Dementia describes a broad spectrum of conditions characterised by memory loss and changes in behaviour. It usually features progressive cognitive decline and accompanying changes in behaviour and language. One such form is frontotemporal dementia; although it is a distinct disease in its own right, it shares similarities with other forms of dementia, such as Alzheimer's disease and vascular dementia.

This article aims to help you understand how to recognise the common signs and symptoms associated with frontotemporal dementia. Although we will briefly cover investigation and management, the primary focus will be on identifying the symptoms.

What is frontotemporal dementia?

Frontotemporal dementia (FTD) is a specific kind of dementia, sometimes referred to as Pick’s disease.¹ It is typically caused by the formation of abnormal proteins,² which leads to shrinkage (or ‘atrophy’) of the front and sides of the brain,³ which approximately corresponds to the centres that control our emotions, language, and problem-solving abilities. Although less common than other forms of dementia, it typically affects younger adults, with the majority of cases being diagnosed in those under 65 years.⁴ FTD is an umbrella term for a group of disorders that share similar features. 

What are the risk factors for frontotemporal dementia?

• Several studies have demonstrated that genetics play a role in FTD.⁵ An estimated 20% of FTD patients have underlying genetic mutations predisposing them to this condition
• Up to 40% have a first-degree relative with a neurodegenerative disorder (either FTD or similar).^6,7
• Previous head injuries have been linked with dementia
• A link between occupational exposure to certain heavy metals (such as aluminium), pesticides and selenium-containing dietary supplements has been suggested⁹
• In general, some small studies suggest that this may also apply to FTD

Although further research is needed in this area.⁸ 

Common signs and symptoms of frontotemporal dementia

As with all forms of dementia, many potential symptoms may be present. These often co-exist, but many patients typically find that one group of symptoms predominates as the most disruptive to daily life. We will discuss below the four most common ways frontotemporal dementia can present.^10,11

1. Personality change or altered patterns of behaviour

It is very difficult to watch someone close to you undergo personality changes. Sometimes, it is obvious, and sometimes, it is far more subtle. It may include:

• A personality that has ‘coarsened’ or changed, making them sound different to before
• Poor engagement with work, a lack of enjoyment in activities or general apathy
• Being unable to take initiative or make independent judgements
• Social withdrawal, including being verbally dismissive of close friends and family or acting inappropriately in a social setting
• Self-neglect, which can include forgetting to wash or not cleaning a living space 
• Poor control of impulses, such as irresponsible spending, lacking inhibition or engaging in activities without fully considering any negative consequences
• Repetitive actions or behaviour patterns

2. Language and communication difficulties

This includes word processing challenges on multiple levels, for example:

• Difficulty with speaking (aphasia), such as hesitation and using simplified language
• Impaired speech and difficulty finding the correct words
• Problems understanding language
• Difficulty reading and writing

3. Cognitive decline

Typically, this is a gradual process that shows an overlap with other forms of dementia, although the early signs can be subtle. They include:

• Memory impairment
• Struggling to solve problems independently
• Difficulty with planning, decision-making, and multitasking

4. Movement and motor symptoms

You may notice stiffness, slow movement, tremors, weakness, or other signs of difficulty with movement. FTD often overlaps with disorders such as atypical Parkinson's disease and motor neurone disease.

What are the variants of frontotemporal dementia?

FTD can be divided into three categories:

Behavioural variant FTD

As the name would suggest, behaviour FTD is characterised predominantly by changes to the personality of those affected. Individuals may develop alterations in their personalities, repetitive or obsessive habits or show a lack of inhibition, as well as more subtle changes such as a reduction in libido or a change in preferences, e.g. music.¹² MRI imaging in this group often demonstrates shrinkage in the frontal part of the brain (the area in charge of decision making and judgement).

Primary progressive aphasia (PPA)

Aphasia refers to difficulty with language or speaking. This FTD subtype predominantly affects communication. It comes in two forms:

• The semantic variant (svPPA)– also called semantic dementia- is an issue with recalling information; it impacts the ability to assign meaning to words or objects.¹³ Initially, someone with this may be able to compensate quite well, but as the disease progresses, deficiencies will become more apparent
• Nonfluent variant (nfvPPA) - also called progressive non-fluent aphasia It is an issue with articulating information; it can include the use of inappropriate words or phrases, the mixing up of syllables or misplacing words in a sentence¹⁴

FTD with motor neuron disease (FTD-MND)

In any condition with processes leading to brain deterioration, there can be heavy overlap with neurodegenerative and movement disorders. As such, FTD can overlap with motor neurone disease and associated syndromes, including progressive supranuclear palsy or corticobasal syndrome.¹⁵ In these conditions, the issues with speech, language and behaviour discussed above overlap with movement disorders.

How will my healthcare provider reach a diagnosis of frontotemporal dementia?

There are several different scoring tools which your assessing clinician may use, as recommended by the UK-based National Institute for Clinical Excellence (NICE).¹⁶ 

Initial assessment may include:

• A review of the symptoms you are experiencing  
• Clinical examination of the nerves in your head and limbs
• Formal memory assessments
• Blood tests (in particular if your clinician is concerned about other causes for your symptoms)
• Imaging (e.g. MRI) 

If a diagnosis of FTD is suspected, you may be offered a referral to a specialist. As the principal goal is to manage the symptoms, a primary care practitioner (such as a GP) is likely to be involved in much of your care.

How is FTD managed?

FTD is a progressive disease for which currently there is no definitive cure.17 As such, it is important to recognise the symptoms early by seeking a medical review. Often, those with the disease may not be aware of its development, so if you are concerned by new changes in those around you, don’t delay a review. A primary care practitioner can assess your symptoms and, if appropriate, refer you onward to further specialists such as neurologists or geriatricians.

The broad range of symptoms that can develop with FTD can make management very challenging. Treatment is designed to be supportive and tailored to managing the symptoms.

Typically, a care plan will be created. The aim is to preserve as much independence as possible for patients, but it allows for any support that may be needed with daily tasks and allows for assistive modifications to be made to your home. Individuals with mild symptoms may be able to cope in their home environment, but as the needs progress, a further support network of care may be required, possibly in the form of in-house carers or full-time care in a specialised nursing environment. Using social societies, such as dementia memory cafes, can help keep the brain stimulated and may stall cognitive decline.

There is mixed evidence about the use of medical therapies, although certain antidepressants (called selective serotonin reuptake inhibitors (SSRIs)) can be used to help with disinhibition or compulsive behaviours.

If you are a carer or become one in the future, remember to ensure you are taking regular breaks. Some local areas can offer respite care, allowing you to rest and recharge. 

Summary

Frontotemporal dementia is a progressive neurological condition that can affect judgement, language and personality parts of our brain. There are several different variants, but if you are concerned that you, or someone close to you, is showing signs of personality change, speech difficulties, memory impairment or coordination/movement issues, then assessment by a clinician would be advised.