WHAT IS POSTERIOR CORTICAL ATROPHY? (aka BENSON’S SYNDROME or PCA)
I have created this web page about Posterior Cortical Atrophy (Benson’s Syndrome), a rare, neurodegenerative, terminal brain disease, in an effort to help those, like myself, whose loved one has received this horrible diagnosis.
When my wife was finally diagnosed with PCA, we’d been struggling for years with the initial symptoms. The GP visits, subsequent brain scans, and finally the visits to a geriatrician, which brought us to this devastating diagnosis, all happened at least five years AFTER the initial onset of symptoms.
There is no treatment and no cure. Some people suffer with symptoms for a decade or more, most die within five or ten years of diagnosis. Most people are diagnosed in their fifties.
Because this disease is rare and virtually unknown, and because it creeps up on its victims slowly, by the time a diagnosis is made, years have gone by during which the victim and their loved ones could have been helping and planning.
It would be too easy to say “abandon all hope ye who enter here”, but it would be fitting. If you or your loved one has been diagnosed with PCA, gather all the help and strength you can get your hands on, you are going to need it.
LINKS TO INFORMATION ON PCA
Stages of Posterior Cortical Atrophy
Scientific and anecdotal information on PCA gathered off the internet in 2020 (79 pages, PDF 45MB)
News stories about people and families struggling with PCA (31 pages, PDF 3MB)
PCA and DEMENTIA
PCA (Posterior Cortical Atrophy or Benson’s Syndrome) is a disease that initially attacks the back of the brain, and eventually, the entire brain. The cause is not known, but is believed to be the same plaques and tangles that cause Alzheimer’s. The belief is that the plaques and tangles form in the brain cells, and the cells die off. This loss of brain tissue is called cortical atrophy. In a PCA victim, the brain cells are dying, the brain is withering.
DEMENTIA is the symptom caused by the atrophy. Dementia itself is not a disease, dementia is the result of any damage or abnormality to the brain. Dementia can be caused by, but not limited to, strokes, head injuries, brain tumors, lack of oxygen to the brain, abnormal brain chemistry, loss of brain tissue, etc. Dementia presents at any time the brain is unable to function properly. This covers a vast area of human functioning. The person with PCA will exhibit signs of dementia. Over time the dementia will get worse. Eventually the person will lose all ability to function.
OUR PCA STORY (written in June 2021)
In 2015, my wife and I were living in New York City. We were both 53 years old. Our daughter was in year 2 at the local elementary school. My wife, O, was working as a waitress at a successful restaurant and volunteering at the school. I was trying valiantly to maintain a career as a fine artist, while working a string of odd jobs, something I’d done my entire adult life. At this particular point in time, I was unemployed, unable to sell artwork, and was busy selling everything we’d ever owned, in order to make ends meet. We were renting (NYC rents at that time were astronomical, and rising rapidly), and over the course of the decade, I’d depleted all my life savings, my 401k, etc. We were broke.
It was during this financially difficult period that O’s first symptoms appeared. I was born and raised in the United States, O is from Australia. In May of 2015, O’s mother passed in Australia, and we were unable to attend her mother’s last days, or to attend any memorial services. It was these traumatic circumstances which had caused O to be confused and unable, at times, to function properly. That is what I thought. Little did I know she was at the beginning of a long, horrible battle with PCA.
These initial incidents were;
O walked into a closed door.
O could not always remember the names of some of the parents, or teachers, from the school.
O could not always remember where places were in the neighbourhood, or how to get there.
I feared she might lose her way on the subway. Her ability to use an iPad or computer were getting worse, and she could not master the new technology that was being implemented at her restaurant job. Again, I chalked this up to stress, depression, or just a sheer inability to learn new tech at age 53.
