Approach
In common with all other forms of dementia, treatment and care for people with 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.
An important early step is to carefully identify and list all medicines that the patient is taking. Minimum medications should be used in patients with FTD. This list should be reviewed and updated regularly, because the adverse effects of medicines (e.g., sedation, confusion, disorientation, restlessness, agitation, and hallucinosis), whether alone or in interaction, can cause or contribute to cognitive disability or behavioural disorder.
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.[89]
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 problems. 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 medicines, cooking meals, etc.
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.[90]
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.[91]
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.[92] Correcting or confronting the patient should be avoided; it is important to remain calm, and move away from the patient if challenging behaviour occurs.[93]
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.[92][94] Carers should be advised about coping techniques, and about local and national support organisations.
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
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).[95] Non-pharmacological strategies may be as or more effective than pharmacological treatments, and have fewer adverse effects.[96][97]
Cognitive stimulation therapy or cognitive training may improve cognitive function among patients with mild to moderate dementia, but evidence is of low quality.[98][99]
One meta-analysis concluded that music‐based therapeutic interventions may reduce depressive symptoms and anxiety, and improve overall behavioural problems, emotional well‐being, and quality of life, in people with dementia, but have no effect on agitation, aggression, or cognition.[100] Music therapy combined with physical activity may be useful in controlling anxiety, irritability, and restlessness in patients with FTD.[101]
A comparison of pharmacological and non-pharmacological interventions for treating aggression and agitation in adults with dementia found that multidisciplinary care, massage and touch therapy, and music combined with massage and touch therapy were clinically more efficacious than usual care.[102]
Sleep disturbances (e.g., hyposomnia, insomnia, night-time restlessness, night-time roaming) are not uncommon in patients with FTD.[103] 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.
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 medication with anticholinergic effects.
Pharmacological management strategies may be appropriate for some specific behavioural presentations in patients with FTD, although few studies have investigated this.[89][104][105] It should be noted that family members are sometimes concerned that symptoms are being caused by medication. There is no evidence that acetylcholinesterase inhibitors and memantine are effective for treating the behavioural and psychological symptoms of FTD.[89]
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.[104][106][107]
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 medication and principles governing its 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 sedative medication 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 side effects.[106]
Antipsychotic drugs are associated with serious adverse effects and increased mortality in patients with dementia.[108] Therefore, they should only be considered if symptoms are severe, are dangerous, and/or cause significant distress to the patient. The American Psychiatric Association has published practice guidelines on the use of antipsychotics to treat agitation or psychosis in dementia.[106] Treatment should be time-limited and regularly reviewed. 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.[109] 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.
Other symptoms
Compulsions that dominate significantly or interfere with everyday life: there is limited, low-quality evidence that selective serotonin-reuptake inhibitors (SSRIs) improve these symptoms.[85][89][110]
Sleep disturbances: 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.[107] Drugs that may be considered include mirtazapine (at low doses), zolpidem, eszopiclone, zaleplon, trazodone, melatonin, ramelteon, and a benzodiazepine.[111]
Distractibility, perseveration, and restlessness: amantadine is an option for the treatment of dysexecutive states associated with dementia.[112] 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.[113][114]
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.[115][116] In both Europe and the US, valproate medicines must not be used in younger female patients (of childbearing potential) unless there is a pregnancy prevention programme in place and certain conditions are met.[117] Valproate is associated with risk of congenital malformations and developmental problems in the infant/child. There is some evidence for topiramate as an anti-impulsive agent.[104]
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.[104][118]
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.
Treatment of pneumonia and urinary tract infections in FTD follows accepted guidelines for drug treatment of older adults.[119]
Caution should be exercised when renal impairment is suspected, and drug doses adjusted accordingly. Drug toxicity is more likely when drug excretion is impaired.
Some antibiotics are associated with psychotoxicity (e.g., fluoroquinolones). These are effective antibiotics either alone or in combination with beta-lactams but are linked to increased risk of confusion and drowsiness, which may be mistaken for symptoms of FTD.
Treatment of infections in patients with FTD is a demanding therapeutic challenge: swallowing difficulties and refusal to take oral medications are frequent and when other medications (antipsychotics, anticholinergics, or antihistamines) are used, the risk of pneumonia is increased.
Causal agents vary, but community, nursing home, and hospital-acquired pulmonary infections can generally be attributed to one or more of Streptococcus pneumoniae, Haemophilus influenzae, Staphylococcus aureus (including MRSA), Pseudomonas aeruginosa, and many gram-negative bacilli.
Choice of antibiotic regimen depends on disease severity, treatment setting, and causal agent.
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.[120][121] 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). 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.[122]
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