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The frontotemporal dementias (FTD) typically present as a specific clinical syndrome, but as these diseases progress, patients usually develop a wider set of cognitive, behavioral, language, and motor symptoms that affect their daily living. Clinicians can face a changing cluster of symptoms whose management is often best served by a multidisciplinary approach. As there are no disease-modifying medications to stop the progression of FTD, the clinician’s familiarity with an array of pharmacologic and non-pharmacologic tools can guide the practical management of the diverse symptoms that will likely be encountered throughout the disease course.
Initial clinic contact and early-stage management
An early and accurate diagnosis is an important starting point for the practical management of FTD. For many reasons, including symptom overlap with psychiatric disorders and a lack of familiarity with FTD in mental health settings, this issue continues to plague the field, with mood disorders and Alzheimer’s disease (AD) being common misdiagnoses of FTD [1]. Frustration with the process of diagnosis is reported as a major complaint and source of stress for FTD caregivers [2]. More detailed and sensitive criteria have recently been proposed for diagnosing behavioral variant FTD (bvFTD) and primary progressive aphasia (PPA) [3, 4]. The familiarity of clinicians with the salient features of the diseases as outlined in these criteria should help detect FTD at an earlier stage.
Early symptoms of bvFTD commonly include apathy, socially inappropriate behavior, loss of empathy, perseverative speech or behaviors, dietary changes, and executive function deficits with relatively preserved memory and visuospatial function [3]. Functionally, difficulty with complex tasks such as managing correspondences and planning finances characterize mild stages of bvFTD [5].
Patients with bvFTD are at particular risk for being misdiagnosed with a psychiatric disorder [6]. Clinicians should be aware of “red flags” that can help with an accurate diagnosis. For example, patients often exhibit the apathy and decreased energy seen in major depressive disorder, but rarely show emotional distress or depressed mood. Patients with repetitive and stereotyped behaviors usually do not experience the anxiety and obsessions that are characteristic of obsessive–compulsive disorder. Other indications of neurodegenerative disease may include the presence of cognitive dysfunction, the absence of previous psychiatric history, and an unusual psychiatric presentation [7].
In the earliest phases of PPA, patients may describe increasingly effortful speech and word-finding difficulty. General cognitive and functional abilities can be largely spared, with preserved performance on formal tests outside of language function [8]. Clinicians sometimes attribute speech deficits to emotional distress or dysfunctional vocal cords. New criteria developed for diagnosing PPA have been shown to be sensitive to early detection of characteristic cortical atrophy patterns [8].
An early diagnosis of FTD is important for several reasons. First, it allows the patient to seek resources that may help manage symptoms. Second, accurate diagnosis provides a framework for families to understand the often dramatic changes in personality and behavior that occur to their loved ones. Understanding behavioral symptoms as a by-product of neurodegenerative disease, and not deliberate intention, is usually helpful for families. FTD caregivers identify thorough explanation of the diagnosis and support by a knowledgable physician as important interventions [9]. Finally, future disease-modifying treatments will most likely be effective early in the disease process. Delays in diagnosis will prevent patients from their best chance at benefit.
At this stage, clinicians should also be prepared to discuss disease course with the patient and caregiver. Given the heterogeneity in clinical presentation and pathology, it has been difficult to establish a single model of disease progression in FTD. Progressive neurodegeneration leads to greater behavioral and cognitive deficits in both bvFTD and PPA, and often to motor deficits as well. These symptoms reduce the capacity of patients to live independently, making them dependent on the caregiver. Ultimately, the accumulation of cognitive and neurologic deficits leads to reduced lifespan.
The time course for these changes is variable. Estimates of survival have been reported to be from 3 to 14 years after diagnosis [10]. There is no evidence that disease severity at diagnosis, age of onset, or demographic characteristics affect survival in FTD. However, patients presenting with motor neuron disease (MND) symptoms, genetic mutations, and co-occurring behavioral and language symptoms decline more rapidly [11, 12]. Greater cognitive abilities and language fluency at diagnosis correlate with slower progression in PPA [13]. New biomarkers may soon also be able to help clinicians with prognosis [14]. Communicating the progressive nature of FTD allows for patients and families to mentally and emotionally prepare for further cognitive and functional declines. Families may also opt to begin handling legal and financial issues while the patient has intact cognitive abilities. At this stage, clinicians can also discuss the possibility of genetic testing and brain autopsy, and the implications that these may have for the family.
