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Terry Pratchett and his rare Alzheimer's diagnosis

Read about Terry Pratchett's diagnosis of posterior cortical atrophy (PCA), the rare form of Alzheimer's disease.

In 2007, aged 59, Sir Terry Pratchett publicly announced that he had a rare form of early-onset Alzheimer’s disease, called posterior cortical atrophy.

A docudrama on BBC2 followed the literary career of the beloved author, who was well known for his humorous and thought provoking fantasy novels. 

Here, Alzheimer’s Society Research Communications Officer Tim Shakespeare discusses what we know about posterior cortical atrophy.

Terry Pratchett and dementia

Before I joined Alzheimer’s Society, I carried out research into posterior cortical atrophy - the rare form of Alzheimer’s that Terry Pratchett was diagnosed with.

In this post I describe what it is, how it affects people, and what research is taking place.

Just to warn you, the post discusses the end stages of Alzheimer’s disease which some may find upsetting.

Terry Pratchett had a type of dementia that affects vision, not memory
 

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For many people, the first time they heard of posterior cortical atrophy (PCA for short) will have been when Terry Pratchett announced his diagnosis with the condition

Posterior cortical atrophy literally means ‘back of the brain shrinkage’. The back of the brain is where we interpret visual signals from the eyes and posterior cortical atrophy affects the ability of the brain to process these signals.

Symptoms of PCA can include difficulty reading, recognising objects and judging distances. Problems with spelling and calculation are common, as these skills rely on similar areas at the back of the brain.

Memory and language skills are usually less affected in the early stages of the condition, but as the disease spreads to more parts of the brain, people may develop more typical symptoms of Alzheimer’s disease such as memory loss and confusion.

Vision and perception can be affected by other types of dementia too, but in posterior cortical atrophy these are the first and most severe symptoms.

People tend to develop posterior cortical atrophy at a relatively young age, in their mid-50s or early 60s. We don’t have exact numbers but there are about 42,000 people in the UK with a form of dementia that started before the age of 65, and of this group only a small proportion are likely to have posterior cortical atrophy.

Raising questions about posterior cortical atrophy

From a scientific point of view, there’s an interesting question that could be answered with research into posterior cortical atrophy.

People with posterior cortical atrophy have the hallmarks of Alzheimer’s disease in their brain (clumps of toxic proteins called amyloid and tau) but these clumps are found in the back of the brain.

In Alzheimer’s disease, these toxic proteins are found in the memory centres, which is why memory loss is one of the main symptoms associated with this condition.

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The reasons why Alzheimer’s disease can affect different parts of the brain in different people are not understood. There must be some genetic, lifestyle, and/or environmental factors that cause the visual areas to be vulnerable or the memory centres to be protected in some people.

Finding out what these are could give us important insights into Alzheimer’s disease.

It’s also important to improve our understanding of the symptoms that people with posterior cortical atrophy experience so that we can support them better.

Some of these symptoms might seem quite unusual, for example, some individuals have a disordered sense of balance and they might experience dizziness or lean to one side.

Alzheimer’s Society has given research funding to Professor Sebastian Crutch at UCL to investigate these balance problems. We hope the project will improve understanding, detection, and management of these more unusual symptoms.

A progressive condition

As with other forms of dementia, there is no cure for posterior cortical atrophy, and how quickly the symptoms progress depends on the individual person.

Treatments that work for Alzheimer’s disease may help relieve symptoms for some people with posterior cortical atrophy, but over time the disease progresses to affect more aspects of thinking and more aspects of everyday life.

Terry Pratchett grappled with the issues around the later stages of Alzheimer’s and discussed them in the years following his diagnosis.

The period when someone with dementia is approaching the end of their life is difficult and emotional. People with dementia have the right to dignity and a ‘good death’ and people may wish to discuss these issues in different ways and at different stages.

For those who wish to find out more, there is information about end-of-life care on our website.

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31 comments

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Hi
My father in law has been diagnosed recently with PCA ,but deterioration is massive, from week to week is getting worse. I work in care system and look after many people how have mental illnesses dementia alzheimer's, but have not seen anything like that.its scary and we all feel helpless.

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Hi Ella,

We're really sorry to hear about your father in law, it sounds like you and your family are having a very difficult time.

We would strongly recommend speaking with one of our dementia advisers, by calling the Dementia Connect support line on 0333 150 3456. They will be able to listen to you, and provide advice and support specific to your family's situation. (More information about the support line, including opening times, can be found here: https://www.alzheimers.org.uk/dementia-connect-support-line)

You might also benefit from connecting with other people affected by dementia within our online community, Talking Point. Here, carers and other people affected by dementia share stories, advice, and offer support to others who may be going through similar situations. You can have a browse of other people's experiences, or sign up for free here: https://www.alzheimers.org.uk/get-support/dementia-talking-point-our-on…

We hope this helps, Ella.

