End Stage Clinical Features and Cause of Death of Behavioral Variant Frontotemporal Dementia and Young-Onset Alzheimer’s Disease

Abstract

Background: Limited literature exists regarding the clinical features of end stage behavioral variant frontotemporal dementia (bvFTD). This data is indispensable to inform and prepare family members as well as professional caregivers for the expected disease course and to anticipate with drug-based and non-pharmacological treatment strategies. Objective: The aim of the present study was to describe end stage bvFTD in a broad explorative manner and to subsequently evaluate similarities and dissimilarities with the end stage of the most prevalent form of young-onset dementia, Alzheimer’s disease (yoAD). Methods: We analyzed medical files on patients, using a mixed model of qualitative and quantitative approaches. Included were previously deceased patients with probable bvFTD and probable yoAD. End stage was defined as the last 6 months prior to death. Primary outcome measures comprised somatic, neurological, and psychiatric symptoms and the secondary outcome measure was cause of death. Results: Out of 89 patients, a total of 30 patients were included (bvFTD; n = 12, yoAD; n = 18). Overall, the end stages of bvFTD and yoAD were characterized by a broad spectrum of clinical symptoms including severe autonomic dysfunction and an increased muscle tone. Patients with bvFTD displayed more mutism compared with yoAD while compulsiveness was only present in bvFTD. Conclusion: Our study describes the full clinical spectrum of end stage bvFTD and yoAD. In this study, symptoms extend far beyond the initial behavioral and cognitive features. By taking both somatic, psychiatric, and neurological features into account, family members and professional caregivers may anticipate (non) pharmacological treatment.

Altered visual and haptic verticality perception in posterior cortical atrophy and Alzheimer's disease

“…2018; Van Engelen et al . 2020) consistent with disorientation in the vertical plane. In PCA relative to tAD, neuroanatomical differences comprise reduced grey matter volume in posterior parietal and occipito‐temporal regions and relative preservation of medial temporal, hippocampal and entorhinal regions (Whitwell et al .…”

“…while sitting, commenting 'I felt like I was about to fall off the edge of the world' when walking, and noting sensations of 'floating along the ceiling' after descending stairs (Crutch et al 2018). Postural disturbances such as leaning to one side during walking or standing have been observed in a number of PCA and late stage young-onset AD patients (Crutch et al 2018;Van Engelen et al 2020) consistent with disorientation in the vertical plane. In PCA relative to tAD, neuroanatomical differences comprise reduced grey matter volume in posterior parietal and occipito-temporal regions and relative preservation of medial temporal, hippocampal and entorhinal regions (Whitwell et al 2007;Lehmann et al 2011;Firth et al 2019).…”

“…Disorientation in the vertical plane can manifest as compromised postural or locomotor functions as well as perceptual deficits. Therefore, parietal vulnerability in Alzheimer's disease (AD) may contribute to the perceptual disturbances, disorders of gait, balance and posture, increased falls incidence, and spatial disorientation that have been reported (Allan et al 2009;Golden et al 2015;Coughlan et al 2018;Hardy et al 2020;Van Engelen et al 2020).…”

Prediction of 3-Year Survival in Patients with Cognitive Impairment Based on Demographics, Neuropsychological Data, and Comorbidities: A Prospective Cohort Study

“…However, in our cohort, no differences were detected between surviving and deceased patients regarding these symptoms. A possible reason is that we did not screen the disease stage of AD patients, who accounted for 52.8 percent of our cohort; however, the stage of AD can have a significant impact on the incidence of NPS and patients’ survival [ 37 , 38 ]. From this point of view, it can also be said that our model is more suitable for use in primary health care facilities in cases where the stage of cognitive impairment remains unclear.…”

Synaptic biomarkers in the cerebrospinal fluid associate differentially with classical neuronal biomarkers in patients with Alzheimer’s disease and frontotemporal dementia

“…The samples were screened for their levels of CSF Aβ42, Tau, and PTau to support their AD diagnosis (Tau/Aβ42 > 0.46) [21]. The FTD diagnoses were based on consensus guidelines for FTD criteria along with psychiatric evaluations as a part of the Amsterdam Dementia Cohort [22,23]. All FTD patients in this cohort were classified as "probable behavioral" variant.…”