Understanding the 7 stages of frontotemporal dementia

What is frontotemporal dementia?

Dementia describes a number of conditions that tend to cause issues with a person’s memory, thinking skills or communication. Frontotemporal dementia (FTD) is a type of dementia that occurs as a result of damage to the nerve cells in two specific lobes in the brain – the frontal lobe and the temporal lobe. This may happen as the result of several types of diseases that target these two areas, including a group of disorders that involve a buildup of the protein tau, and a group of disorders that involve the buildup of the TDP43 protein. These disorders cause deterioration of certain connections, and deplete the chemical messengers required for brain function.Frontotemporal dementia is not as common as some other types, but it is thought to account for around 1 in 30 cases of dementia in the UK, meaning an estimated 31,000 people suffer from the condition. It tends to affect younger age groups, being most common in people aged between 40 and 60. Like other types of dementia, the symptoms of frontotemporal dementia differ depending on the person and their general health. As this type affects the frontal and temporal lobes which are responsible for things like personality, behaviour, language and speech, the main symptoms a person may experience include behavioural or personality changes, and problems with speech and language. Memory loss is common in the early stages of other dementia types, but this is not the norm in the early stages of frontotemporal dementia. You can learn more about the variations of dementia in our guide to the different types of dementia.

Are there different types of frontotemporal dementia?

There are two main types of frontotemporal dementia (FTD); behavioural variant FTD, and primary progressive aphasia, known as PPA. 

• Behavioural variant FTD – This occurs when the damage to the frontal lobes causes issues with a person’s behaviour and personality that are uncharacteristic. This happens because the lobes involved in this type of dementia are responsible for emotions, planning skills, problem-solving skills, and focus.
• Primary progressive aphasia (PPA) – This occurs when the damage to the temporal lobes causes language issues due to its role in remembering names, the meaning of words, and recognising objects and people.

What are the 7 stages of frontotemporal dementia?

All types of dementia progress at different rates for different people, and a broad overview of the stages include the mild, moderate and severe stages. You can read more about this in our guide to managing the stages of dementia.Medical professionals may use the 7-stage Global Deterioration Scale (GDS) designed by Dr. Barry Reisberg in 1982 to categorise dementia symptoms. These stages include: 

Stage 1: No Cognitive Decline

The very early stages of frontotemporal dementia can be difficult to detect, and may not show up on dementia assessments. If any signs are noticeable, these will often be slight unusual occurrences, personality changes or behavioural abnormalities which may be considered normal signs of ageing by loved ones, or could even be misdiagnosed as a psychiatric condition or depression. Some examples of personality changes could include becoming more impulsive, saying inappropriate things, or having fewer inhibitions.Studies suggest frontotemporal dementia specifically is often confused with primary psychiatric disorders due to the overlapping early stage symptoms and relatively young age of onset. Around 50% of frontotemporal dementia patients with the behavioural variant receive a psychiatric diagnosis before their dementia diagnosis.At this early stage, carers or loved ones should focus on promoting the person’s overall health, wellness and cognitive function. The earlier the diagnosis, the better, so if you do notice any subtle signs in yourself or a loved one do not hesitate to seek medical advice. 

Stage 2: Very Mild Cognitive Decline

Very mild cognitive decline in frontotemporal dementia may start to present with some more noticeable symptoms of cognitive decline, but these may still be overlooked by loved ones depending on how slight they are. In stage 2 the person could experience more difficulties when socialising, driving, or while at work, and could start to experience apathy or disinterest in usual activities. Early detection of dementia is still important at this stage, so if you suspect you or a loved one may be experiencing this, speak to your GP as soon as possible. This early stage could be a good time to consider practical steps such as creating an Advance Statement for Care, a Living Will, and a Health And Welfare Power Of Attorney. The earlier these are created, the better for both the person experiencing dementia, and for their loved ones. You could also start to find patient, creative ways to communicate with the person if they are beginning to lose language skills or struggle to find the right words.

