Approach

In common with all other forms of dementia, treatment and care for people with frontotemporal dementia (FTD) is based on ensuring the safety of the patient and others, providing reassurance and support for patient and carers, and providing adequate guidance and supervision for a care team in managing behavioural complications and their practical consequences.

A number of factors may trigger behavioural and psychological symptoms in patients with dementia, so these should be managed or mitigated where possible, particularly if the patient is being treated in hospital. These include environmental and social factors (e.g., being in an unfamiliar environment, being exposed to loud noises, bright lights, or different temperatures, and being away from relatives/carers), physiological factors (e.g., pain), and pharmacological factors (e.g., polypharmacy).[146]​​

An important early step is to carefully identify and list all drugs that the patient is taking. This list should be reviewed and updated regularly, because the adverse effects of drugs (e.g., sedation, confusion, disorientation, restlessness, agitation, and hallucinosis), whether due to the drug itself or because of a drug-drug interaction, can cause or contribute to cognitive disability or behavioural disorder. Minimum treatments should be used in patients with FTD. Note, however, that discontinuing antihypertensive treatment in older patients with dementia is not recommended because of an association with serious adverse events.[147]

There is no evidence for effectiveness of drug treatment for cognitive symptoms associated with FTD. Acetylcholinesterase inhibitors and memantine have not demonstrated clinically significant efficacy in patients with FTD, and may hasten cognitive decline or worsen behavioural symptoms.[148][149]​​[150]

Supportive care

The first step in the care of a patient with FTD is to provide education and support for the patient and their family and carers, focusing on the key behavioural and psychological symptoms of FTD and how these translate to care needs. Planning should focus on meeting current needs and anticipating future issues. It is important to address long-term care needs early, because early planning and action maximise options and the patient's ability to participate. Family and carers should be empowered to assist the patient in making decisions regarding health and property, managing finances, taking drug treatments, cooking meals, etc. One systematic review of implementing non-pharmacological interventions in managing the behavioural and psychological symptoms of dementia in care homes found the key factors to be collaboration among staff and families, organisational support, staffing, education, and staff familiarity with both the interventions and residents.[151]​ Strengthening these areas could enhance care outcomes for aged‐care residents with dementia.

Case managers in collaboration with family physicians may have a role in addressing the needs of the patient. They should coordinate and integrate referral, transitions, and communication across all agencies involved in the assessment, treatment, support, and care of people with dementia, their carer(s), and families.[152]

Environmental review

A home safety evaluation should be undertaken, as well as an assessment of transport, driving, and self-care needs by an occupational therapist. Modification of the physical environment, such as room temperature and light and noise levels, may be appropriate.[153]

Roaming behaviour can serve a purpose for patients with behavioural variant FTD and so should not be completely discouraged, but may require supervision. The patient will typically follow the same pattern or routine. Providing a safe protected area for roaming that is free of clutter and obstacles, and wearing non-slip footwear, will help reduce fall risk.

Communication

Strategies that may improve communication between carers and patients with FTD include using short sentences, explaining things, making eye contact, and active listening.[154] Patients with dementia may be more willing to cooperate with care tasks when carers use high entitlement requests (e.g., ‘I’m going to do X’ or ‘I need to do X’) rather than low entitlement requests (e.g., ‘Is it ok if I do X?’ or ‘Do you mind if I do X?’).[155]​ Correcting or confronting the patient should be avoided; it is important to remain calm, and move away from the patient if challenging behaviour occurs.[156]

Support for family and carers

Guiding carers and providing support and encouragement is an integral focus of caring for a patient with FTD. Family and carers should be educated in communication and other techniques. Carers are often exposed to aggressive behaviour from patients with behavioural variant FTD, which can cause significant stress; a behaviour log may help to identify precipitators and patterns of aggression and warning signs.[154][157]​ Carers should be advised about coping techniques, and about local and national support organisations. An intervention that provides carers with personalised, structured post-diagnostic support can help them provide high-quality care to patients with dementia at home.[158]

In-home assistance, respite services, and residential care

Many patients require professional help in the home to provide respite to the family, and supervision and assistance to the patient. Daycare services can offer respite to carers and patients, and may be used in combination with in-home care.