In September of 2015, we folded our tent in NYC, and went to live with my elderly mother and father. O’s symptoms became worse. Still, I figured it was because she didn’t like being in a strange house, in a strange town, and that’s why she was acting as she was. At my parent’s home, there is no public transport, a car is a necessity. I tried to teach O to drive (she’d driven a car earlier in her life in Australia), but it was useless. Again, I figured “mid-life crisis”. She doesn’t want to learn, and so I backed-off. Thank god, because little did we know, she was developing a serious brain disease.
We had left NYC for the refuge of my parent’s house, but the plan was always to ultimately get to Australia. In January of 2016, 3 months later, we migrated. The 3 of us lived in a spare bedroom at my sister-in-law’s house in Adelaide. During this time, more symptoms appeared. O could not always get the words she needed when relating events, events either from the past or from the day before. We were fortunate to obtain a car from my brother-in-law, and I drove. Again my attempts to teach her to drive failed. O had difficulty doing simple things around her sister’s house, like using doors, opening windows, or using the coffee maker. I attributed it to new and strange surroundings.
In April 2016 we scraped together enough money to move into our own flat. O began a government works program, where she took classes, applied for jobs, and was given a benefits allowance. I applied for a permanent visa, and began searching for work. I’m not sure what the people at the jobs program thought of O. It was clear to me that her reading and writing skills were poor. We had met in 2005, both aged 43, so we had not known each other as young adults. I knew O had only completed year 10 of high school, and so I figured her difficulties reading and writing were due to a literacy issue, or low IQ issue. Little did I know her ability to read and write was slipping away due to PCA.
In 2017 O had an episode of “Bell’s Palsy”. One side of her face drooped, and we thought she’d had a stroke. After several months, and a course of meds, it went away. O was awarded a position at our daughter’s primary school, helping out in return for a government allowance. I was awarded an “artist-in-residence” at a local gallery, an unpaid volunteer position. Things seemed to be looking up. O and our daughter went off to the school each day on foot (our old used car had died and been sold off to scrap). I walked to the gallery every day. We were a desperately poor, somewhat functional, almost happy family.
I can’t lie, those were difficult years. I gave up my art practice in Sep 2018. I was constantly angry that O was so apathetic and careless. I kept having to pick up the slack, and I resented it. Looking back, I will always cherish those days. For 3 years we ignored O’s “daftness”. We went about our lives without knowing we were doomed.
GETTING A DIAGNOSIS
Towards the end of 2019, I noticed O’s usual “daft” ways had taken a serious turn. During that year, she would constantly ask me what day it was, or what time it was. I would angrily direct her to the clock or the calendar. I didn’t know why, and she did not know how to explain why, she couldn’t “see it” or “find it”, let alone read it and understand it. In January of 2020 I became so alarmed, I wrote a list of things she couldn’t do properly. When I saw the list, an entire page long, I panicked. She must be losing her mind, I thought. Brain cancer? Mental breakdown? Was it the Bell’s Palsy? We had a big argument over the list, and so I let it be. Her job at the school was everything to her. Why risk it? We let it be.
In August of that year, O and I were both 58. Our daughter was in year 8 and getting top marks. One day the school principal called O into his office and told her to go see a doctor. He told us that O’s behaviour was not suitable for her to continue at the school, and until we sought medical advice and treatment, he could no longer continue to employ her. Whilst O had done nothing wrong, he feared her confusion and sight problems might cause an accident.
This was the alarm bell, the wake-up call. We could no longer pretend “nothing was wrong”.
We immediately went to a GP. I explained O’s “cognitive decline”. The GP probed us about family issues, drug and alcohol use, and O’s previous employments. Then she gave O a cognitive test, which O failed miserably. We were sent for a CT scan.
The CT scan results said, and I quote;
“ventricular and sulcal prominence is indicative of volume loss, more than expected for age, and particularly involving the occipital lobes. Possible calcified meningioma, left parasaggital location”
A second visit to the GP, and we were sent for an MRI.
