Introduction

Definition of frontotemporal dementia

Frontotemporal dementia (FTD) is a group of neurodegenerative diseases that are characterised by deficits in behaviour, language and executive functions that worsen over time.¹ FTD is used to describe types of dementia that do not come under the branch of Alzheimer’s. The regions affected by FTD usually include the frontal and anterior temporal parts of the brain.² Those who have FTD, showcase symptoms seen in patients with psychiatric disorders such as schizophrenia, depression and bipolar disorder.^1,3

Importance of addressing behavioural and psychological symptoms

The impact of symptoms can affect patients, their loved ones, caregivers and healthcare providers. Once a diagnosis has been made, it is imperative to ensure management strategies. This can allow for a tailored approach for the patients, and make it easier for them to transition into living with these symptoms.

Overview of behavioural and psychological symptoms in FTD

Behavioural changes

Laganà et al. (2022) stated that some of the most common behavioural changes that can occur due to FTD, include disinhibition, apathy and compulsive behaviours.³ FTD can manifest a different level of severity in each patient, such as a patient having a significant increase in aggression, engaging in impulsive behaviours or developing a lack of social understanding of what is appropriate. Other symptoms of behavioural changes include a personality change, where there is a loss of interest in the activities the patients were usually engaged in, and they can also show a lack of motivation in other general settings, such as going outside or seeing friends. Other changes include a reduced sense of basic function, where a patient may engage in compulsive or repetitive behaviours that they otherwise would not do.³

Psychiatric symptoms

Laganà et al. (2022) explored some psychiatric symptoms that can be shown due to FTD.³ This includes psychosis, where a patient may experience hallucinations and delusions. Other symptoms can occur within the patient's mood, in which not only are they likely to experience depression and anxiety, but they can also be affected emotionally by being apathetic and lacking an emotional response to situations.³ These symptoms can lead to tension in their relationships - with family, friends, other loved ones and caregivers.

Pathophysiology of behavioural and psychological symptoms in FTD

Neurological basis

The areas of the brain that are affected by FTD include the frontal and temporal lobes.² The frontal and temporal regions of the brain are involved in behaviour control, language and executive function.¹

Core behavioural symptoms such as disinhibition, apathy, compulsive behaviour and so on, were significantly linked to a specific gene variant called MAPT, in comparison to other variants. This led to a high frequency and severity of behavioural symptoms when there was a notable presence of the MAPT gene variant.⁴ Psychiatric symptoms were “highly expressed”⁴ in other gene variations, which suggests the importance of genetic variants that lead to a higher frequency and severity of certain symptoms. These genes contribute to both behavioural and psychiatric symptoms and illustrate the variability in symptom presentation amongst patients.⁴

Progression of symptoms

Symptom onset can occur without the patient knowing, and can often be mistaken for other disorders. For example, the symptom of hallucinations could initially be diagnosed as a schizophrenia symptom, instead of a FTD symptom.¹ As stated by Cerejeira, et al. (2012), there are different severities and frequencies related to symptoms from when FTD starts.⁵ Overall, behavioural and psychological symptoms of dementia were still present after two years, which was shown in a longitudinal study. Some symptoms were rated as being more persistent than others, such as delusions.⁵

Assessment and diagnosis of behavioural and psychological symptoms in FTD

Clinical evaluation

Instruments to assess the behavioural and psychiatric symptoms include clinical evaluations, such as looking into patient history, and tools, for example, the Neuropsychiatric Inventory.⁶

Early detection of behavioural and psychological symptoms is important, as it allows the patient to understand what is happening to them, as well as preparing those around them for when they encounter any issues or worrying situations. Pharmacologic and nonpharmacologic approaches can be utilised to ensure the patient can deal with certain symptoms that arise.⁷

Differential diagnosis

The complexity of the symptoms found in FTD implies that treatment evaluations need to be tailored to suit the symptoms that are being expressed in the patients.⁷ Biomarkers are associated with FTD, such as a protein, which can help to identify depressed patients at the early stages of FTD, focusing on the psychological symptoms that can arise.⁸ This is useful for finding patients who have FTD instead of having depression. 

Management strategies for behavioural and psychological symptoms

Non-pharmacological approaches

Behavioural interventions include cognitive behavioural strategies, which are useful in focusing on daily problems, such as dealing with aggressive behaviour. This can help reduce symptoms for a few months.⁶

Psychosocial support can provide education for patients and caregivers. This helps reduce symptoms, due to addressing stressful events, providing information about the disease and how the caregiver can assist. By utilising multicomponent groups, it can engage information sharing and experiences to learn from.⁶ 

Modifying the patient's lifestyle to be more structured, and including daily activities, can help build a sense of purpose. One activity could be physical activity, which can improve outcomes in patients with dementia in a cognitive and behavioural sense.⁶ Another activity is sensory stimulation. This can reduce certain symptoms such as disruptive behaviours, but this seems to be a short-term effect.⁶

Pharmacological treatments

By addressing particular symptoms, especially if they are one of the more prominent symptoms in a patient, it can help the patient in moderating their symptoms. However, in elderly patients, they are more vulnerable to pharmacological side effects.⁶

Antidepressants can be used to target anxiety and depressive symptoms in FTD and could have a knock-on effect on other symptoms such as aggression⁶ state that SSRIs are the more “favourable treatment” option but they come with severe side effects.⁶

In extreme cases, antipsychotics are used in some countries, such as the atypical antipsychotic risperidone, whose purpose in FTD treatment is for symptoms of aggression. Other antipsychotics can be used for other symptoms, such as haloperidol treating delirium, but it could cause adverse effects on other symptoms a patient may have, and they have severe side effects as well.⁶

Multidisciplinary approach

Since the diagnosis of FTD, the beginnings of clinical evaluations are essential to building the ideal team for the patient and their family. With the involvement of a multidisciplinary team - neurologists, psychiatrists, psychologists, social workers and so on, the patient's treatment plan can be tailored. A non-pharmacological approach being used first, and only integrating pharmacological treatments in severe circumstances, can allow for the patient's needs to be met and reduce the risks, for example, the use of drug treatment can lead to adverse side effects. By engaging in regular assessments to ensure the effective use of these approaches, the patient will be kept up to date in the monitoring of their symptoms, and when it would be best to add or take away any treatments if they are not needed anymore.⁶ 

Challenges in managing behavioural and psychological symptoms

Ethical considerations

When it comes to treatment decisions, there are ethical considerations to take. This includes a level of consent, and the autonomy a patient and their family have as individuals, especially since the patient will experience symptoms relating to cognitive decline. The two most important factors are patient safety and balancing it with quality of life for the patient, which are noticeably imperative to consider especially with pharmacological treatments and the adverse side effects they bring.

Caregivers perception

Caregivers may have difficulty in coping with the change, which is why strategies to manage the distress can be helpful. For example, the caregiver’s perception of the patient can lead to them being upset over the change in the patient, and in turn, can lead to distress on the patient's end.⁹ Strategies to cope can include communication with support groups and interventions in a family setting.⁹

Conclusion

FTD symptoms are behavioural and psychological. Therefore, having multiple approaches to target each individual symptom is important. This type of management leads to the need for multidisciplinary and comprehensive care. Having an early diagnosis, allows the patient to benefit from a more tailored and concentrated approach by monitoring the symptoms as early as possible. This can lead to a more predictive model of symptom frequency and severity, which can be accounted for by the patient, caregiver and loved ones. The preferred management strategy includes the non-pharmacological approach, but in severe cases of symptoms, pharmacological treatments can be adopted. Specific challenges in managing symptoms of FTD include ethical concerns and the caregivers’ perception of the patient.