Alzheimer's Society blog team

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Hi Ella,

My family member has just been diagnosed too and we are experiencing the same. Heavy week to week deterioration. I find it hard to connect his diagnosis and symptoms with other as his seems to be extreme.

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I was diagnosed with it the same time as Sir Terry Pratchett. I had all the scans and was diagnosed with Alzheimers. The most frustrating thing I find is that none of the specialists were able to tell me what to expect as they said everyone is different. I asked if anything would help and they said not really. However I started doing daily quizzes and stopped watching soaps and changed to anything informative. History, Discovery, Biographical, Detective series etc that gave you chance to think and try and work out who did it before they told you.
I am not saying that my symptoms slowed down or stopped but I do feel trying has definitely helped me. I read a lot and embraced social media to some extent.

One thing I was totally opposed to was joining clubs or going to sessions with others also afflicted. I suffer with mental health problems like depression and frustration but have to try and control it. I have tried mental health services but I have to say that their involvement was minimal, 2 visits and they stopped coming. When at my lowest I was told try not to think of the negatives and concentrate on your achievement raising a family etc and that was it, one session and a follow up phone call and then they signed me off. My children are all grown up and have their own lives to lead so interaction is probably minimal except for one daughter who I see almost every day. I live with my son but he has his own problems and we can clash. I am hyper critical to the extreme and his sometimes expects me to do more but physically I am unable.
I think spending most of my time in my room with my dog allows us to rub along. I have other major illnesses and think how easy it would be to give in but my psyche is to say Fxxx It, it will not win. It will not belittle me and when I do go I will have fought it every step of the way.

I do think though that it is a shame that more time couldnt be given to support us, even if it was just to allow us to talk and get things of our chest. My advice to others is decide what you are going to do about it. It is to fight it do it with resolve and fight every step of the way

I know it will get me but until then ve day to day. Start every day as if it is your first. Appreciate the little things

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Hi my husband was diagnosed very early on with PCA, about 8yrs ago. I changed his diet and life style. Whether this slowed it down not sure. However, he struggles with word search and stammers. His emotion can be extreme to me, when he crys he sobs , its heart breaking and when he laughs way over the top. He has no filter when saying things. If he's thinking something he just says what he's thinking and some are very inappropriate. Wondering what stage of his illness he is at.

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My husband is 67 and was diagnosed with PCA 4 years ago. My heart is breaking snd I’m scared too death. The comments on this page really help me. Thank you

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With my pca i only have breakfast each day,, have done for months,, just dont feel like eating,, dont lose weight,, drinking ok,, but if i force myself to eat i get stomache pains. I feel full all the time. I do get pains at the back of my head,,, its just a terrible thing.

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My Husband Bryan has PCA he's had it for 6 years and is 58 .Everything is upside down and back to front , he can't dress himself and a lot of other things but he has just started flinching .He does it when he's awake and asleep quite a lot .It started a couple of weeks ago .

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Hello Sally,

Thanks for getting in touch. We're really sorry to hear about the recent changes you've noticed in your husband's symptoms. It sounds like you're going through an especially tough time.

Please know you can speak with one of expert advisers on 0333 150 3456. They will listen to the situation and provide information, support and advice: https://www.alzheimers.org.uk/dementia-connect-support-line

We hope this is helpful, Sally.

Alzheimer's Society blog team

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Hi my husband is 52 now and was diagnosed at 50 with early onset after several years of being told he has clinical depression, after reading about night blindness I think he has PCA. The reason I’m interested in your post is that he is flinching and has been doing this for some time I have asked about this often and been dismissed, with thing like he’s dreaming, while he eating? Have you any idea what the flinching means?

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Hello Lorraine, thanks for getting in touch.

Our online community is the ideal place to talk to others going through a similar situation. If that appeals to you, take a look at Talking Point, where you can share your experiences and ask questions of the community, or just read what other people affected by dementia have to say: https://forum.alzheimers.org.uk/

You may also benefit from speaking with one of our dementia advisers on 0333 150 3456 if you have questions about different symptoms of dementia: https://www.alzheimers.org.uk/dementia-connect-support-line

We hope this is helpful, Lorraine.

Alzheimer's Society blog team

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My wife was diagnosed with PCA 3 years ago but had been experiencing difficulties and symptoms 3 years before that. She has been twitching/flinching as well for the past 9 -10 months and it is now getting worse and more pronounced. We have been in our home for 22 years and she finds problems now finding her way around and also has problems dressing/undressing.

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My husband has PCA and has tic like movements especially in his left arm which is his affected side. Seizure meds reduced the major jumping of his arm. He still has fine tics in his fingers.