Stage 3: Mild Cognitive Decline

Still under the umbrella of mild dementia, mild cognitive decline is the third stage, and tends to come with more noticeable symptoms such as confusion, difficulty undertaking simple tasks they once did easily, executive function difficulties, or issues with language. They may also experience difficulties when planning, organising, decision-making and problem-solving, which could cause a noticeable decline in work performance.In stage 3 the person may still be able to confidently live independently, but they may also be experiencing symptoms that could make their employment more difficult if they are still working. They may need assistance with certain activities, have home adaptations made to pre-empt any physical needs down the line, and start a home care routine so they get used to the idea of having help with small tasks. 

Stage 4: Moderate Cognitive Decline 

Next is moderate dementia, which increases the noticeability of symptoms. A person at this stage may experience a significant decline in things like problem-solving skills, memory, and communication difficulties. They may need more help with simple tasks they were previously able to do themselves, such as daily personal care tasks or cooking dinner by themselves. Behavioural changes are also common here. Home care may become more necessary at this stage to help with everyday tasks that they cannot do alone, and help them attend dementia-related appointments. More advanced symptoms of frontotemporal dementia may start to appear, such as tremors, coordination issues, and difficulty swallowing. Medical professionals may recommend speech and language therapy to improve communication between the person and their caregivers if this is an issue, as often by this stage of frontotemporal dementia speech and finding certain words is a big challenge. This can negatively impact their quality of life and ability to ask for what they need, so it is an important symptom to address. As their caregiver, you may need to find ways to manage difficult behaviour and challenging conversations as they start to display more complex behaviour changes. Each person’s needs will be unique, but some strategies you might wish to use include:

• Use clear, simple language and short sentences when speaking to them so they can understand more 
• Utilise non-verbal cues with gestures, facial expressions and visual aids
• Listen and validate their feelings, even when they are difficult to understand 
• Establish a structured routine to offer consistency and reduce the person’s anxiety
• Distraction techniques could redirect them towards more positive topics or activities  
• Create a calming environment to minimise the person’s stress and agitation
• Involve the help of professional caregivers and other types of support, such as behavioural therapists

Stage 5: Moderately Severe Decline

At this stage, as frontotemporal dementia progresses, further decline in cognition and memory issues may be noticed which can be similar to that of Alzheimer’s disease, as well as further behavioural symptoms that can be difficult to manage, such as aggression. This often requires more assistance such as medical care and home care, if they choose to stay at home instead of a care facility. In the moderate to severe stages, a person may need help with all steps involved in their personal care routine, and may also require help with mobility and moving around the home safely. The World Health Organization recommends people with dementia stay at home to maintain a strong sense of wellbeing and remain in familiar surroundings where they have a lower likelihood of confusion and anxiety as their condition progresses. Further home adaptations may be needed to help with physical decline, which can be common in the moderate or advanced stages of all types of dementia. Some lesser known physical symptoms of dementia include things like repetitive movements, sleep issues, ‘sundowning’, and mobility problems. You can read more about these symptoms in our guide to the physical symptoms of dementia.

Stage 6: Severe Decline

Severe dementia of any kind can cause significant cognitive and physical decline that requires constant care. At this stage a person will almost certainly need professional caregivers at least on a part-time basis to help with their symptoms and daily living, unless they have the help of a full-time family carer. Communication can be extremely difficult or even non-existent at this stage, and they may no longer be able to recognise some of their family or friends. Behavioural changes might involve aggression, paranoia or agitation, which can be difficult to manage on a daily basis.  Physical abilities and motor skills may also decline further at this stage, so caregivers may be required to help with all forms of movement so the person can do things like move around the home and get out of bed. On the other hand, their condition may be so advanced that they become bedbound. You may find more useful information in our guide to advanced dementia.