The clinician–caregiver relationship is an essential part of managing FTD. Patients often exhibit lack of insight into the nature of their behavioral and cognitive deficits [15]. Speech deficits in PPA can also present a barrier to communication with the physician. Caregivers therefore are usually the best source of information about behavioral and cognitive changes. Clinicians should seek to establish supportive relationships with caregivers at the outset in order to facilitate the management of symptoms. This includes introducing educational and supportive local resources. Referrals to a genetic counselor should be made if genetic testing is under consideration. Referral to nationwide organizations, such as the Association for Frontotemporal Degeneration (AFTD) in the USA, are helpful [16]. Clinicians can also suggest that caregivers maintain a symptom diary. This can be a useful tool to track the evolution of and triggers for symptoms, especially behavioral symptoms, as the disease progresses, and to see the effects of therapeutic interventions as they are introduced [17].
Management at middle stages
The middle stages of FTD are generally characterized by a widening circle of behavioral symptoms, increasing cognitive dysfunction, and greater decline in functional abilities. The FTLD–Clinical Dementia Rating and Frontotemporal Dementia Rating Scales are tools that can help clinicians track disease severity and symptoms [5, 18]. Neuropsychological testing can help clinicians track the rate of cognitive decline.
Patients with PPA generally experience broader language impairments and a deteriorated ability to communicate. Instruments such as the Progressive Aphasia Severity Scale (PASS) can help clinicians track impairment in language domains and functional communication [19]. Patients with non-fluent variant PPA (nfvPPA) will generally have involvement of other areas of cognition besides language, especially executive dysfunction. Patients with semantic variant PPA (svPPA), associated with anterior temporal impairment, will usually present with a behavioral syndrome in addition to their semantic deficits. The degree to which this is due to involvement of frontal structures involved in behavioral regulation, the role of the anterior temporal lobes in behavior, and the effects of semantic loss on behavior is not currently known [20]. Although functional abilities inevitably decline, there is some evidence that PPA patients are able to maintain relative independence in living for longer than patients with bvFTD [5].
Caregivers usually experience increasing burden and strain as the disease progresses. As the patient loses functional abilities, caregivers must take on tasks such as refilling prescriptions, scheduling and taking patients to appointments, and handling correspondence [21]. In patients with behavioral symptoms, the caregiver may need to eliminate hazards in the home or provide close supervision of the patient during meals as patients can put excessive and even dangerous amounts of food in their mouths. Communication may become very limited, either due to language symptoms or as a symptom of apathy. Home health aides can provide some practical help in managing the patient’s symptoms, giving caregivers much-needed respite.
Continuity of care is an important part of managing FTD. Patients often show fluctuation in behavioral symptoms, with new ones developing over time and others worsening. Frequent clinical visits can help to manage these evolving symptoms on a timely basis. Even if no medicines are prescribed or altered, a visit in which problematic behaviors and non-pharmacologic strategies are discussed can be very valuable for the patient and caregiver. A collaborative relationship with the patient’s primary care provider should also be maintained between visits. Keeping an accurate log of symptoms can help clinicians differentiate disease fluctuations from possible side effects of medications or other causes.
Pharmacologic management
Given the diversity of symptoms and pathologic heterogeneity of FTD, it is perhaps not surprising that pharmacologic treatment of the symptoms of FTD needs to be tailored to the patient. Cognitive and language impairments and certain behavioral symptoms in FTD are generally not amenable to pharmacologic treatment. The behavioral symptoms that are not usually improved with medication treatment include reduced empathy, deficits in social cognition, and apathy. Important goals of pharmacotherapy in FTD include educating the family as to what symptoms the clinician is targeting with medication treatment, that medications can ameliorate, but generally not remove, behavioral symptoms, and the creative integration of pharmacologic and non-pharmacologic treatments (see Table 17.1). For example, medications can be used to attempt to reduce compulsive pacing and wandering, but measures should also be undertaken to prevent elopement or minimize its consequences, e.g., locked doors and identification bracelets.