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My wife has been diagnosed with PCA yesterday. She is 62 years old and her Geriatrician has recommended her to use Exelon patch(9.6mg). Does this help?

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Hi Alex,
I'm really sorry to hear about your wife's diagnosis.
We have some information on our website which you may find useful. You can read about drug treatments (including Exelon) here: https://www.alzheimers.org.uk/about-dementia/treatments/drugs/drug-trea…
If you need any further advice and support, remember you can always call our helpline. Please find the details here: https://www.alzheimers.org.uk/get-support/national-dementia-helpline
Hope this is helpful
--
Alzheimer's Society blog team

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I was first diagnosed with frontal temporal dementia in 2016 at the age of 56. About 6 months ago they decided that although most of my right frontal lobe and parietal lobe have died off I was not exhibiting the symptoms that would have gone along with this diagnosis. In December of 2018 I had another of several MRI'S and during my appt with my neurologist he told me that I had PCA. If anyone has documented the symptoms of someone who has suffered through this horrible disease could you let me know. Not knowing is the worst part.
Thank you, Angela

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Hello Angela, thanks for getting in touch.
I am sorry to learn about your recent diagnosis of PCA.
I hope that I will be able to provide you with some information which may help alleviate the difficulties of not understanding your diagnosis.
Posterior cortical atrophy (PCA) is a form of dementia where damage is focused at the back (posterior) of the brain, the region responsible for visual processing.
In nearly all cases the underlying cause of cell degeneration is amyloid and tau pathology, the same as in Alzheimer's disease. PCA is thus often called an 'atypical Alzheimer's disease'.
Early symptoms of PCA are problems with vision, performing skilled movements and literacy skills. Unlike typical Alzheimer's, someone with PCA will initially have well-preserved memory. Later stages of PCA progress to resemble typical Alzheimer's disease.
PCA tends to affect people in their 50s or early 60s. They are often referred to an optician before seeing a neurologist. PCA is rare (five per cent of all cases of Alzheimer's disease) and is often slow to be diagnosed.
Drug treatment is as for Alzheimer's disease. Practical visual aids such as talking clocks and watches can help, as can talking books and audio recordings available on CD or online.
I have attached a link to a page on our website which includes a signpost to a PCA support group which I hope that you will find helpful.
https://www.alzheimers.org.uk/about-dementia/types-dementia/Posterior-c…
I do hope that this has been helpful. Please don’t hesitate to contact our Helpline on 0300 222 1122 if you require any further information, advice or support.
You may also find it helpful to visit our online community 'Talking Point', as other people may have discussed similar issues. You don't have to sign up to search through the forum, so you could just read other peoples' posts and find out about their experiences. The forum can be found at: https://forum.alzheimers.org.uk/
Kind regards
Helpline Adviser

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My husband is in nursing home after stay inhospitable with urinary infection. I have just found out that he was diagnosed in 2004 with Dementia 22. he was not allowed home as it takes 3 people to change him.He cannot talk feed himself is doubly incontinent. He cannot stand.Are these later stages of this ?

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Hello Elizabeth, thank you for your comment.
I am sorry to hear of the change in your husband’s current condition.
The symptoms that you have highlighted such as an inability to feed himself, incontinence may be synonymous with the later stages of the dementia. However, this is not finite. A medically trained professional would be in a better position to carry out a comprehensive examination and assessment of your husband in order to rule out any other possible causes for these changes, such as further infection.
I have attached a link to factsheet: The Later Stages of Dementia, which looks at the the progression of dementia and symptoms in the later stages.
https://www.alzheimers.org.uk/sites/default/files/pdf/factsheet_the_lat…
Talking Point is an online community run by Alzheimer's Society that provides valuable support, tips and advice to anyone affected by dementia, this includes family friends, carers as well as people with dementia. Some people have found this to be a useful support: https://forum.alzheimers.org.uk/
Should you wish to discuss any of the above please do not hesitate to contact National Dementia Helpline. Telephone: 03002221122; Email: [email protected]. Opening times: Monday to Wednesday 9am-9pm; Thursday, Friday 9am-5pm; Saturday, Sunday 10am-4pm.
Kind regards
Helpline Adviser

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My mom, 76, was diagnosed with PCA. Can someone please give me an idea of what to expect? Will she forget who I am, will she need full time care, will she die???

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Julie: I am so sorry about your mother. This is a very challenging diagnosis. My husband died one year ago at age 64 of PCA. I live in the US. I kept notes over the course of his progression (18 months) that you might find helpful in adjusting. It was entirely trial and error (many minor adjustments were a big help), but perhaps my solutions might help you with your mother. I was able to manage very him well at home until his death.

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I have a client who is 64 with PCA. He was diagnosed 6 yrs ago and recently took a huge decline. Any info is helpful. He is having hallucinations now.

