Long-Term Care of Patients With
Jennifer J. Merrilees, RN, MS, and Bruce L. Miller, MD
Frontotemporal dementia (FTD) results from the progres-
onstrate alterations in dress and hygiene. Hyperorality and
sive dysfunction of the frontal and/or temporal lobes of the
ritualistic behaviors around eating can occur. Socially inap-
brain. It is a presenile dementia with a mean age of onset of
propriate behaviors, including theft, offensive speech, assault,
52.8 to 56 years.1 FTD affects men and women equally. The
and public urination or masturbation, occurred in almost half
course of FTD has a mean duration of 8 years.2 There are no
of patients with FTD with atrophy of the frontal and temporal
known risk factors for FTD, although approximately 40% of
patients have a family history of dementia.3 With this disor-
Language dysfunction dominates left-sided involvement,
der, there are marked changes in personality and behavior. As
although behavioral symptoms do emerge. Progressive non-
a result of the profound impact on patients’ function, most
fluent aphasia (PNFA) occurs in patients with left frontal
patients with FTD will eventually require placement in a
involvement and is characterized by diminished ability to
express language. Patients experience difficulty with speech
Consensus criteria from 1998 outlines 3 clinical presenta-
initiation, pronunciation, and word finding. Associated clin-
tions: frontotemporal dementia (FTD), semantic dementia
ical features include depression, social withdrawal, and pres-
(SD), and progressive nonfluent aphasia (PNFA).4 The term
ervation of social graces.1 Behavioral symptoms do not usually
frontotemporal lobar degeneration (FTLD) is often used in
appear until later in the disease.6 Semantic dementia is caused
reference to the 3 syndromes. The clinical features of the 3
by involvement of the left temporal lobe; patients demon-
subtypes are determined by the region of the brain affected.
strate progressive loss of knowledge about words and objects.
Key features of patients with right frontal and bifrontal lobe
Speech might be fluent, but is characterized by semantic
involvement are behavioral symptoms and personality
paraphrasias and substitutive phrases. New artistic talents and
changes. Patients can exhibit disinhibition and impulsivity.
skills have been observed in some patients.7
Stereotypic behaviors such as hoarding, impulse buying, and
Patients with FTD typically present with 1 of the 3 differ-
compulsions are also common. Apathy and lack of drive also
ent clinical syndromes. Onset of symptoms is insidious and
occur. Personality changes include emotional blunting and
slowly progressive. As the disease progresses, patients can
blunting of empathy; it is common to confuse these symptoms
exhibit features of the other clinical syndromes.
with depression, midlife crisis, and marital problems. Cogni-
Motor abnormalities are common in FTD as a result of the
tively, patients with FTD have difficulties with organization,
association with 3 other neurodegenerative diseases: cortico-
planning, and reasoning. They tend to have limited insight
basal ganglionic degeneration (CBD), progressive supranu-
about their behavioral and personality alterations.
clear palsy (PSP), and motor neuron disease (MND). Con-
Remarkably, sometimes the changes can be viewed as pos-
versely, patients with PSP and CBD often develop behavioral
itive. Early disinhibition in one patient resulted in increased
symptoms. Apathy and depression have been observed in
assertiveness and self-confidence. She demonstrated a new
patients with PSP. Patients with CBD can exhibit personality
interest in the stock market and made successful transactions
changes, disinhibition, and irritability.
over a period of months. She exhibited a new flare in her style
There is currently no treatment for slowing or reversing
and dress. Her family found her to be more affectionate. As
FTD. Acetylcholinesterase inhibitors, used to slow progres-
her disease progressed, she exhibited poor judgment, socially
sion in Alzheimer’s disease, are avoided in FTD for several
inappropriate behaviors, and profound apathy requiring 24-hour supervision and eventual long-term placement.
reasons. In clinical experience, they tend to offer no improve-
Right temporal patients also present with behavioral symp-
ment in cognition and can promote agitation and irritability.
toms that can appear strange and psychiatric in nature. They
Management of FTD is aimed at reduction of problematic
can exhibit disinhibition, irritability, and loss of empathy.