Symptomatic treatments for FTD spectrum disorders
| Domain | Symptom | Pharmacologic tx | Non-pharmacologic tx |
|---|---|---|---|
| Language symptoms | Expressive aphasia | None | Speech therapy; caregiver education; compensatory tools such as scripts and augmentative and alternative communication devices (AACs) |
| Naming and comprehension deficits | None | Speech therapy; caregiver education on communication methods | |
| Behavioral and neuropsychiatric symptoms | Apathy and inertia | None | Caregiver education and support; supervision and direction |
| Agitation, aggression, and impulsive behaviors | Antidepressants, atypical antipsychotics | Caregiver education; monitoring and removal of environmental triggers, caregiver oversight of physical and social environment | |
| Lack of empathy and sympathy | None | Caregiver education; caregiver support groups | |
| Perseverative and ritualistic behaviors | Antidepressants | Caregiver oversight; toleration of behavior; distraction | |
| Compulsive eating and dietary abnormalities | Antidepressants | Caregiver oversight of diet; environmental and physical modifications; consultation with dietician | |
| Cognitive symptoms | Executive dysfunction | Evaluation for medications that could impair cognition | Consultation with cognitive rehabilitation therapist; compensatory tools |
| Motor symptoms | Falls | Evaluation for medications that could contribute to parkinsonism, orthostasis, or balance impairment | Environmental modifications; physical therapy; consultation with occupational therapist; walkers and/or wheelchairs |
| Dystonia | Botulinum toxin injections | Splinting; physical therapy | |
| Parkinsonism | Carbidopa/levodopa trial (in part, for diagnostic purposes) | Caregiver support |
Antidepressants can reduce some behavioral symptoms, particularly disinhibition, compulsive behaviors, and hyperorality. Although there have been few double-blind, placebo-controlled studies, case series and open label studies have tested the effects of selective serotonin reuptake inhibitors (SSRIs) (fluoxetine, sertraline, paroxetine, fluvoxamine, citalopram) and trazodone [22]. FTD demonstrates significant serotonergic deficits in imaging and autopsy studies [23]. Despite the lack of large-scale systematic clinical trials, there is some evidence for their symptomatic efficacy [24]. FTD patients are generally physically younger and healthier than other dementia patients and can usually tolerate doses similar to those used for psychiatric indications. Potential side effects include nausea, headache, and sexual symptoms, but usually these medications are well tolerated in FTD, and some adverse effects, e.g., decreased appetite or libido, may even be beneficial. Antidepressant use has been associated with an increased rate of falls in elderly patients and patients with Parkinson’s disease [25]. While it has not been directly assessed, this finding suggests that antidepressants should be used cautiously with disorders such as progressive supranuclear palsy (PSP) for which falls are a prominent symptom. The choice of a particular antidepressant is often informed by the receptor profile and medication interactions of the medication. Sertraline (Zoloft) and citalopram (Celexa) are SSRIs with minimal effect on other receptors and cytochrome P450 enzymes. Although recent evidence showing QT prolongation with citalopram use, especially at higher doses, has limited its use.
