Frontotemporal Degeneration Dementia
Draws More Attention, Research Funding
This early onset dementia strikes 10
years earlier than most; almost $6 million in research being funding by
three agencies of the National Institutes of Health
Oct. 24, 2014 - Approximately
50,000 Americans live with frontotemporal degeneration, or FTD, which
strikes people most often in their 50s or 60s, and causes severe
behavioral changes and problems with language and cognition. The
National Institutes of Health will award three large, five-year projects
targeting this specific form of dementia, known as frontotemporal
because of the areas of the brain that are affected.
As the disease progresses,
individuals have difficulty planning activities, interacting with others
and caring for themselves. (Read more about the
disease below news story.)
The projects, funded by the NIH’s
National Institute of Neurological Disorders and Stroke (NINDS),
National Institute on Aging (NIA) and the National Center for Advancing
Translational Sciences (NCATS), announced today total more than $5.9
million for 2014.
“The grants cover a wide spectrum
of FTD research, from fundamental discoveries of the genetics behind
this disorder to testing potential therapies in patients. We hope that
these projects will provide answers and new avenues of treatment for
this devastating condition,” said Walter Koroshetz, M.D., acting
director of NINDS.
“The projects aim to advance our understanding of
frontotemporal degeneration by improving diagnosis, identifying
preventive strategies and providing new insights into the genetics
underlying this complex disorder,” said Margaret Sutherland, Ph.D.,
program director at NINDS.
“This opportunity to identify the biomarkers that
may signal the onset and progression of FTD in symptom-free volunteers
with the familial form of the disease may one day lead to effective
interventions,” said John Hsiao, M.D., a program director at NIA.
“These multicenter, multi-disciplinary projects
will enable scientists to combine their areas of expertise to design
novel approaches for FTD research, with the ultimate goal of providing
treatments to more patients more efficiently,” said Pamela McInnes,
D.D.S., M.Sc.(Dent.), deputy director of NCATS.
The grants are:
● Longitudinal Evaluation of Familial
Frontotemporal Dementia Subjects
Principal Investigator: Bradley F. Boeve, M.D.; Mayo Clinic, Rochester,
Dr. Boeve and his colleagues will enroll 300
members of families with familial frontotemporal lobar degeneration with
changes in genes associated with the disorder. Study participants will
undergo annual brain scans, blood and cerebrospinal fluid analysis as
well as behavioral and cognitive tests. Dr. Boeve’s team will use that
data to identify biomarkers, which will help determine the effectiveness
of potential therapies.
● The Frontotemporal Lobar Degeneration
Clinical Research Consortium
Principal Investigator: Adam L. Boxer, M.D., Ph.D.; University of
California, San Francisco;
Dr. Boxer and his colleagues will work with
advocacy groups to establish a clinical research consortium to support
development of FTD therapies. The groups involved in this project
include: the Association for Frontotemporal Degeneration, Alzheimer’s
Drug Discovery, Bluefield Project to cure FTD, CBD Solutions, Cure PSP
and the Tau Consortium. The goals of the research network will be to
improve clinical trial design and bring together a variety of techniques
and methods to generate new types of treatments for FTD. The
Frontotemporal Lobar Degeneration Clinical Research Consortium is part
of the NIH NCATS Rare Diseases Clinical Research Network.
● Pathobiology of Neurodegeneration in C9ORF72
Principal Investigator: Leonard Petrucelli, Ph.D.;
Mayo Clinic, Jacksonville, Florida;
A specific change, or mutation, in the C9ORF72 gene
is the most common cause of FTD and amyotrophic lateral sclerosis (ALS),
a neurodegenerative disease that affects the muscles. Using cells in a
dish, animal models and human tissue, Dr. Petrucelli and his team will
comprehensively investigate the mechanisms underlying C9ORF72-related
neurodegeneration and develop therapies to overcome the effects of the
NINDS is the nation’s leading funder of research on the brain
and nervous system. The mission of NINDS is to seek fundamental
knowledge about the brain and nervous system and to use that knowledge
to reduce the burden of neurological disease.