behavioral symptoms, health maintenance and prevention of
Their thinking becomes rigid, stereotyped, and they can dem-
future problems, and adaptation to changes in functionalabilities. With disease progression, patients become morefunctionally dependent. They demonstrate a reduction inspeech, eventually becoming mute. Motor abilities decline
Department of Neurology, University of California at San Francisco, San Fran-
and swallowing difficulties can occur. Some behavioral symp-
toms diminish and others surface. Death typically results from
Address correspondence to: Jennifer Merrilees, RN, MS, UCSF Memory andAging Center, 350 Parnassus Avenue, Suite 706, San Francisco, CA 94143-1207.
Patients with FTD and their families face a myriad of
Copyright 2003 American Medical Directors Association
overarching concerns. FTD causes profound disruption for
families as they deal with behavioral, personality, and func-
Medications for Neurobehavioral Symptoms of FTD
tional alterations. One family member described herself as
aimed at reduction of risk can be used.9 Swallowing problems
being “crushed by the demands” she faced as the primary
can develop, requiring referral for swallowing assessment. Di-
etary changes such as substituting thick for thin liquids areoften made.
MANAGEMENT OF BEHAVIORAL SYMPTOMS
Early planning for incapacity is important in making deci-
Methods to ameliorate behavioral symptoms ideally incor-
sions about future healthcare needs and protection of assets.
porate strategies that focus on environmental and behavioral
Clarification of wishes around topics such as resuscitation, life
manipulation, as well as appropriate use of medication. Al-
support, and autopsy are ideally made when the patient is able
though there have been some studies evaluating efficacy of
medications for behavioral symptoms in FTD, there have
been no systematic evaluations of appropriate environmentaland behavioral interventions. It is tempting yet speculative to
The patterns of change in the patient’s functional abilities
use interventions for Alzheimer’s disease (AD). Yet, there are
are unique and variable in FTD. Deficits in organizational and
important cognitive differences between AD and FTD. Gen-
planning abilities, poor judgment, and apathy affect job per-
erally, memory is preserved until later in the disease. This
formance, driving, chores, and hobbies. Counseling regarding
memory preservation can be viewed as an asset and should
early retirement, disability, and driving is often necessary.
influence future research into behavioral interventions. The
Language difficulties affect job performance as well, although
fields of psychiatry and neurorehabilitation hold promise as
in the absence of executive dysfunction, many of our language
models for research into effective interventions for behavioral
patients continue to work successfully. Patients elect to stop
working as their language and speech deficits advance. Ne-
Serotonin deficits are thought to be a cause for behavioral
glect for personal care and safety risks occur in patients with
symptoms in FTD; therefore, selective serotonin reuptake
disabling compulsive behaviors. An obsession with rock col-
inhibitors (SSRIs) are used for many of the behaviors. SSRIs
lecting made one patient unable to care for her young child.
have been shown to be helpful in treating impulsivity, com-
Motor complications cause functional deficits in self-care and
pulsions, depression, and carbohydrate craving.8 SSRIs can be
increased risk for injury, especially when behavioral symptoms
helpful with aggression, delusions, and anxiety. Atypical an-
such as impulsivity and stubbornness are present.
tipsychotics have been used for aggressive behaviors. Valproic
DETERMINING THE NEED FOR CARE
acid has use for agitation and aggression. Antipsychotics suchas haloperidol are not used in FTD as a result of undesirable
Each patient has a unique expression of the disease. Clin-
side effects. Benzodiazepines are not recommended because
ical experience shows that factors such as home setting, rural
of their negative cognitive effects; however, they can be
versus urban setting, available resources, quality of family
useful as short-term treatment, in selected patients, to
caregiving, and other demands on the family unit all factor
complete a necessary test such as magnetic resonance im-
into the planning of long-term care needs. Like with all forms
of dementia, it is the unique interplay of behavioral symp-toms, self-care abilities and needs that often determine
HEALTH MAINTENANCE AND PREVENTATIVE CARE
The decline in speech and language ability creates commu-
nication challenges for patients with FTD and their families.