Atypical antipsychotics can be prescribed to treat disinhibition and agitation. There have been few formal studies of these medications as symptomatic treatments in FTD. However, this class of medications has historically been used to treat psychosis and agitation in patients with dementia from AD [26]. Quetiapine (Seroquel) is often a first-line agent because of its relatively low D2 receptor occupancy, given the increased risk of extrapyramidal symptoms (EPS) in FTD. Its use, however, is associated with significant sedation and should be used cautiously in patients with orthostatic hypotension owing to its anti-histaminic, -adrenergic, and -muscarinic actions, and clinicians should be familiar with prescribing the other agents in this class of medications. The therapeutic and adverse effects of this class of medications can generally be predicted by their profile of receptor blockade. Typical antipsychotic medications are generally not used because of their increased risk of EPS. Olanzapine (Zyprexa) is rarely used in FTD because it is often associated with an increase in appetite, due to its blockade of H1 and 5-HT2C and 3 receptors. Clozapine (Clozaril) has minimal D2 occupancy, but has many potential adverse effects, including agranulocytosis, requiring blood draws and limiting its use. Risperidone (Risperdal) has a relatively higher D2 occupancy, and thus higher incidence of EPS than most of the other atypical antipsychotics, but it appears to reduce agitation with less sedation than quetiapine and can be useful in patients who do not tolerate quetiapine because of excessive sedation. Clinicians should prescribe these medications with caution, as their use in elderly dementia patients has been linked to a 1.5- to 1.7-fold increased risk of mortality and a black box warning from the US Food and Drug Administration (FDA) [27]. The cause of this increased mortality is incompletely understood, but is linked to an increased risk of cerebrovascular events. Evidence indicates that patients with AD do not tolerate the high doses of antipsychotic medication that are used to treat schizophrenia. Furthermore, beyond a certain dose, e.g., the equivalent of approximately 2 mg of risperidone, there is a minimal increase in efficacy in treating agitation and psychosis in AD [26]. However, it is unclear how this literature translates to FTD, in which the patients are generally younger and healthier than AD patients. There is little evidence on length of treatment for both antidepressants and antipsychotic medications. In general, patients should be evaluated for continued need for medications for behavioral symptoms, especially for antipsychotic medications given their increased mortality. Some experts have argued that a discontinuation trial of antipsychotic medication should be considered for patients whose neuropsychiatric symptoms have been in remission for three to six months [27]. However, emerging evidence has shown in patients with AD that agitation appears to be a symptom that often does not spontaneously remit and discontinuation of antipsychotic medications results in a recurrence of symptoms [28].
Acetylcholinesterase inhibitors (AChEIs) are prescribed for up to 40% of FTD patients [29]. These medications are used in AD, in which the cholinergic system is disrupted. In FTD, the relatively intact cholinergic system presents a poor target for these medications. Clinical studies do not support their use as effective symptomatic treatments for FTD, and one study has documented worsening behavioral symptoms [22]. These considerations have prompted recommendations to avoid treating FTD with AChEIs [30]. Prescribing these medications may be warranted in cases of diagnostic uncertainty with AD or logopenic variant PPA, which usually exhibits AD pathology.
Memantine is a non-competitive N-methyl D-asparate (NMDA) antagonist approved for use in AD. It is prescribed for 10–15% of FTD patients [29]. Initial positive case reports with bvFTD patients spurred hope for the use of memantine in FTD. Since then, however, several placebo-controlled studies have shown no therapeutic effects of memantine on cognition or behavior [31, 32], with one study showing adverse effects on cognition [31].
A complete review of the pharmacologic treatment of the motor syndromes associated with FTD is beyond the scope of this review and readers are directed to other sources [33, 34]. However, a few themes emerge in relation to the interaction of motor and non-motor symptoms of these disorders. Patients with FTD-ALS (amyotrophic lateral sclerosis) should be evaluated for possible riluzole (Rilutek) treatment. While cognitive impairment is not necessarily a contraindication for riluzole treatment, clinicians need to consider issues such as medication compliance and effects of cognitive impairment on quality of life in the decision. Pseudobulbar affect can be a prominent symptom in MND and can complicate the diagnostic evaluation of patients with FTD-ALS. If problematic to patients, this symptom can be treated with serotonergic antidepressants or dextromethorphan/quinidine (Nuedexta). Most patients with PSP and corticobasal degeneration (CBD) will not have a significant or sustained response to levodopa treatment [35], but most experts recommend a trial of levodopa treatment, in part for diagnostic purposes [35].
Other pharmacologic treatments for FTD are under investigation. Lithium, most commonly used to treat bipolar disorder, inhibits tau hyperphosphorylation [36]. However, it was not tolerated at doses used to treat bipolar disorder in a trial for PSP [37]. There is theoretical evidence to suggest that dopaminergic augmentation may represent a future symptomatic treatment for FTD. FTD patients exhibit low levels of cerebrospinal fluid dopamine metabolites and reduced dopamine transporters in the caudate [23]. Executive function deficits in attention-deficit hyperactivity disorder are effectively treated with dopamine augmentation. One study has shown a reduction in risk-taking behavior following treatment with methylphenidate, a stimulant that enhances synaptic dopamine [22]. A medication that increases prefrontal dopamine levels, tolcapone, is currently under investigation as a symptomatic treatment for FTD [38]. Molecular mediators of social cognition may offer another approach in treating FTD. Administration of oxytocin, a neuropeptide implicated in human bonding and trust, leads to increased empathy and cooperative behavior in normal adults [39]. An initial test of oxytocin as a possible therapeutic agent for bvFTD yielded promising results. Small improvements were seen across a wide set of behavioral symptoms [40], leading to a dosing study to test the safety and tolerability of intranasal oxytocin [41].