The MRI results said, and I quote;
“image findings are entirely consistent with posterior cortical atrophy. Occipital volume loss on CT, possible left meningioma. There is a quite striking discordance between the sulci of the occipitoparietal regions and the frontotemporal regions. Jagged cortex with volume loss in both grey and white matter in both occipital and parietal regions is evident. This is the typical appearance of posterior cortical atrophy (Benson’s syndrome)”
We were referred to a geriatrician, even though O was only 58, and he conducted further cognitive tests, during which O displayed her now constant inabilities. We were sent for a SPECT scan.
The SPECT scan results said, and I quote;
“marked posterior hypo-perfusion is shown. This affects the parietal, occipital and posterior temporal lobes. Perfusion to posterior cingulate/pre cuneus is markedly decreased on the right, and mildly decreased on the left. Scan findings would be compatible with posterior cortical atrophy”
Despite the closure of finding out what is wrong, despite the minor relief at finally knowing what is causing the strange behaviour, any sense of relief one gets from solving the mystery is quickly destroyed by what they tell you next;
There is no treatment. There is no cure. It just keeps getting worse, and then you die.
OUR NEW LIFE AS PCA VICTIMS
To say that the rug has been pulled out from under you is an understatement. The future, whatever you thought it was, has been replaced with the capital punishment prison sentence of PCA.
Thank god for our teenage daughter. She is the light that shines through the darkest hours.
In the weeks following the diagnosis, we were struck down. We couldn’t get off the sofa or do anything. It was beyond depressing, we were stunned. A blackness descends and it is overwhelming.
If you find yourself in this situation, get help. Grab onto anything, the tiniest thing, and count its blessing.
As you alternately rail against the unfairness of it, and crumple under the reality of it, you will undoubtedly ask “WHY US?!”
O WAS A FIT AND HEALTHY INDIVIDUAL.
She had a good diet, no junk food. She never smoked cigarettes, drank alcohol, or did drugs. She was active. Unlike an alcoholic, chain smoker, junk food couch potato, or drug addict, she did not bring this on herself.
SHE DOES NOT DESERVE THIS.
This is one of the hardest aspects of the diagnosis to deal with.
We were fortunate in our journey of diagnosis. Our GP had sent us for a brain scan right away, and the radiologists knew what PCA was, and how to identify it. I pursued both financial and care aid, and we were granted both. The Australian government and health care system, Dementia Australia, and O’s family and friends, have been above and beyond in their response to our crisis. I cannot praise them enough.
Our relationship had always been one of O working, while I held down the household, and chased my art jobs or other jobs. I had always been the “admin” of the household, and so I continue to do so. Doing the laundry (at a laundromat 3 blocks away, with no car), grocery shopping and cooking, cleaning, managing the bills, and overseeing our daughter’s school stuff, was always my domain, and so we are fortunate in that O’s disability did not rob our family of those crucial supports.
If this is you, be prepared to do more. A lot more. Caring for a person who is slowly losing their abilities is a lot harder than it might sound, especially if that person is your life partner. Whatever your career, hobby, or life’s work outside of the home, will be curtailed, or cancelled altogether. The outside world will not be aware of your loved one’s mental handicap, and will fail to give way, show patience, or lend a hand. You must learn to forgive people for thinking that the person with PCA is careless, rude, drunk, or possibly mad.
My sense of privacy and dignity will not allow me to describe the daily details of O’s disability or how we manage it daily. Suffice it to say, the person suffering from PCA will slowly lose the ability to function. You may have to help them dress, eat, walk, bathe, and toilet. If you are not up to this, get help, and place your loved one in the care they deserve.
The person with PCA will also become apathetic, depressed, and/or angry, as will the loved one who is caring for them. Don’t let this monster swallow you.
If doctors, insurance companies, government agencies, and health care workers are not helping you, be assertive and persistent. We have been helped, but I chased it all down. Reach out. Be pro-active. Don’t wait. Nobody’s coming to the rescue. Nobody cares.
