Frequently asked questions
We are commonly asked questions about FTD from people with FTD, their family members, and other professionals. We aim to put our answers to the most commonly asked questions here, with questions about genetic testing on our Genetic FTD and living at-risk page.
FTD can be difficult to diagnose. Symptoms can be very subtle to start with. For bvFTD, people have little insight into their symptoms and are often in denial of them. Furthermore, many primary care doctors will only see a handful of people with FTD during their entire career. This all means it can be a frustrating time getting a diagnosis for the person with FTD. It is well recognised that diagnosis of FTD is often delayed compared with other forms of dementia such as Alzheimer’s disease.
We are always happy to receive referrals from GPs or other hospital doctors to answer the question about whether someone has a form of FTD. We can also direct people to their local specialist FTD clinic if not close to us, or not in the UK.
It is also important to recognise that not every change in behaviour is FTD. Other neurological disorders can cause changes, as can psychiatric disorders such as depression and late onset psychosis. We sometimes see people in our clinic who have been diagnosed with FTD but do not have it on further review and investigation.
Although we describe individual syndromes, like bvFTD, PPA, etc. it is not uncommon for these conditions to overlap and for people to accumulate diagnoses over time:
- People with bvFTD can develop language problems (a PPA) or movement problems (either MND or Parkinsonism).
- People with nfvPPA can develop behavioural symptoms (bvFTD) or movement problems (often PSP or CBS, rarely motor neurone disease).
- People with svPPA develop behavioural symptoms (bvFTD) but only extremely rarely movement problems.
- People with lvPPA develop memory problems similar to Alzheimer’s disease.
- People with CBS or PSP may develop behavioural problems (bvFTD) or language problems (a PPA).
There have been few trials of drugs in FTD and so doctors sometimes prescribe medications that have been used in other conditions to manage symptoms. See our Management page to see what medications are recommended in FTD.
This is always a difficult question and not one that we have a good answer to. We can quote an average for different forms of FTD but by its very nature there will always be some people with a shorter duration illness and some with a longer duration illness – the range of disease durations is wide. We don’t yet understand the factors that cause an illness to be shorter or longer than average.
For the different clinical syndromes the average duration from first symptom to death is:
- bvFTD – 8 years
- nfvPPA – 9 years
- svPPA – 11 years
- lvPPA – 8 years
- FTD-MND – 3 years
- CBS – 7 years
- PSP – 7 years
Our collaborative international study looked at the duration of disease in people with genetic FTD. For the different genetic groups the average duration was:
- MAPT mutations – 9 years – although the average was different for different mutations, such as 5 years for P301S, 7 years for N279K, 8 years for P301L, 11 years for 10+16, 14 years for V337M, and 17 years for R406W
- GRN mutations – 7 years
- C9orf72 expansions – 6 years
There are no validated staging systems that have been developed for FTD. However, one can consider each of the syndromes to roughly have very early, early, middle and later stages.
- Very early – subtle change in personality, sometimes an extension of previous personality traits; can be difficult to characterize as abnormal; often decreased motivation or a change in empathy towards close family members.
- Early – clear change in personality with behavioural symptoms from the range of different symptoms seen in bvFTD [link to Clinical syndromes section]; difficulties with more complex areas of thinking including making decisions, solving problems, planning; usually able to carry on normal day-to-day activities.
- Middle – more severe behavioural symptoms and thinking impairment which impacts on day-to-day activities but usually able to live at home.
- Very early – subtle difficulties with speech production, often worse when speaking on the phone or publicly; may say the opposite of what they mean e.g. yes for no (or vice versa).
- Early – clear difficulties with speech production which may sound effortful; making errors in speech including saying the wrong sounds and getting words in the wrong order; writing and spelling may start to become difficult; usually able to carry on normal day-to-day activities.
- Middle – speech production is now very effortful with multiple errors in speech; comprehension may be starting to be impaired; clear difficulties with writing and spelling; difficulties with other parts of thinking include making decisions, solving problems, planning; changes in behaviour may start to be noticeable; usually able to live at home but may need some assistance with day-to-day activities.
- Very early – subtle problems with finding the right words, often more difficult (low frequency) words; may seem to ‘not hear’ things properly due to mild problems with comprehension; may ask what something is which they should clearly know.
- Early – word-finding difficulties with impaired ability to name items; often using a wrong word of similar meaning or saying ‘thing’; may have problems with reading, particularly words that have an irregular spelling (cannot be sounded out); impairment of understanding; may be subtle behavioural symptoms such as being obsessive or eating more sweet things; usually little effect on day-to-day activities.
- Middle – increasing word-finding problems with difficulties now with easier (high frequency) words; difficulties with reading and writing; difficulty recognizing people’s faces; worsening behavioural symptoms; usually able to live at home but may need some assistance with day-to-day activities.
- Very early – subtle difficulties with word-finding, may pause in the middle of a sentence or not complete a sentence.
- Early – speech becomes more hesistant with more pauses during sentences; occasional wrong words (which may sound similar to the word that was meant); may be developing subtle problems with other parts of thinking including reading, spelling and day-to-day memory; usually able to carry on normal day-to-day activities.
- Middle – speech now very hesitant; worsening day-to-day memory for recent events; impairment of many parts of thinking including reading, writing, arithmetic and spatial awareness; may have behavioural symptoms such as irritability, anxiety and decreased motivation.
For all forms of FTD, in the the later stages there is commonly minimal speech output and impaired comprehension as well as behavioural symptoms and thinking impairment; usually requires residential care; often swallowing problems, issues with continence, and impaired mobility.
These are all made more complicated if there is an additional motor problem such as MND/ALS or parkinsonism (including CBS/PSP).
Furthermore, because the disease progresses at different speeds in different people it can be difficult to say how long people spend at each stage before moving on.