Non-pharmacologic interventions
While particular behavioral symptoms may improve with pharmacologic treatment, managing FTD relies in large part on a diversity of non-pharmacologic interventions (see also Chapter 16). Environmental modifications, compensatory tools, rehabilitation therapies, and behavioral interventions can all play a role in helping to facilitate life functioning for the patient and family. As in the introduction of medications, the particular interventions to be utilized will depend on the specific symptoms and types of impairments of the individual patient.
Cognitive impairments
Early cognitive impairment in FTD is usually secondary to deficits in executive function [3]. The term “executive function” encompasses more complex cognitive abilities including reasoning, abstraction, and mental flexibility. Deficits in these abilities frequently cause difficulty for patients with mentally demanding jobs that involve planning and problem-solving, eventually causing them to take on less demanding positions at work or into early retirement. At home, these deficits can manifest in difficulty with tasks such as managing finances, preparing complex meals, and planning trips.
To compensate for some of these functional impairments, families may need to adjust roles. The primary responsibility for long-term legal, financial, and healthcare decisions often shifts to the caregiver. Caregiver supervision may be needed to help ensure the safe completion of more complex activities of daily living. A symptom of FTD is behavioral rigidity and difficulty learning new behaviors or altering behaviors based on feedback. Patients’ families should be educated to lower their expectations about how much behavioral change they can expect from reasoned debate with FTD patients, even when the patient states that he or she understands and will alter their behavior.
Cognitive rehabilitation professionals also have expertise that may help ease the effects of cognitive impairment in FTD. The progressive nature of FTD precludes adopting the approaches often used with stroke and traumatic brain injury patients. Remediation of skills and a return to normal, independent functioning is not a viable goal. However, patients can benefit from being introduced to compensatory techniques that can help maintain life functioning for the patient and the family. Daily planning tools can help organize the steps needed to complete particular job-related tasks. Checklists and home assessments may be effective in helping to avoid hazardous outcomes at home or at work. Routines that take advantage of the relative preservation of memory seen in FTD may also be suggested. A thorough assessment of the particular deficits and life activities of the patient can lead cognitive rehabilitation professionals to suggest specific compensatory remedies.
Cognitive impairments can present a particular hazard in patients with FTD-ALS. Because of their impaired judgment and attention, these patients are particularly at risk for reduced compliance with feeding tube procedures, non-invasive ventilation, and occupational and physical therapy [42]. Physical safety can be compromised if they are unable to understand how to prevent and cope with falls or choking episodes. Patients may not be able to use assistive technology such as computer interfaces that require learning and training. As deteriorating cognitive function can compromise the ability to make the various healthcare decisions that are required in ALS, families should discuss plans for future action as early as possible in the disease course.
Communication impairments
Communication impairments in FTD are of two types. In bvFTD, early problems in communication are often related to problems with social cognition, motivation, and emotional expression. Patients who fail to respond with appropriate language or tact can experience a breakdown in communication with friends and neighbors that are unaware of the FTD diagnosis. The perception of emotional detachment can also lead others to shy away from initiating conversation with the patient. Indifference and apathy can lead to diminished speech, causing some patient needs to not be met. While these symptoms usually cannot be directly targeted in the patient, educating caregivers on better communication styles can help minimize their effects. Open-ended questions may fail to generate responses, but patients often respond better when given defined choices. Indirect repairs that restate an understanding of what was said can lead to higher quality interactions than direct repairs that provide immediate corrective feedback. Talking down to patients using “elderspeak” (e.g., using “honey” or “sweetie”) can create resistance and negative consequences. There is some evidence in patients with AD that dementia caregivers can successfully be taught some of these communication skills, leading to fewer behavioral disturbances [43].