Everybody’s sense of privacy and dignity is different. At first O did not want to believe the diagnosis, then she did not want to tell anyone. If you want help, and understanding, and even sympathy, you need to reach out. We alerted immediate family immediately, but have been slow to open up to the network of older friends and acquaintances whom we don’t see regularly. This has the effect of drastically closing down our social circle. The isolation is inevitable, as you will find that the carer job is a never ending, 24-7 shift.
This disease does not give you a day off, not even a coffee break.
GET HELP
Once you realize the severity of this disease, and what it is doing to your loved one, you must quickly decide if you are going to be the carer, or let someone else do the heavy lifting whilst you get on with your life. Because the PCA sufferer needs constant care. They can no longer function independently.
I have chosen to take this on. I do not like the idea of O being in a care home where nobody really cares about her, they are just doing their jobs. I do not like the idea of our family being split up. Our daughter is having to grow up a bit quicker than she might have, because she now must help me take care of mummy. This is our situation, everybody’s is different.
At the time I write this, we have been fortunate enough to have a govt. supported carer come twice a week (soon to be 3 times a week), who takes O out of the house and in to the community for 3 hours each time. Even this small amount of help is like a pile of gold, once you’ve been doing the carer thing 24-7 for 9 months.
I don’t know if it was a good or bad thing to have this hit us during the covid pandemic. Having a loved one who requires 24-7 care is like being in a lockdown anyways, and we were fortunate to live in a country that has no covid cases. At the same time, the general stress of the covid TV news cycle did not help during this time.
We have also utilized the services of Dementia Australia. Whilst they do not provide actual hands-on care, they are an invaluable source of information and support networking. To date we have attended one DA support group at which we met another “young onset dementia” sufferer, with whom O has created a friendship. I am also attending a “carers” support group conducted by DA.
One thing you will quickly realize, is how rare PCA is. The “young onset dementia” victims are few and far between. In the groups I have attended, we were the only people who were in our fifties and still raising a teen at home. Despite this, the groups are invaluable. The social connection and the “group therapy” you will derive from sessions like these are necessary for your survival. It is too easy to feel, after the doctor visit, or the group session, or when the support worker’s shift has ended, to feel as if the whole thing is now a candy coated turd. You must fight this “no light at the end of the tunnel” blackness. The cloud’s silver lining is tin foil now, you’ll have to lower your expectations, and readjust your view of the future. Or walk away all together.
THE PERSON WHO HAS PCA
I do not have the disease, O does. I cannot imagine how horrible it must be to not be able to see or to think properly. And since O has lost much of her ability to communicate, she does not like to go into detail about it. In this situation you try to make everything seem normal, even though it is not.
The person you knew is still that person, but you will interact in a different way. Gone are the types of conversations you had, replaced with the kind of talk you might have with a toddler or an extremely elderly person. Like the toddler, the PCA victim needs 24-7 care. They cannot navigate the world on their own, nor can they be left alone.
Over the past five years, O has lost these abilities;
- cannot read or write
- cannot tell time from analog or digital clock
- usually does not know what time it is, or what day it is
- cannot drive
- cannot use computer, iPad, or smart phone
- gets confused in any place other than the apartment
- cannot use public transport, or go to the shops without help
- is often confused inside the apartment
- cannot manage small tasks like getting a drink of water, making a coffee, cooking an egg, or washing dishes
- extreme difficulty with anything (i.e. cannot use hangers, or manage a chest of drawers)
- is often confused about who certain people are, except for me, and our daughter
- extreme difficulty finding words (aphasia)
- has trouble going up or down stairs
- cannot find anything in the apartment or anywhere else
- cannot understand complex conversation
- cannot have TV, movies or a mirror, as she misunderstands who is talking or who is in the mirror
- has difficulty getting in and out of a chair, a bed, or seats on the bus, a car, the doctor’s office
- needs help getting and eating meals
- needs help bathing, using the toilet, and getting dressed
- cannot remember when or how to take medications
Somebody must do all of this for the PCA victim. The person who does this obviously must be paid, or they will be forced to abandon the person, to go to a paying job. These decisions are part of the crisis one will be forced to navigate after a diagnosis of PCA. Life will not be the same, especially if you choose to be the full-time carer for the PCA victim. You will need to get supports in place. You will need to create a safe and healthy routine and stick to it. The dementia support group is always saying “it’s a marathon, not a sprint”. There is no trophy at the end of the marathon. Only the satisfaction of having provided the proper love and care the victim deserves.