In many cases, continued home care is no longer possible due to the nature of the care situation (e.g., a spouse who cannot retire) or to problem behaviours (e.g., night-time roaming, belligerent behaviour). Patients who require residential care should be cared for in a specialist dementia unit.

Non-pharmacological and behavioural interventions

The aim of non-pharmacological stratagies is to prevent problematic behaviours, relieve behavioural symptoms, and reduce carer distress. Non-pharmacological and behavioural approaches comprise various types of intervention: cognitive and emotion-oriented approaches (e.g., cognitive stimulation, reminiscence therapy, validation therapy), sensory stimulation (e.g., music and dance therapy, touch therapy), behaviour management techniques, multicomponent interventions, and other therapies (e.g., exercise).[159] In addition, non-pharmacological approaches to management in FTD include lifestyle modifications, speech, occupational, and physiotherapy, peer and carer support, and safety considerations.[160]​ Lifestyle modifications including aerobic exercise increase strength and balance, and may prevent falls. Non-pharmacological strategies may be as or more effective than pharmacological treatments, and have fewer adverse effects.[161][162][163]​​ One Cochrane review evaluating the effectiveness of psychological interventions to reduce the use of antipsychotics​ in care home residents was unable to make a conclusion because of low certainty evidence. However, the study found that psychosocial interventions are not associated with an increase in harmful events such as accidental falling or hospital admissions.[164]

Cognitive stimulation therapy or cognitive training may improve cognitive function among patients with mild to moderate dementia, but evidence is of low quality.[165][166]​​

One meta-analysis concluded that music‐based therapeutic interventions may reduce depressive symptoms, and improve overall behavioural problems and social behaviour, in people with dementia, but likely have no effect on agitation, aggression, anxiety, emotional well-being, or cognition.[167]​ Music therapy combined with physical activity may be useful in controlling anxiety, irritability, and restlessness in patients with FTD.[168]​ One systematic review found that massage and touch therapy, and cognitive stimulation combined with exercise and social interaction were more efficacious than usual care at reducing the symptoms of depression in people with dementia.[163]​ Cognitive behavioural therapy and other types of psychological therapy may help reduce symptoms of depression and/or anxiety in patients with dementia.[169][170]​​

Studies comparing pharmacological and non-pharmacological interventions for treating aggression and agitation in adults with dementia have found that multidisciplinary care, massage and touch therapy, music combined with massage and touch therapy, animal-assisted intervention, and personally tailored interventions were clinically more efficacious than usual care.[171][172]​​

Sleep disturbances (e.g., insomnia, night-time restlessness, night-time roaming) are not uncommon in patients with FTD.[173] There is no evidence about specific interventions for patients with FTD, but keeping active during the day, avoiding naps, and good sleep hygiene may improve sleep quality. One Cochrane review found that physical and social activities, as well as carer interventions may have some benefits on sleep for people with dementia.[174]

Treatment of some behavioural and psychological symptoms, including disinhibition, apathy, compulsions, and hypersexuality, in people with FTD remains a challenge in clinical practice. Although some pharmacological treatments show promise, none demonstrate clear symptom benefit and non-pharmacological treatment continues to be a fundamental tool for the management of these symptoms. Strategies that might be helpful include:[81][175][176][177]​​

  • Encouraging engagement in recreational activities or games to reduce disinhibition.

  • Using receptive music therapy to help prevent apathy.

  • Providing alternative strategies to limit compulsive behaviours, such as using a stress ball to prevent touching strangers, or sucking a lollipop to diminish repetitive vocalisation.

  • Delivering psychoeducation, support groups, and tailored interventions for patients and carers to alleviate emotional and psychological distress caused by hypersexuality.

Pharmacological interventions for behavioural and psychological symptoms

Pharmacological interventions for behavioural and psychological symptoms should be considered only when non-pharmacological options have been unsuccessful. With any drug, a minimum effective dose should be established through dose titration on a case-by-case basis. High dosages and polypharmacy can contribute to cognitive impairment; clinicians should aim to minimise the use of antipsychotics, mood stabilisers, benzodiazepines, and any drug with anticholinergic effects.