THE TRANSITION TO A NEW LEVEL OF CARE
Clinical experience suggests that speech therapy offers bene-
It is vital to match the needs of the patient with resources
fits for some patients, yet it is unclear the role that therapy
of the facility. Families are important sources of information
plays in this disease. In particular, the patients with nonfluent
for insight about the patient’s cognitive, behavioral, and phys-
progressive aphasia tend to benefit.
ical attributes. Patients with a history of wandering and elope-
Exercise is recommended for all patients. As motor symp-
ment will need careful attention to safety needs. Strategies
toms emerge, patients will be at risk for complications of
currently exist for such patients, including warning alarms on
immobility, falls, and fractures. Rehabilitation principles
exits, secured grounds, or one-on-one companionship/super-
vision. Restraint use is associated with profound detrimental
care facility. Caring for this group of patients is nearly always
effects, and their use is not encouraged.10,11 Speech and
extremely challenging, yet often rewarding. New therapies for
language deficits result in alternative forms of communica-
FTD are likely to emerge in the coming years, and with these
tion. Strict attention to body language and behavioral cues
therapies, there will be new, exciting clinical challenges.
are necessary. It is important to evaluate the patient’s activitylevel and needs to ensure they can be met by the facility.
No standard way of structuring the transition to a different
1. Miller BL, Boone K, Mishkin F, et al. Clinical and neuropsychological
level of care has been studied in this population of patients.
features of frontotemporal dementia. In: Kertesz A, Munoz D, eds. Pick’s
Some families have been encouraged to stay away for a period
Disease and Pick Complex. New York: Wiley-Liss, 1998:23–33.
2. Snowden JS, Neary D, Mann DMA. Fronto-temporal dementia. In:
of time to allow for adaptation and acceptance; others spend
Snowden JS, Neary D, Mann DMA, eds. Fronto-Temporal Lobar De-
a lot of time at the new facility. It might be helpful to conduct
generation. New York: Churchill Livingstone, 1996:1– 41.
shorter respite visits as a way of introducing the patient to the
3. Chow T, Miller BL, Hayashi V, et al. Familial studies of frontotemporal
new facility. It has been fairly typical for patients to express
dementia. Arch Neurol 1999;56:817– 822.
anger toward the family caregiver for the move to long-term
4. Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degen-
eration: A consensus on clinical diagnostic criteria. Neurology 1998;51:
The transition to a long-term care facility is marked by
5. Miller BL, Darby AL, Swartz JR, et al. Dietary changes, compulsions and
disruption of a familiar routine, new names and faces, and
sexual behavior in frontotemporal degeneration. Dementia 1995;6:195–
unfamiliar residents. Catastrophic reactions characterized by
increasing disorganization, irritability, and agitation occur
6. Rosen HJ, Lengenfelder J, Miller BL. Frontotemporal dementia. Neurol
when the patient becomes overwhelmed and overloaded by
7. Miller BL, Cummings J, Mishkin F, et al. Emergence of artistic talent in
demands placed by the environment. Every attempt should be
frontotemporal dementia. Neurology 1998;51:978 –982.
made to decrease the stress for the patient and find acceptable
8. Swartz JR, Miller BL, Lesser IM, et al. Frontotemporal dementia: treat-
methods for stress reduction. In a few rare cases, a temporary
ment response to serotonin selective reuptake inhibitors. J Clin Psychi-
move to a secure unit for medication management has been
9. Robinson KM. Rehabilitation applications in caring for patients with
Pick’s disease and frontotemporal dementia. Neurology 56(11 Suppl
In summary, patients with FTD are characterized by pro-
found changes in their behavior, language, and personality.
10. Talerico KA, Evans LK. Responding to safety issues in frontotemporal
dementias. Neurology 2001;56:S52–S55.
The course of FTD is heterogeneous and slowly progressive.
11. Castle NG, Mor V. Physical restraints in nursing homes: A review of the
Patients require assistance and supervision for a myriad of
literature since the Nursing Home Reform Act of 1987. Med Care Res
reasons, and most eventually need placement in a long-term
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