Focus on Frontotemporal Dementia (FTD)

Focus on Frontotemporal Dementia (FTD)

3D illustration. Concept of artificial neuron.

NINDS Program Description

Frontotemporal dementia (FTD), also refered to as Frontotemporal lobar degeneration (FTLD), is a clinically and pathologically heterogeneous group of non-Alzheimer dementias characterized collectively by progressive atrophy involving the frontal or temporal lobes, or both. In the past three decades the complexity of these diseases and their unique status as examples of selective brain degeneration has been fully appreciated. Although onset is typically in the sixth decade of life, it may begin as early as the third or as late as the ninth decade, and the prevalence of FTD in older age groups has almost certainly been underestimated.

The NINDS is interested in Clinical Trial readiness applications for Frontotemporal Degeneration (FTD).  To learn more please read NOT-NS-18-082.

The NINDS supports a number of projects covering all aspects of FTD research.  Some of our bigger initiatives are list here:

Advancing Research & Treatment for Frontotemporal Lobar Degeneration (ARTFL)

The Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) consortium is a group of academic medical centers in the USA and Canada seeking volunteers to participate in a longitudinal study that will collect clinical and genetic data to learn more about the progression of FTD. The ARTFL project will establish a large cohort of patients with FTLD syndromes including Corticobasal Degeneration Syndrome (CBD or CBS), primary progressive aphasias (PPA) including semantic variant (svPPA) and non-fluent variant (nvPPA), behavioral variant Frontotemporal Dementia (bvFTD), Frontotemporal Dementia with Amyotrophic Lateral Sclerosis (FTD-ALS), and Progressive Supranuclear Palsy (PSP). Healthy family members of patients with genetic causes of FTLD are also enrolled.

Tau Center without Walls 

The purpose of this Funding Opportunity Announcement (FOA) is to support innovative interdisciplinary, multi-institute research that will lead to the identification and validation of molecular mechanisms relevant to human biology that contribute to tau toxicity associated with Frontotemporal Degeneration (FTD). It is anticipated that this research will also contribute to tool development that can be applied to target validation in FTD clinical trials. The Tau CWOWs aim to identifying genes and pathways that modulate tau toxicity in FTD and link tau proteostasis with neuronal activity in frontotemporal dementia.

FTD Sequencing Consortium

The purpose of this consortium, developed in resoponce to this FOA, is to support the genetic discovery, replication and validation of disease causing mutations, risk variants and genetic modifiers which contribute to the pathophysiology, proteinopathies and clinical heterogeneity representative of the neurological syndromes that are classified under the broad spectrum of Frontotemporal Degeneration (FTD). The FTD Sequencing Consortium FOA supports whole genome sequencing, replication and functional variant validation studies of FTD case/control and family-based cohorts identified through clinical diagnosis or autopsy confirmation.

  • FTD is the most common form of dementia for people under age 60
  • Onset often occurs in a person’s 50s or 60s
  • FTD shares genetic causes and often co-occurs with ALS, a motor neuron degenerative disease
  • No known treatments prevent or stop FTD

NIH Estimates of Funding for Various Research, Condition, and Disease Categories

Research/Disease Areas* FY 2016
(Actual)
FY 2017
(Actual)
FY 2018
(Actual)
FY 2019
Estimated
Frontotemporal Dementia (FTD) $65 $91 $94 $98

*Dollars in millions and rounded

Proceedings & Outcomes

FTD and the National Plan to Address Alzheimer’s Disease

On January 4, 2011, the National Alzheimer's Project Act (NAPA) was signed into law. The Act defines "Alzheimer's" as Alzheimer's disease and related dementias (AD/ADRD), which includes frontotemporal degeneration (FTD), Lewy body dementia (LBD), vascular contributions to cognitive impairment/dementia (VCID), and mixed etiology dementias (MED).

ADRD 2016 Summit Overview for FTD

The 2016 Alzheimer’s Disease-Related Dementias (ADRD) Summit was hosted by the National Institute of Neurological Disorders and Stroke (NINDS) in collaboration with the National Institute on Aging (NIA). The 2016 ADRD Summit was mandated by the National Plan to address Alzheimer’s Disease as a follow-up to the 2013 ADRD Summit. The Summit set research priorities for FTD research, including:

Basic Science: Pathogenesis and Toxicity

  1. Clarify the mechanism of tau pathogenesis and associated neurodegeneration
  2. Determine the molecular basis for C9ORF72 expansion and GNR mutation-related neurodegeneration
  3. Determine the mechanisms of RNA Processing pathogenesis and toxicity
  4. Develop better FTLD in vivo and cell-based model systems

Clinical Science

  1. Expand efforts to genotype patients with FTD and identify new genes and their functional relationship to FTLD pathogenesis
  2. Develop FTD biomarkers for diagnosis and disease progression
  3. Create an international FTD clinical trial network
  4. Understand phenotypic heterogeneity and natural history

Resources and Tools

The Registry is an online database to collect information from those affected by all types of Frontotemporal Degeneration.  Persons diagnosed, (current/former) caregivers, family, and friends can join.