# Diagnostic and prognostic certainty in behavioral variant frontotemporal dementia

> **NIH NIH R01** · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · 2021 · $795,714

## Abstract

ABSTRACT
Frontotemporal dementia (FTD) is a common neurodegenerative cause of early age-of-onset dementia. The
behavioral variant of FTD (bvFTD) results in profound changes in personality, as well as social and emotional
functioning. Diagnostic uncertainty remains a common concern in bvFTD in spite of improved diagnostic
criteria that focus on particular behavioral and cognitive features accompanied by characteristic neuroimaging
patterns. Accurate diagnoses at the first visit are especially challenging, when cognitive impairment can be
mild and functional abilities are more preserved. Other dementia syndromes can mimic features of bvFTD and
there is partial overlap with the symptoms of psychiatric illnesses. With the clarity provided by longitudinal
follow-up clinicians sometimes change bvFTD diagnoses. There are over 15 potential neuropathological
diagnoses that underlie bvFTD. Even when a bvFTD diagnosis is clear it is challenging to predict the specific
molecular subtype causing an individual patient’s symptoms. There is substantial heterogeneity in the clinical
course in bvFTD as well, with some declining rapidly within 2-3 years and others surviving over more than a
decade. Motor features may be present or absent, and their severity in the context of bvFTD can impact the
level of care a patient requires. There are few reliable indicators to prognosticate a patient’s disease course.
The proposed research will study 60 patients with bvFTD longitudinally with neurological examinations,
cognitive testing, and structural and functional neuroimaging, and will retrospectively review the clinical
features of 284 autopsied patients with bvFTD. The central hypothesis of this proposal is that in spite of the
similarities between patients with bvFTD there will be clinical, neuropsychological, neuroimaging, serum
marker, genetic, and gene expression differences that permit improved predictive certainty at the first visit. In
Aim 1 we will use longitudinal assessment of diagnostic stability in order to determine factors that predict
certainty in the bvFTD diagnosis. In Aim 2 we will identify clinical, gene expression, and imaging profiles in a
cohort of autopsied patients with bvFTD that allow accurate prediction of a patient’s pathological diagnosis. In
Aim 3 we will use longitudinal data on patients with bvFTD to determine factors that allow accurate
prognostication of the clinical course. The results of the proposed research will provide guidance to clinicians
who are considering a diagnosis of bvFTD and will improve the diagnostic and prognostic information available
to patients, families, and clinicians, leading to improved clinical care from the time of the first visit. It will also
facilitate enrollment of patients into observational research and molecularly targeted clinical trials.

## Key facts

- **NIH application ID:** 10260561
- **Project number:** 5R01AG062758-02
- **Recipient organization:** UNIVERSITY OF CALIFORNIA, SAN FRANCISCO
- **Principal Investigator:** DAVID C PERRY
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $795,714
- **Award type:** 5
- **Project period:** 2020-08-15 → 2025-04-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10260561

## Citation

> US National Institutes of Health, RePORTER application 10260561, Diagnostic and prognostic certainty in behavioral variant frontotemporal dementia (5R01AG062758-02). Retrieved via AI Analytics 2026-05-24 from https://api.ai-analytics.org/grant/nih/10260561. Licensed CC0.

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