It should be noted that family members are sometimes concerned that symptoms are being caused by drugs. There is no evidence that acetylcholinesterase inhibitors and memantine are effective for treating the behavioural and psychological symptoms of FTD.[148][149][150]

Pharmacological management strategies may be appropriate for some specific behavioural presentations in patients with FTD, although few studies have investigated this.[148][178][179]

Acute irritability, restlessness, agitation, or aggression

A benzodiazepine or an antipsychotic drug may be trialled if non-pharmacological treatments have proved unsuccessful, although evidence for efficacy is limited.[178][180][181]

Sedative drugs should be used cautiously in all early-onset dementias, including FTD. When rapid sedation is required and non-drug interventions (e.g., distraction techniques, de-escalation techniques, massage, touch therapy) have not been effective, or when personal injury seems likely, an anxiolytic drug (e.g., lorazepam) can provide useful short-term benefits. These drugs can also be used in planned combination with non-drug treatments, although their benefit in the long-term management of aggressive behaviour is not established.

Introduction of sedative drugs and principles governing their use, including risks, should be discussed with the patient's main carers or relatives. Treatment should be time-limited and regularly reviewed. Regular review of sedatives should include examination for possible parkinsonian adverse effects and akathisia, because these may prolong behavioural problems (or be misidentified as behavioural problems), as well as monitoring for metabolic and cardiovascular adverse effects.[180]

Antipsychotic drugs are associated with serious adverse effects and increased mortality in patients with dementia.[182][183]​​​​ Therefore, they should only be considered if symptoms are severe, are dangerous, and/or cause significant distress to the patient; physicians and families must consider the potential risks and benefits of antipsychotics for the individual patient. The American Psychiatric Association has published practice guidelines on the use of antipsychotics to treat agitation in dementia.[180] Treatment should be time-limited and regularly reviewed. Antipsychotics may increase the risk of falls, so strategies to prevent falls should be implemented.[184]​ No advantages in terms of efficacy or safety have been identified for any specific antipsychotic drug for the treatment of behavioural and psychological symptoms of dementia, although one meta-analysis found that risperidone was probably the best option for short-term use.[185][186]​​ In order to avoid polypharmacy, antipsychotics that can be used safely by both the oral route and by injection are sometimes preferred. Initial doses of an antipsychotic should aim to achieve optimum benefit at the minimum possible dose. Generally, antipsychotics should be avoided in patients who already manifest parkinsonism as a feature of the dementia. However, if the need for an antipsychotic arises, quetiapine would be the preferred option for cases complicated by parkinsonism. There is an increased risk of stroke with antipsychotics for patients with dementia.

Psychosis

Managing psychosis in people with FTD can be challenging. Patients with FTD are susceptible to adverse reactions to antipsychotics, especially their extrapyramidal adverse effects. In patients with FTD and severe behavioural disturbances who invariably require an antipsychotic, atypical antipsychotics (e.g., risperidone, quetiapine, olanzapine, aripiprazole) are preferred as they have fewer extrapyramidal adverse effects. Atypical antipsychotics improve psychiatric and behavioural symptoms in some patients with FTD.[187]​ Therefore, physicians and families must consider the potential risks and benefits of antipsychotics for the individual patient. The American Psychiatric Association has published practice guidelines on the use of antipsychotics to treat psychosis in dementia.[180]​ Generally, antipsychotics should be avoided in patients who already manifest parkinsonism as a feature of the dementia. However, if the need for an antipsychotic arises, quetiapine would be the preferred option for cases complicated by parkinsonism. There is an increased risk of stroke with antipsychotics for patients with dementia.