PHASE 2 – “BPSD” hallucinations, delusions and hostile aggressive behaviour (written in April 2022)
The second phase of Orietta’s illness was something so horrible, and difficult, that no amount of Dementia Australia seminars, google research sessions or Geriatrician consults could have prepared us for it.
In August of 2021, we were a year since O’s diagnosis, and probably at least 6 years into the disease. We had finally got an NDIS plan sorted, which allowed a carer to come 3 days a week, 6 hours each time, to take O out into the community. Mind you, the carer does not do toileting, bathing or dressing. The carer had been engaged April 2021.
In August 2021, O started to act much more confused than before. She confabulated events either about myself, our daughter, the carer, or relatives/ friends. At first these “imaginary events” seemed innocuous enough, and O was able to be calmed and reassured that things were ok.
An example of such an incident was, O claimed I had taken one of her keepsakes, her father’s hat, and thrown it in the street, which I had not. Over a few days, this paranoid delusion escalated to the claim that I’d taken all sorts of her things and got rid of them.
This was distressing to all of us, little did we know this was the tip of the iceberg.
Over the course of August and September 2021, O became increasingly confused, paranoid, agitated and hostile. She began to wake up constantly in the night, with what we thought were bad dreams, but soon came to realise this was hallucinations and delusions brought on by the PCA.
At first it was that she’d wake up, and say;
– she’d cut herself and had to go to the doctor immediately
– she was upset that someone was messing with her teeth whilst she slept
– why were certain relatives/friends in our room, and why won’t they go home?
– she’d yell out “what’s happening?” or “shut the fuck up!”
She’d want to get up and go out at 3am… I’d tell her the time and she’d call me a liar… I’d get her to look out the window to see that it was the middle of the night, and she’d argue with me that it was daytime
She then began having auditory hallucinations, thinking a conversation was taking place, when none was.
She began seeing text in places where there was none. For example, she’d run her finger over her dress, and say “what does it say here? who is writing these things about me?” She would see text on all kinds of blank surfaces, the table top, the bed sheets, etc… always thinking there was something bad written about her there.
It came to a head in September 2021 when I woke up in the night to find she’d wet and soiled the bed. She’d also been ranting to the carer about paranoid delusional things. We got a phone consult with her specialist, he said take her to the ED, she’s got a UTI, and he’d arrange a scrip for anti-anxiety pills. We spent 10 hours in hospital, she was dehydrated, had a UTI and gastro infection. They pumped her full of fluids, antibiotics and psych meds.
After that, we learned to mind her water intake, and to do her toileting better, but she was going downhill with the BPSD (Behavioural and Psychological Symptoms of Dementia). She refused to take the prescribed tablets, Escitalopram, claiming we were trying to poison her. In October 2021 there was an incident where she was out with the carer, went crazy, and ran into the road. The carer quit on us.
We had another phone consult with the specialist, and he prescribed Risperidone. Again, she refused to take the tablets, and we were unable to sneak them into her food or drink. This had the unfortunate effect of her not eating much any more, and she lost a lot of weight.
During Nov 2021 – Feb 2022, her BPSD got so bad, we were dealing with the following;
– O running out of the flat and into the neighbourhood streets, ranting and raving
– O waking up several times a night, seeing malicious texts on the bed sheets, screaming that there were people in the flat, screaming that either myself or one of our friends/relatives had raped our daughter.
