Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

all patients

1st line – supportive care

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ONGOING
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all patients
1st line – 

supportive care

Provide education and support for the patient and their families 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.

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]

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.

Strategies that may improve communication between carers and patients with FTD include using short sentences, explaining things, making eye contact, and active listening.[92]

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.

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 generally be cared for in a specialist dementia unit.

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]

Plus – benzodiazepine or antipsychotic

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ONGOING
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all patients
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with acute irritability, restlessness, agitation, or aggression
Plus – 

benzodiazepine or antipsychotic

Treatment recommended for ALL patients in selected patient group

A benzodiazepine or an antipsychotic may be trialled if non-pharmacological treatments have proved unsuccessful, although evidence for efficacy is limited.[104][106][107]

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, a benzodiazepine (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]

Alternatively, an antipsychotic drug can be used. 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 drugs 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 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.

Primary options

lorazepam: 2 mg orally/intramuscularly every 30-60 minutes when required, maximum 10 mg/day

Secondary options

risperidone: 0.25 mg orally once or twice daily initially, increase according to response, maximum 2 mg/day

OR

quetiapine: 25 mg orally once or twice daily initially, increase according to response, maximum 150 mg/day

OR

olanzapine: 2.5 to 5 mg orally once daily initially, increase according to response, maximum 5 mg/day

OR

haloperidol: 0.25 to 0.5 mg orally once or twice daily initially, increase according to response, maximum 2 mg/day

Plus – treatment of concurrent illness

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ONGOING
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all patients
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with concurrent urinary or pulmonary infection
Plus – 

treatment of concurrent illness

Treatment recommended for ALL patients in selected patient group

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

Choice of antibiotic regimen depends on disease severity, treatment setting, and causal agent; it follows accepted guidelines for drug treatment of older adults.[119] 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.

Caution should be exercised when renal impairment is suspected, and drug doses adjusted accordingly.

Some antibiotics are associated with psychotoxicity (e.g., fluoroquinolones), leading to increased risk of confusion and drowsiness, which may be mistaken for symptoms of FTD.

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.

Consider – selective serotonin-reuptake inhibitor

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ONGOING
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all patients
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with compulsions that dominate or significantly interfere with everyday life
Consider – 

selective serotonin-reuptake inhibitor

Additional treatment recommended for SOME patients in selected patient group

Selective serotonin-reuptake inhibitors (SSRIs) have an established place in the treatment of compulsions in psychiatry, particularly for obsessive-compulsive disorder. There is limited, low-quality evidence that these drugs may ameliorate compulsive behaviour in patients with FTD.[85][89][110]

SSRIs may be prescribed to patients with severe compulsions that dominate everyday behaviour. A treatment effect may be apparent within a few weeks, but a 6- to 8-week trial at an optimal dose may be required (the optimal duration of therapy for compulsions in dementia has not been formally investigated).

If effective, it may be continued indefinitely. However, a discontinuation trial may be considered after 6 to 12 months of symptom remission.

SSRIs are well tolerated, although hyposomnia and sexual dysfunction are established adverse effects. Hyponatraemia is a rare complication, due to syndrome of inappropriate antidiuretic hormone hypersecretion. Citalopram seems to have fewer adverse effects than other agents when used in older people with dementia, and this may be so in patients with FTD.

Primary options

citalopram: 20-40 mg orally once daily

OR

sertraline: 50-200 mg orally once daily

OR

fluoxetine: 20-80 mg orally (immediate-release) once daily

OR

paroxetine: 10-40 mg orally (immediate-release) once daily

Consider – non-pharmacological interventions and pharmacotherapy

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ONGOING
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all patients
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with sleep disturbances
Consider – 

non-pharmacological interventions and pharmacotherapy

Additional treatment recommended for SOME patients in selected patient group

Sleep disturbances, such as hyposomnia, insomnia, night-time restlessness, and night-time roaming, are not uncommon in patients with FTD.[103] There is no evidence about specific non-pharmacological interventions in this population, but keeping active during the day, avoiding naps, and good sleep hygiene may improve sleep quality.

Pharmacological intervention may be required when sleep hygiene regimens are not effective or impractical.

These 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 the lack of evidence in patients with FTD, variation in the clinical picture and neuropathology, and uncertainty around the balance of benefits and risks. Drugs that may be considered include mirtazapine (at low doses), zolpidem, eszopiclone, zaleplon, trazodone, melatonin, ramelteon, and a benzodiazepine (e.g., clonazepam).[111]

Higher doses of zolpidem and eszopiclone may cause next-day drowsiness and psychomotor or memory impairment. Higher doses of zolpidem should not be used in women.

Primary options

mirtazapine: 7.5 to 15 mg orally once daily at bedtime

OR

zolpidem: 5 mg orally (immediate-release)/sublingually once daily at bedtime; 6.25 mg orally (extended-release) once daily at bedtime

OR

eszopiclone: 1-2 mg orally once daily at bedtime

OR

zaleplon: 5-10 mg orally once daily at bedtime

OR

trazodone: 25-150 mg orally once daily at bedtime

OR

melatonin: 0.3 to 2 mg orally once daily at bedtime

More

OR

ramelteon: 8 mg orally once daily at bedtime

OR

clonazepam: 0.25 to 0.5 mg orally once daily at bedtime

Consider – amantadine

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ONGOING
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all patients
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with distractibility or perseveration
Consider – 

amantadine

Additional treatment recommended for SOME patients in selected patient group

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]

Careful observation in the first week after initial prescription and each increase in the dose of amantadine is required.

Primary options

amantadine: 50-100 mg orally twice daily initially, increase according to response, maximum 400 mg/day

Consider – valproate semisodium or topiramate

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ONGOING
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all patients
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with euphoria, excitability, mania, impulsivity, irritability, persistent restlessness, persistent agitation, or persistent aggression
Consider – 

valproate semisodium or topiramate

Additional treatment recommended for SOME patients in selected patient group

Anticonvulsants are used widely in neuropsychiatry for the treatment of manias, hypomanias, labile emotions, impulsions, irritability, agitation, and aggression.

Typically valproate semisodium (valproic acid and sodium valproate in a 1:1 ratio) is prescribed (unless the main indication is a co-occurring epileptic state); 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]

Primary options

valproate semisodium: consult specialist for guidance on dose

Secondary options

topiramate: consult specialist for guidance on dose

Consider – topiramate

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ONGOING
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all patients
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with abnormal eating behaviour
Consider – 

topiramate

Additional treatment recommended for SOME patients in selected patient group

Topiramate is known to suppress appetite for food. There have been some case reports suggesting effectiveness in patients with abnormal eating behaviour associated with FTD.[104][118]

Primary options

topiramate: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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