Patients with PPA suffer impairments in communication due to the accumulation of deficits in language abilities. Behavioral interventions are the mainstay for ameliorating the effects of these impairments. These generally fall into two categories. One approach is to attempt to maintain or improve deteriorating language skills through language therapy. Thus far, case reports suggest that abilities such as comprehension of spoken instructions and questions, production of sentences, and word retrieval may be amenable to short-term improvement using domain-specific language exercises [44]. Formal studies documenting the efficacy of these types of interventions are lacking, as is evidence that these benefits remain after the conclusion of therapy. Patients that have retained insight, are motivated to learn, and have supportive and actively involved caregivers are the best candidates to pursue these interventions. Speech-language pathologists (SLPs) that have experience working with neurodegenerative conditions can help tailor interventions to individual needs.
More commonly, patients with PPA will benefit from interventions that maximize functional communication through compensatory strategies. Although they are underutilized in PPA [45], SLPs are trained to introduce a variety of such interventions for patients with stroke and other disorders [46]. At the mild stages, SLPs may suggest adopting strategies such as self-cueing and script training to enhance conversation. In more moderate stages of the disease, augmentative and alternative communication devices (AACs) such as communication books can be utilized to supplement speech. Electronic and computer AACs require intact learning ability, necessitating training while cognitive impairment is relatively mild. SLPs can also suggest ways for families to promote better communication with the patient. Families can be advised that speaking slowly, simply, and face-to-face facilitates greater language comprehension. Noise and other distractions should be minimized to ease understanding. Providing more time for communication, asking for clarification, and supplementing speech with gestures are other tips that will reduce a breakdown in communication.
Motor impairments
Patients with FTD spectrum disorders can present with motor deficits that complicate the management of the disease. Falls are frequent in PSP especially. Physical therapists can play an important role in intervention by assessing mobility and prescribing weighted or rolling walkers or wheelchairs as needed. Trained health aides and family members can help reduce falls. Physical therapy can also be instituted to promote passive range of motion in affected muscles, especially for the dystonia of CBS, helping to prevent contractures and maintain limber joints [47]. Environmental modifications are essential interventions to ensure patient safety and can be suggested by occupational and physical therapists. These may include instituting predictable schedules, arranging for supervision when travel is required, and reducing environmental hazards such as throw rugs and clutter.
Behavioral disturbances and neuropsychiatric impairments
Social and behavioral disturbances are the most frequent cause of distress for FTD caregivers [48]. Pharmacologic measures can sometimes reduce positive symptoms such as disinhibition and agitation, but non-pharmacologic interventions are frequently necessary for dealing with the full scope of symptoms. Given the lack of insight commonly seen in FTD, environmental modifications and educational measures targeting the caregiver can be useful to help manage these symptoms.
Apathy and inertia. Patients lose interest and drive in undertaking previously rewarding activities (apathy), or are not able to initiate or complete actions on their own (inertia). For example, patients may give up some hobbies in favor of passively watching TV, or be unable to finish preparing a meal or brushing their teeth. Caregivers can sometimes help the patient successfully engage in life activities. One suggestion is to offer several options for activities to do together rather than leaving things up to the patient in an open-ended way. In other cases, the caregiver may need to direct the person as to what to do. For completing activities around the home, the caregiver may be able to divide a task into small, simple steps and provide guidance to help the patient finish it. For chores such as cooking that may be hazardous if not done in an appropriate way, supervision may be provided. Participating in passive activities such as watching movies with the patient can still be a source of mutual satisfaction. It should be noted that families often experience frustration when trying to find activities that will engage the patient’s interest. To reduce tension and strain, they should be counseled to lower their expectations about how interested the person can be and that their lack of interest is a symptom of the illness.
Disinhibition and impulsive behaviors. Patients with bvFTD show a decreased ability to assess the social and practical consequences of their words and actions. As such, they exhibit poor decision-making and judgment. This may manifest as vulnerability to sales pitches, impulse buying, reckless driving, indiscriminate sharing of personal information, gambling, or shoplifting. In social situations, patients can become more impulsive and violate typical norms. They may laugh inappropriately, make offensive jokes, or stand too close to strangers. Caregivers can modify the environment in order to reduce harmful consequences from these behaviors. Postal delivery can be changed to a post office box so that mail can be better supervised. Access to bank accounts and credit cards can be limited to avoid financial recklessness. Car keys can be kept away from the patient. Guns and other weapons should be removed from the house. In social contexts, caregivers can be advised to monitor the patient’s contact with the public. Going to venues where the patient is already known and explaining the diagnosis to neighbors can reduce the chances of misunderstanding the patient’s behavior. Families may choose to sit in more secluded areas of the theater or restaurant to minimize disruptions. Electronic devices with GPS can be used track the patient’s location and prevent wandering out of sight. Cards that explain the patient’s behavior as a symptom of illness can also be passed out if necessary.