The National Institute on Aging leads the federal
government effort conducting and supporting research on aging and the
health and well-being of older people. It provides information on
age-related cognitive change and neurodegenerative disease specifically
at its Alzheimer’s Disease Education and Referral (ADEAR) Center at
NCATS focuses on what is common among diseases and
the translational science process. The goal is to get more treatments to
more patients more efficiently by developing new approaches,
technologies, resources and models; demonstratingtheir usefulness; and disseminating the data,
analysis and methodologies to the community. For more information, visit
About the National
Institutes of Health (NIH): NIH, the nation's
medical research agency, includes 27 Institutes and Centers and is a
component of the U.S. Department of Health and Human Services. NIH is
the primary federal agency conducting and supporting basic, clinical,
and translational medical research, and is investigating the causes,
treatments, and cures for both common and rare diseases. For more
information about NIH and its programs, visit
The Association for Frontotemporal Degeneration
Frontotemporal Degeneration is Second Most Common
Cause of Early Onset Dementia
Frontotemporal degeneration is not as rare as once
thought; it is considered to be the second most common cause of early
onset dementia. However, because of the wide range of symptoms and their
gradual onset, FTD is often initially misdiagnosed as a psychiatric
problem, Alzheimer’s disease, Parkinson’s disease or vascular dementia.
FTD vs. Alzheimer’s Disease
Both frontotemporal degeneration (FTD) and
Alzheimer’s disease (AD) are characterized by atrophy of the brain, and
a gradual, progressive loss of brain function. However, several
important distinctions can help to differentiate between the two:
● FTD is primarily a disease of behavior and
language dysfunction, while the hallmark of Alzheimer’s disease is loss
● FTD often begins earlier than AD with an
average age of onset in the 50s and 60s, a full 10 years before the
average Alzheimer’s patient is diagnosed.
● FTD patients exhibit behavioral and
personality changes (lack of concern for social norms or other people,
lack of insight into their own behaviors), but retain cardinal features
of memory (keeping track of day-to-day events, orientation to space and
● AD patients display increasing memory
deficits, but typically retain socially appropriate behavior.
● Some FTD patients may have only language
dysfunction (this is seen in the two types of progressive aphasia:
semantic dementia and progressive non-fluent aphasia). And the pattern
of language loss may be specific, such as an inability to name a
familiar, everyday object.
● The language decline seen in AD patients
involves a milder problem with recalling names and words.
● FTD patients are more likely to display early
motor abnormalities, such as difficulty walking, rigidity or tremor
(similar to Parkinson disease), or muscle atrophy and weakness.
Dementia is the loss of cognitive functioning—thinking,
remembering, and reasoning—and behavioral abilities to such an extent
that it interferes with a person’s daily life and activities. Dementia
ranges in severity from the mildest stage, when it is just beginning to
affect a person’s functioning, to the most severe stage, when the person
must depend completely on others for basic activities of daily living.
Many conditions and diseases cause dementia. The
most common cause of dementia in older people is Alzheimer’s disease.
Other causes include different kinds of brain changes that lead to
vascular dementia, Lewy body dementia, and frontotemporal disorders.
In addition, some people have mixed dementia—a
combination of two or more disorders, at least one of which is dementia.
A number of combinations are possible. For example, some people have
Alzheimer’s disease and vascular dementia at the same time.
Other causes of dementia include Huntington’s
disease, Creutzfeldt-Jakob disease, head injury, and HIV. In addition,
some conditions that cause dementia, such as normal pressure
hydrocephalus, thyroid problems, and vitamin B deficiency, can be
reversed with appropriate treatment. For an overview of all types of
dementia, see the booklet
The Dementias: Hope Through Research. For more information
about these conditions, visit the
National Institute of Neurological Disorders and Stroke.
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