What Are The 7 Stages Of Frontotemporal Dementia (FTD)?

Over 55 million people worldwide live with dementia and the number is gradually increasing. Frontotemporal dementia (FTD), a type of dementia, is a devastating neurodegenerative disease which affects relatively young individuals in their forties to sixties. In this blog, we will understand the symptoms and types of FTD and explain the seven stages of Frontotemporal dementia that characterize the progression of FTD.
Frederika Malichová

Frederika Malichová

Neuroscientist at the University Of Cambridge.

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What is FTD?

Frontotemporal dementia is a neurodegenerative disease and the second most common cause of early dementia, affecting individuals between the ages of 40 to 60. It belongs to the group of frontotemporal disorders and is a type of frontotemporal lobar degeneration.

It is characterized by neuronal loss in the frontal and temporal lobes of the brain. The neuronal loss is a consequence of protein accumulation. In FTD, the proteins that may accumulate and cause this neuronal loss, are either microtubule-associated protein tau (MAPT), TAR DNA-binding protein 43 (TDP43), fused in sarcoma protein (FUS) or ubiquitin [1, 2, 3].

The protein accumulation targets areas responsible for social behaviour, language and personality. Therefore, the clinical symptoms may differ from patient to patient. However, the three most common clinical types of FTD are behavioural, language (semantic dementia) and motor variants [2, 4].

FTD can be either genetically inherited (familial FTD) or sporadic. The mutations responsible for FTD are on genes GRN (progranulin), MAPT and C9orf72 (Chromosome 9 open reading frame 72) [4].

Frontotemporal Dementia Symptoms

Symptoms of frontotemporal dementia vary depending on the clinical type of FTD [2, 5, 6]. However, the symptoms from different clinical types of FTD can overlap.

Behavioural Variant Frontotemporal Dementia

  • Inappropriate behaviour - This includes sexually or socially inappropriate behaviour.
  • Changes in personality - The patient may become aggressive or acquire compulsive behaviour.
  • Socio-cognitive changes - The patient experiences dietary changes, such as a sweet tooth.
  • Apathy - The patient is not interested in their environment or their loved ones.
  • Loss of empathy - The patient experiences a lack of empathy.

Language Variant Frontotemporal Dementia

  • Problems with understanding language - The patient has problems with understanding their language.
  • Problems with generating language - The patient can’t properly pronounce words, or they use words with different meanings.
  • Lack of engagement in conversations - The patient does not engage in conversations.
  • Mutism - The patient stops talking.

Motor Variant Frontotemporal Dementia

The motor variant of FTD is underpinned by symptoms observed in Amyotrophic Lateral Sclerosis. Typical symptoms of ALS, include muscle weakening, muscle atrophy, and difficulty with speaking [7].

FTD can also affect the eyes.

What are the 7 stages of Frontotemporal Dementia?

Frontotemporal dementia is a progressive neurodegenerative disorder. This means that the disease progresses, and unfortunately, the symptoms get worse in time. We can divide the progress of the disease into approximately seven stages.

Stages of Frontotemporal Dementia

Stage 1: Normal

The patient seems normal. There might be slight misunderstandings in communication, or behaviour, however, nothing which signals the disease. The patient goes to work and is capable of all daily activities.

Stage 2: Early Stage of Mild Cognitive Decline

The patient might start to repeat the same phrases, show mild problems with communication or behave slightly inappropriately. The patient can increase their daytime sleepiness. However, again, it is not a clear sign of the disease, but rather something that can be attributed to the disease after its diagnosis.

Stage 3: Moderate Cognitive Decline

The patient can experience problems with driving and a lack of interest and engagement in their work. We observe memory problems and compulsive behaviours. Further, the patient does not engage in conversations. In this stage, the loved ones have a suspicion that something is not right with their relative.

Stage 4: Severe Cognitive Impairment

The patient is usually unable to continue working, or driving and may start having problems with daily activities such as eating and cooking. In the case of language variant, the patient produces a minimum amount of words which are often “Yes”, “No”, or “So”. We observe severe cognitive decline and memory loss. However, the patient can walk and do sports activities. This is usually the time when the patient needs daily supervision.

Stage 5: Appearance of Motor Symptoms

We observe a problem with balance when executing sports activities. The movements of the patient may appear to us as slow. We observe a lack of interest in activities. Further, the patient starts having problems with swallowing food. Oftentimes, the patient experiences problems with incontinence and constipation. The patient continues to need daily supervision.

Stage 6: Worsening of Motor Symptoms

The patient becomes completely muted. Further, we observe a worsening of the motor symptoms and an increase in daytime sleepiness. The patient has problems with balance, and walking and shows a slough posture. The patient needs 24-hour care.

Stage 7: Late Stage: The End-stage of FTD

This is the end-of-life stage. The patient is unable to walk and is bedbound. In this stage, the patient needs constant care, has problems with eating and is prone to pulmonary infections.

Treatment for FTD

Currently, there is no effective cure for FTD. However, treatments to alleviate symptoms exist. These include:

  • Pharmacological Treatment - Some medications are used to relieve the behavioural symptoms. A selective serotonin reuptake inhibitor treatment has been shown effective [8, 9].
  • Non-pharmacological Treatment - Non-Pharmacological treatment of FTD includes physical therapy and language therapy [10, 11].

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Frederika Malichová

Frederika Malichová

Frederika is a postgraduate researcher at the University of Cambridge, where she investigates new biomarkers for Frontotemporal Dementia and other tauopathies. Her research has been published at prestigious conferences such as the Alzheimer’s Association International Conference 2023. She obtained her BSc in Biomedical Sciences from UCL, where she worked closely with the UK Dementia Research Institute.