Other symptoms

  • Sleep disturbances: pharmacological interventions have not been formally evaluated in patients with FTD, their use deriving from experience in a wide range of neuropsychiatric conditions, including Alzheimer’s disease and other non-FTD dementias. Successful treatment of sleep disturbances may have secondary benefits in improving mood and drive, and ameliorating distractibility and irritability. A preferred pharmacological approach is difficult to define due to a lack of evidence in patients with FTD, as well as variation in the clinical picture and neuropathology, and uncertainty around the balance of benefits and risks.[181] Drugs that may be considered include mirtazapine (at low doses), zolpidem, eszopiclone, zaleplon, trazodone, melatonin, ramelteon, and a benzodiazepine.[188]​ Note, however, that several of these drugs may be associated with an increased risk of falls and fractures in older people and those with dementia.[189][190]

  • Distractibility, perseveration, and restlessness: amantadine is an option for the treatment of dysexecutive states associated with dementia.[191] Use of memantine (a drug related to amantadine that is used to treat moderate to severe Alzheimer’s disease) for treatment of FTD is not uncommon, but there is insufficient evidence to support any conclusions about efficacy, and memantine has no place in the treatment of FTD.[192][193]

  • Euphoria, excitability, mania, impulsivity, irritability, persistent restlessness, persistent agitation, or persistent aggression: valproate semisodium (valproic acid and sodium valproate in a 1:1 ratio) is typically prescribed; there are reports suggesting value in the treatment of FTD complicated by agitation, but no controlled-trial data.[194][195]​​ There is some evidence for topiramate as an anti-impulsive agent.[178]

  • Abnormal eating behaviour: topiramate is known to suppress appetite for food. There have been a few case reports suggesting effectiveness in patients with abnormal eating behaviour associated with FTD.[178][196]

  • Pseudobulbar affect: patients with FTD with motor neuron disease and progressive supranuclear palsy may show symptoms of pseudobulbar affect, characterised by sudden bouts of uncontrollable crying or laughter. Symptoms may respond to dextromethorphan/quinidine, which specifically targets pseudobulbar affect.[197]

Valproic acid and its derivatives may cause major congenital malformations, including neurodevelopmental disorders and neural tube defects, after in utero exposure.

  • These agents must not be used in female patients of childbearing potential unless other options are unsuitable, there is a pregnancy prevention programme in place, and certain conditions are met.

  • Precautionary measures may also be required in male patients owing to a potential risk that use in the 3 months leading up to conception may increase the likelihood of neurodevelopmental disorders in their children.

  • Regulations and precautionary measures for female and male patients may vary between countries, with some countries taking a more heightened precautionary stance, and you should consult your local guidance for more information.

In some countries, topiramate is also contraindicated in pregnancy and in women of childbearing age unless the conditions of a pregnancy prevention programme are fulfilled to ensure that women of childbearing potential: are using highly effective contraception; have a pregnancy test to exclude pregnancy before starting topiramate; and are aware of the risks associated with use of the drug.

Treatment of concomitant illnesses

Behavioural complications may derive in part or full from concurrent illness such as urinary tract infections or pneumonia, which may manifest as heightened confusion and disorientation, lethargy, irritability, agitation, hallucinations, and paranoia. Treatment of the underlying illness will usually lead to a prompt resolution of the acute state.

Choice of antibiotic regimen depends on disease severity, treatment setting, and causal agent; regimens should follow accepted guidelines for drug treatment of older adults.[198]

See Community-acquired pneumonia in adults, Urinary tract infections in women, and Urinary tract infections in men.

Decisions to withhold treatment must not be made when the patient is unwell and the infection is first detected. Rather, they must be made, if at all, when the patient is well and in consultation with relatives and senior nursing staff. This type of decision must be recorded and must be open to scrutiny.

End-of-life care

Inevitably FTD will culminate in profound disability and death. Therefore, it is essential to establish and record, early in the course of the illness, the preferences of the patient and family regarding end-of-life interventions, including treatment, resuscitation, and prolonging life when treatable conditions arise.[199][200]​ End-of-life care generally is focused on providing comfort and basic needs (e.g., help with feeding and cleanliness, adequate pain control, good skin care, and prevention of injury through falls or misadventure). There is little evidence about the best way to provide end-of-life care for people with advanced dementia and further research in this area is warranted.[201]​ Behavioural complications other than loss of willpower and lethargy are uncommon at this stage and do not warrant pharmacological intervention.

Swallowing difficulties are not uncommon and can be managed by diligent hand feeding and varying the consistency of the diet. For patients who choke frequently on food or lose the ability to swallow, feeding by gastrostomy tube may help in the short term, but does not improve survival, morbidity, or quality of life in the long term.[202][203]​​

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