontotemporal asymmetrical brain degeneration. A TREDEM Registry Case Report

“…In a recent study, the longitudinal trajectories of frontotemporal dementia (FTD) and psychiatric disorders with behavioural symptoms were examined and the results provide strong evidence of a faster worsening of the symptoms in FTD, with a stable profile in psychiatric conditions. This may provide grounds to assume that our patient's clinical trajectory is due to a frontal lobe neurodegeneration, rather than the evolution of a purely psychiatric condition . Another recent study, describing the syndromic overlap between frontotemporal lobar degeneration and several psychiatric disorders, reported a similar case.…”

“…This may provide grounds to assume that our patient's clinical trajectory is due to a frontal lobe neurodegeneration, rather than the evolution of a purely psychiatric condition. 2 Another recent study, describing the syndromic overlap between frontotemporal lobar degeneration and several psychiatric disorders, reported a similar case. Particularly, a patient diagnosed with BD type I at the age of 33, was eventually diagnosed with suspected FTD at the age of 68, presenting with frontal and temporal bilateral atrophy and hypometabolism.…”

Old Age Dementia: Clinical Patterns of Progression. Part 1

“…Второе место после БКЯ в группе деменций с быстрым прогрессированием почти единодушно отводится лобно-височной деменции (ЛВД) и, прежде всего, ее поведенческому варианту [58][59][60][61][62]. Однако исследователи признают, что верификация диагноза ЛВД нередко следует за диагнозом психического заболевания, что искажает представление о скорости прогрессировании болезни [58,63].…”

“…В другом исследовании изучали траекторию заболевания (средний срок катамнеза 3 года) при ЛВД в сравнении с другими заболеваниями [61]. Среди обследованных 34 больных ФТД, 28 -другими нейродегенеративными заболеваниями и 43 -с первичными психическими заболеваниями.…”