Socially disruptive behaviors sometimes necessitate the involvement of clinicians. In legal settings, clinicians may be called upon as expert witnesses to assess responsibility or risk of future criminal behavior in patients. Clinicians should monitor for physically abusive or sexually inappropriate behavior towards minors. In these cases, the safety and welfare of children is paramount. Families should be advised to keep children away from patients displaying these behaviors, and these behaviors will likely necessitate placement in a different setting without children present.
Loss of empathy and sympathy. Diminished responsiveness to other people’s needs and decline in social engagement is a feature of bvFTD. Patients may make hurtful comments that fail to appreciate other people’s feelings. Blunted emotional and facial expression such as lack of eye contact can cause friends and family to see the patient as cold and distant. Family members may not be thanked for attending to caregiving tasks, or the patient may stop playing with children or grandchildren. These symptoms can cause confusion and hurt in family members. Given their strong emotional and developmental needs and lack of understanding, children are especially vulnerable to being affected and may experience feelings of guilt or responsibility when they fail to receive previously available emotional warmth and encouragement. An important intervention for managing the effects of this symptom is education and support about the nature of the disease. Understanding these symptoms as a product of changes in the brain can mitigate emotional upheaval in the family. Caregiver support groups and conferences and FTD websites are often valuable resources towards this end. Well parents should take care to have open and truthful communication with their children, and to monitor their emotional states. Psychotherapy for children or other family members may be indicated. In addition, well parents can support their children by modeling healthy coping skills and finding outlets for engaging their strengths and accomplishments [49].
Perseverative and ritualistic behavior. Patients can engage in repetitive and stereotyped behaviors or compulsively engage in certain actions. Examples include repeatedly saying a phrase or story, washing the same item, walking a fixed route, collecting the same object, or rubbing a body part. The simplest response may be to advise the caregiver to tolerate the behavior, especially if it is relatively harmless. Sometimes a less distressful behavior can be substituted for the current one. For example, a laptop or portable DVD player can allow a patient to watch the same TV show over and over again without getting in the way of going to a doctor’s appointment. Distraction is another method that can be used to displace the patient’s attention away from a repetitive behavior or routine. If the behavior gets in the way of important responsibilities or leads to risky situations, pharmacologic management of the symptom may be warranted.
Compulsive eating and dietary abnormalities. Patients can exhibit profound changes in eating behavior, often developing carbohydrate cravings and eating past the point of satiety. Sometimes, patients ingest inedible items or compulsively consume alcohol. These symptoms can often be very frightening for the family and lead them to institute necessary environmental modifications. Locking cabinets and refrigerators, removing unsafe foods from the home, and providing supervision during mealtimes are all steps that can be taken to minimize danger. Providing only small portions on plates can be useful. A dietitian can be consulted to strategize ways to provide appropriate nutrition while attending to safety needs. As with all behavioral symptoms of FTD, helping families to adjust expectations can be helpful. For example, some family members become very concerned with weight gain during the illness. But conflict with the patient can sometimes be reduced if some increase in food intake, and weight, is tolerated. Eating too quickly and putting too much food in their mouths can be a life-threatening symptom if it leads to choking, and this symptom can be especially dangerous in patients with FTD-ALS and dysphagia.
Aggression and agitation. Irritability, frustration, or anger may be expressed by some patients, sometimes during caregiving tasks. These behaviors cause the greatest burden for caregivers and are correlated with high levels of caregiver depression [9]. Remaining calm, avoiding arguments, and maintaining distance from the patient are strategies that can be adopted to reduce the chances of conflict. Caregivers are advised to look for specific causes for agitated behaviors, such as a medical problem or uncommunicated physical needs. Specific environmental triggers such as loud noises or social setting lead some patients to feel overwhelmed and respond negatively. Attending to these issues can decrease problematic behaviors. New physical limitations and environmental constraints are sometimes a source of frustration for patients. For example, patients with FTD-ALS may refuse to use walkers, putting them at greater risk for injury. In these cases, the caregiver can become overburdened as well as become the target of the patient’s aggression. These challenging behaviors can cause caregivers to crave respite from caregiving responsibilities.