– Trying to get O to the pharmacy for her Covid jab, she cried and said we were taking her to a place where she would be forced to sign papers to give her daughter away, and I had to get our daughter out of school to take mum to the pharmacy
– Said the people on TV were saying horrible things about her
– Thought our reflections in the mirror were strangers in our home
– Twice she went through the flat, pushing things to the floor in a rage.
Her specialist, and the Dementia Australia counsellors, and my sister-in-law, all said to call the ambulance when she gets like that.
By late January, we were at our wits end. O was up at night yelling and screaming all the time. She pushed our daughter in the face, and said “you’re not you, you better go check in the mirror”… she threw a ceramic cup full of scalding coffee at me… then one day in February, when she was out with the NDIS carer, she attacked strangers at the mall. The carer brought her home, and O was screaming and yelling incoherent paranoid rantings, her eyes bugging out of her head, spittle at the corners of her mouth. We called 000.
In retrospect we waiting too long to take this step. I think I was afraid that if I called the ambulance during one of her psycho episodes, the police and medics would take her away from us, and put her in some horrible psych ward. It wasn’t like that.
The ambulance came with two, young, beautiful and capable female medics, who calmed her down, got some pills into her, and whisked her away to the ED. This was at 4pm. I went there that night at 8pm, and they would not let me see her, but a doctor did take copies of O’s medical file from me, and said I should go home and rest and let them handle it.
When I visited the next day, they let me see her, but only because they could not calm her down, and needed my permission to either restrain her or heavily sedate her. It was a horrible thing to see her being held down by a goon squad while she screamed bloody murder, but they gave her halperidol, and the next day she was a drooling vegetable.
She was in the hospital for 8 days, during which they figured out the correct drug cocktail. I went every day and could see the improvement. They had no TV in her room, and the mirror in the lavatory was covered. She had a room attendent 24/7.
I have to say this was the most harrowing experience of our life. The SA ambulance and hospital staff were top notch, and without their help we’d have not survived as a family.
On the day of O’s discharge, we had a huge meeting with what seemed like a dozen nurses and doctors, because she’d been “sectioned”, they had to all agree it was safe for her to return home. We were given a quantity of 3 different drugs which had to be administered 3 times daily in a specific regime. We were told it would be helpful to try for;
– no TV
– no mirrors
– no arguments or other conversations that might cause distress
– no unusual visitors, even family/friends
– keep all activities to a strict routine
Whilst O was in hospital, they managed to get another CT scan of her brain, and could compare the new Feb ’22 scan to the old Oct ’20 scan taken 16 months prior. The results said, and I quote:
“there is generalised cerebral volume loss, which is most pronounced in the occipital-parietal regions. There is prominence of the occipital horns of the lateral ventricles”
As I write this we are 5 weeks into our post-hospital existence, and O has been on the meds without ever refusing them. The medicines are Risperdal, Escitalopram and Oxazepam. O now sleeps through the night, eats with a good appetite, and has gained all the weight back that was lost in 2021. She is foggier and slightly more disabled due to the meds. We have to do the complete showering, toileting, dressing, and some help with eating, but the BPSD is gone. Let’s hope it stays this way.
We also bought a super king mattress, a special lounge chair, and a hospital table, to make sure she has maximum ease and comfort, as we were told that even small discomforts can cause BPSD in dementia patients. We have set her up with headphones and a streaming music account, and she will listen to and sing along to music for hours in this chair.
We also increased the number of carer shifts to 6 days a week, 6 hours each shift. We keep everything to a routine to lessen the confusion. Again, the carers will take O into the community, but are not qualified to do the “personal care” (meal prep, feeding, toileting, bathing, dressing, etc) and cannot do the meds. I must do it all of that. Mostly they make sure O gets outside for fresh air, exercise and minor community contact, whilst I get a rest.
We cannot stop the progress of this disease, or get rid of the confusion, but O is peaceful and happy now, and therefor we are able to do the work of caring for her.
Posterior Cortical Atrophy (Benson's Syndrome) 