Stage 7: Very Severe Decline/End-of-Life Care

Stage 7 in dementia is usually considered the final stage, which is when a person will require palliative care for pain and symptom management, or end-of-life care. Caregivers will typically be in close contact with medical professionals who may provide treatments to help with some symptoms.This stage often means the person is unresponsive, unable to communicate, and will require constant care as they approach the end of their life. This could last for several weeks, months or years depending on the person’s condition, and some of the physical signs that could be present in the final severe stages of frontotemporal dementia include difficulty swallowing, reduced appetite, and recurrent infections. At this stage it may be time to consider the person’s quality of life and comfort, ensuring they maintain their dignity, and putting palliative care in place to help manage any pain they may be experiencing. You may find more helpful information about this stage in our guides: 

• What Is Palliative Care?
• Palliative Care At Home: What To Expect
• When Should Someone Be Offered Palliative Care?
• What Is An End Of Life Care Plan?

How is frontotemporal dementia diagnosed?

A diagnosis of either the behavioural variant of frontotemporal dementia or of primary progressive aphasia is usually based on an evaluation by a medical professional with experience of and knowledge of these disorders. Diagnosis will take into account the patient’s individual symptoms and experiences as well as results from neurological exams and brain scans.As with other forms of dementia, frontotemporal dementia is best treated if diagnosed early in order to better manage symptoms and prepare for the future. At the first sign of any of the aforementioned symptoms in yourself or a loved one, you should seek medical help and a dementia diagnosis, if your GP thinks this is appropriate.

How is frontotemporal dementia treated and managed?

There is no known cure or preventative treatment for frontotemporal dementia, so treatment and management of this condition relies on managing the symptoms in order to reduce their intensity, and ensuring the person’s quality of life is prioritised throughout its progression. With all cases of dementia, a team of specialist doctors, nurses and therapists familiar with these disorders will usually be brought in to guide the treatment, which may include a combination of medications to treat symptoms, and therapy options. Specifically for those with frontotemporal dementia who suffer from behavioural issues, experienced behavioural therapists and/or professional caregivers may be enlisted to help manage symptoms, and provide support to families and loved ones who may struggle by themselves, and who may experience a lot of negative emotions as a result of behavioural symptoms. Keep in mind when caring for a loved one with behavioural variant frontotemporal dementia, challenging them on their behaviour may not help the situation and could further aggravate them. Instead of arguing, try to accept their symptoms, remember they are not in control, and try using redirection or distraction to manage symptoms. You could also try using mindfulness techniques to maintain your patience during difficult moments. 

How can home care help people with frontotemporal dementia?

Having the support of home carers can be extremely helpful for anyone caring for a person with frontotemporal dementia at various points throughout the progression of their condition, but especially in the advanced stages. If you are looking into caring for someone with dementia at home, discussing specialist care can be helpful so you can be prepared for the future and notice signs of progression as they happen. When hiring a professional carer for a loved one with frontotemporal dementia, you may wish to ask questions such as:

• What does dementia care help with?
• How does frontotemporal dementia care differ from other types of dementia care?
• How can a home carer help with frontotemporal dementia behavioural symptoms?
• At what stage is home care required for frontotemporal dementia? 
• Can respite care help someone with frontotemporal dementia? 
• Does a person with frontotemporal dementia need a live-in carer?

At Home Instead, we offer specialist, person-centred care, meaning as long as the person requiring care is able to make decisions about their own care and how they spend their time, our Care Professionals will respect their wishes provided they are safe and well. They will also provide a high standard of dementia-focused care through our unique training programme created by ageing experts, dementia specialists and key medical practitioners from across the world. This training is accredited by City & Guilds, so you can rest assured you are in the right hands. We’re an award-winning home care provider and part of a worldwide organisation devoted to providing the highest-quality relationship-led care for older people in their own homes. Arranging care for yourself or your loved one shouldn’t be stressful, so whatever questions you would like answered, feel free to reach out to the Home Instead team to discuss your needs.