Depression. While symptoms of depression may be seen in both bvFTD and PPA, in bvFTD, somatic factors such as decreased motivation and inertia are often dominant, with little evidence of affective symptoms such as depressed mood, poor self-esteem, or suicidality. These patients, because of their poor reasoning abilities and lack of insight, are generally poor candidates for psychotherapy. Patients with PPA are at greater risk for developing depressed mood and related symptoms [50]. This may be due to their relatively preserved insight into their cognitive and behavioral difficulties [51], combined with increasing inability to express themselves and communicate their emotional needs. Language deficits frequently lead these patients to withdraw from social interaction with peers and family members. An inability to do everyday tasks, such as reading the newspaper and answering the telephone, sometimes leads to feelings of worthlessness. Gradual difficulty in participating in life activities can take an emotional toll on patients with PPA. Introducing new hobbies that do not require language, such as doing puzzles or painting, can help keep patients engaged. Counseling may help some patients come to terms with their loss of identity. Creative arts therapies involving dance or music can promote relaxation. Case studies and small trials suggest that visual art therapy can be harnessed to engage attention and improve neuropsychiatric symptoms and self-esteem [52].
Non-pharmacologic interventions have been shown to be effective in managing some of the behavioral symptoms of dementia both in institutional settings and via caregiver delivery at home [53]. Day programs are one example of an intervention that is delivered in an institutional setting. These programs benefit patients by providing structure and stimulating and meaningful activities, such as gardening and exercise. They also help caregivers by providing much-needed respite and leading to reduced stress. Despite their benefits, FTD patients often do not fit the limits of care of these programs [54]. For example, staff are not trained to deal with socially disruptive behaviors, and are ill-prepared for the changes in eating behavior associated with bvFTD. Preliminary findings in a recently established FTD-specific day program include increases in facial expression and initiation of participation in activities and decreases in inappropriate behaviors [54]. Further research into these types of programs will help clarify the activities most beneficial for FTD patients.
A large part of managing FTD inevitably falls on the shoulders of the caregiver, often the spouse or child of the patient. Largely owing to the centrality of behavioral symptoms, FTD caregivers experience particularly high burden and strain compared with caregivers of other dementias [48, 55]. The young age of onset in FTD often leads to disruption in family life, leading to feelings of loss and anxiety for children. Financial strain is likely to result when a main provider for the family can no longer work. Social isolation and depression are common developments amongst FTD caregivers, especially given the stigmatization they may feel due to a lack of societal awareness of the disease [48]. These factors all add to the nuances involved in the practical management of FTD.
Identifying resources to help caregivers deal with the manifold consequences of this disease is an integral part of the clinician’s responsibility (see Table 17.2). Education and information are most effective when paired with more concrete steps to deal with behavioral symptoms. Referrals to occupational therapists, geriatric nurses, and other clinicians with experience in teaching behavioral modification techniques can help caregivers learn practical skills to better manage distressing behaviors. An example of such a technique is the Advanced Caregiver Training (ACT) model. In this model, dementia caregivers are taught how to identify triggers of patient behavior by monitoring medical conditions, environmental factors, and caregiver communication style [56]. Applying ACT and similar methods can reduce the burden of care as well as caregiver upset [56]. In addition to resources that can help with the practical management of behaviors, caregivers should also be introduced to resources to help them better manage their own mental health. Programs that target dementia caregiver emotional coping strategies can reduce depressive symptoms and psychological distress [57], preventing a breakdown in care and eventually leading to improved quality of life for patients [58]. Clinicians can also recommend psychiatric treatment for some caregivers whom they think would benefit.
Multidisciplinary management and coordination of care at different stages of FTD
Related posts:
The family’s perspective on FTD
The FTD-ALS spectrum
Neuropathology of frontotemporal dementia and related disorders
Functional disability and the impact of frontotemporal dementia in everyday life
Historical introduction to FTD
Overview of frontotemporal dementia and its relationship to other neurodegenerative disorders
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