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Long-Term Care of Patients With
Frontotemporal Dementia
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 DOI: 10.1097/01.JAM.0000095366.91533.22
families as they deal with behavioral, personality, and func- Medications for Neurobehavioral Symptoms of FTD Drug Category
Suggested Use
Medication Name
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 FUNCTIONAL CHANGES
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.
REFERENCES
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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