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Mary,
My husband was just diagnosed with PCA about 3 weeks ago. He had cancer 2 years ago and we thought he had chemo brain, so this was a shock. Can you please tell me what to expect as we progress through this?

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Hi Kathy,

We're really sorry to hear about your husband's recent diagnosis. This must be such an emotional time for you. Please know that support is available and we are here for you both.

You may find it helpful to talk to one of our Dementia Advisers for information, advice and support. You can call our Dementia Connect support line on 0333 150 3456. Find more information about the support line, including opening times, here: https://www.alzheimers.org.uk/dementia-connect-support-line

You might like to talk with other people affected by dementia within our online community, Talking Point. Feel free to browse and read others' experiences, or you can create an account to reply and connect with others who may be going through a similar situation: https://forum.alzheimers.org.uk/

In the meantime, you can browse through a number of pages on our website related to PCA, including real stories and experiences: https://www.alzheimers.org.uk/categories/types/posterior-cortical-atrop…

I hope this is helpful, Kathy. Please do call the support line if you need us.

Alzheimer's Society blog team

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With PCA Valdeana is disabled but we still have Valdeana with us.

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My wife has been struggling with PCA for some years, although she was only diagnosed in 2015 at the age of 60, following a MRI scan showed her Right Parietal Lobe had all but gone. Prior to that her diagnoses was Mild Cognitive Impairment but as she said "there was nothing 'mild' about it for her"! Valdeana had dealt with visual and memory issues since the turn of the millennium, being diagnosed with a Holmes Adie Pupil in 2000, and if I read through the literature I can see minor incidents as far back as the mid 90's. Although we have been plagued by it's consequences for our careers, finances and health, I do feel that it's slow progression has allowed her brain to adapt, considering the total loss of the Right Parietal Lobe and 70% of her left Hippocampus I remain astonished by how she manages with the disabilities Alzheimer's inflicts. Today Valdeana is at daycare, to allow me to work part-time and time to deal with those things I can't deal with when being her carer. Many people who have family at the same Daycare do not, initially realise Valdeana is a suffer. What I would say is that Valdeana is still herself, just with a lot to deal with in her disabilities. She remains my fiery little Red-Head with a mind of her own. And for that, I'll always love her.

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that is so sad and so tender. Thanks for sharing your thoughts. Your redhead sounds like a real cracker!
x

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On line there is a report by a Dr Mary Newport of Tampa , Florida. To the effect that Altzimers is a kind of diabetes of the brain whereby the brain cannot absorb glucose, but that taking pure Coconut oil enables the brain to digest the glucose via a substance called tryglissarines (may have misspelt that) and that there is a Professor Ceiran Clark at Oxford
University working along the same lines, has anyone heard of this and is there any sort of trial going on,

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Hi Peter, thanks for your comment. We have a page on our website about coconut oil here which will give you some more information about this: https://www.alzheimers.org.uk/info/20074/alternative_therapies/119/coco…

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My sister has PCA and is now in the latter stages of the disease. The development of her disease was completely different from Alzheimer's. This caused her and us much distress in trying to help her deal with her disease. When she needed full time care, we were unable to find a care home which understood her condition and she and we were frustrated by the lack of awareness. She is now in a dedicated working age dementia home. I only wish we could have found it earlier. I am so glad that there is now funded research into this condition. If we can help in any way, please contact me.

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Dear Lesley, my sister has been diagnosed with PCA a year ago. We have always had a tricky relationship in that from a very early age she resorted to violence when she felt she couldn't be understood. when she finally told me of her diagnosis, she was distraught (understandably) but wanting to make our relationship work again. Prior to her telling me I had already tried several times, and was apprehensive but glad that wanted to do the same. I subsequently tried calling many times and got nowhere. Finally, through her husband, we managed to arrange a zoom (we are in different countries). She had forgotten that she called me. She had forgotten wanting to try and mend our relationship. She was furious for having to be on the call and gave abrupt one word responses before raising her voice to ask 'why now'. The following day, my husband received a call from her to say that she wants nothing from me and that she has no sister. It is heartbreaking for sure that she has this disease at such a young age. It is equally heartbreaking however that it is nigh impossible to know from afar whether the anger is the anger she always had in her or anger brought on by her condition, or is it indeed possible that she has had some form of this disease for much longer than anyone is suggesting? I for one am very confused as to what I can do. Being physically present in 2020 is not an option, not to mention she has a history of attacking me quite badly which I have no reason to believe has changed. I am struggling to try and identify what are PCA symptoms in her and what are those personality traits I have witnessed in her all our lives. I do not see anyone else speaking of violence as a symptom here, but perhaps it is a combination of this disease affecting everyone differently and not wanting to mention violence in this context. I am very confused.

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