Simulating Alzheimer’s (UK newspaper article)

This post may only be of interest to those dealing with dementia though certainly a similar “simulation” could be created for the non-dementing types of PSP.

In a British newspaper article from last Sunday, there’s a description of a simulation called the “Alzheimer’s Experience.” The company Care UK runs care facilities in Britain. It requires all of its staff to go through the simulation. The one-day simulation “gives staff direct experience of the dramatic effect that dementia and frailty can have on the senses.” The tasks in the simulated experience include:

* “The training starts with a communication task. [The participant] must read words on a piece of white paper written in a fluorescent yellow highlighter pen, which makes them hard to see.”

“Dementia makes it difficult to read whole words…patients cannot make out what they are seeing.”

* “Next, [the participant] is given a newspaper to read. A machine is switched on that emits a din of ‘white noise’ – a fuzzy hum that permeates the atmosphere among sounds of clattering, scraping chairs and doors opening and closing. [The instructor] constantly walks up to [the participant] and asks her endless, random questions.”

“This task simulates what it is like for someone with dementia who may suffer from a constant noise in their head that they cannot shut out, which is a common symptom… Talking, shouting and asking questions further confuses a sufferer – their brain finds it difficult to differentiate between all the different stimuli.”

* “A third task is called Questions And Answers. [The participant] sits in a chair trying to count from one to ten while [the instructor] bombards her with questions as she tries to keep counting.”

“This helps the carer understand-that it takes people with dementia-longer to process information.”

* “The final task, entitled Physical And Sensory Impairment, involves [the participant] wearing headphones emitting white noise, to emulate loss of hearing, and putting on goggles that blur her vision.”

“This simulates a combination of eye-ageing and dementia.” … ather.html

Now I know how alzheimer’s trapped dad in its cruel grip: Bucks Fizz’s Jay Aston on how dementia devastated her father
By Bonnie Estridge
Daily Mail (UK)
Last updated at 1:41 AM on 26th June 2011

Alzheimer’s accounts for about 60 per cent of the 750,000 British adults affected by dementia.

The disease attacks nerves, brain cells and neurotransmitters – chemicals that carry messages to and from the brain. It is a progressive condition usually associated with old age, but the cause remains unknown. It is estimated that more than a million people will suffer from it by 2021.

As Jay Aston walked down the aisle on her wedding day, she struggled to hold back tears. Her pleasure at seeing her soon-to-be husband, musician Dave Colquhoun, waiting for her at the altar was tinged with a deep sadness for the man who was about to give her away.

Jay’s father, Ted, looked dignified and handsome but he was lost to the grip of Alzheimer’s disease – the most common form of dementia.

‘Not long before my wedding in August 1999, Dad had swallowed disinfectant, tried to stab my mother and told her, my brother Lance and me that he would shoot us and then shoot himself,’ says Jay, 50, who found fame in the now reformed Eighties pop band Bucks Fizz.

‘When he walked me down the aisle, he had no idea what he was doing or why he was there at all. The disease had made him the opposite of the man he had once been, and to see what he had become was my worst nightmare.

I know it was his, too. He always had a horror of mental illness and, because he was so physically fit when he eventually went into a care home, it took years for his body to decline and he suffered a hideous, drawn-out death.’

Ted had been one half of Fifties husband-and-wife variety act Ted and Hilda Durante and was renowned for his quick wit. But in 1995 he started to display behaviour that was unlike his normal self.

‘He kept forgetting his gags on stage – he’d had the sharpest wit of anyone I’d met so it was really quite worrying,’ says Jay, who has a daughter, Josie, seven.

Jay’s mother put these lapses down to age – he was 70 at the time – but Jay disagreed and she and her mother fell out over Ted’s health. As time went on, Ted forgot that he loved opera and stopped listening to records, and he could not recall which band Jay was once a member of. It reached a point where he became a danger to himself and other members of the family. He started to drink heavily and would then forget that he shouldn’t be driving, so Jay reported him to the police.

‘I felt like a Judas,’ she admits. ‘But I had to do something. We tried to keep Dad at home (in Tatsfield, Surrey) with Mum but he would let himself out in the middle of the night and wander down the road. He’d lost all sense of direction and it soon got to the point when it became impossible to keep him at home.’

On Boxing Day 2004, not long after Ted and Hilda’s 52nd wedding anniversary-Ted was admitted to a care home. Jay says: ‘We were all devastated. I used to visit him and as time went by he got angrier and angrier – frustrated that his life involved just sitting in a room all day. Because he was so fit, it took a long time for him to deteriorate physically. Over five years, he went from being a strapping 13st man to a 7st shadow of his former self.’

Ted died of pneumonia in 2009. Today, Jay remains passionate about learning to understand the disease that tore her father’s life apart – and has agreed to take part in a unique training programme run by residential care specialists Care UK, which has 57 homes across Britain. Dubbed The Alzheimer’s Experience, it involves a series of tasks that simulate the sensory experiences of those suffering from dementia.

Everyone from senior executives to cleaning staff is required to take the innovative course, which aims to improve services by giving them a better understanding of what residents go through.

‘With the help of doctors and other dementia specialists, we have developed a practical, hands-on, oneday course that gives staff direct experience of the dramatic effect that dementia and frailty can have on the senses,’ says Maizie Mears-Owen, service design manager at Care UK.

‘When one’s cognitive ability is impaired, everyday activities such as following instructions can be confusing – and even terrifying. As well as memory loss, dementia can affect motor skills, which means walking and dressing become increasingly difficult. Jay is going to get an idea of what her father went through.’ The training starts with a communication task. Jay must read words on a piece of white paper written in a fluorescent yellow highlighter pen, which makes them hard to see.

Jay spends several minutes trying to decipher the words. ‘This is incredibly demanding,’ she says. ‘I could make out only 25 per cent of the words.’

Maizie explains: ‘Dementia makes it difficult to read whole words – because of deterioration in certain parts of the brain, patients cannot make out what they are seeing.’

Next, Jay is given a newspaper to read. A machine is switched on that emits a din of ‘white noise’ – a fuzzy hum that permeates the atmosphere among sounds of clattering, scraping chairs and doors opening and closing. Maizie constantly walks up to Jay and asks her endless, random questions.

‘This task simulates what it is like for someone with dementia who may suffer from a constant noise in their head that they cannot shut out, which is a common symptom,’ says Maizie. ‘Talking, shouting and asking questions further confuses a sufferer – their brain finds it difficult to differentiate between all the different stimuli.’

Jay remarks: ‘Towards the end, Dad was very frustrated at not being able to read a newspaper easily. He found it impossible, complaining about all the distractions. He would become angry and often started shouting.’

A third task is called Questions And Answers. Jay sits in a chair trying to count from one to ten while Maizie bombards her with questions as she tries to keep counting.

This helps the carer understand-that it takes people with dementia-longer to process information. Jay could also relate to this task.

‘Dad would respond to questions but then lose his train of thought,’ she says. ‘This, again, was frustrating for him so he would shout, get a headache or simply shut down and go to sleep.’

The final task, entitled Physical And Sensory Impairment, involves Jay wearing headphones emitting white noise, to emulate loss of hearing, and putting on goggles that blur her vision. This simulates a combination of eye-ageing and dementia.

She also wears thick gloves to take sensation away from her fingers, which have been taped together to simulate the difficulty sufferers have controlling their limbs and digits.

Dried beans are put in Jay’s shoes, making it difficult and painful for her to walk, similar to the difficulties faced by Alzheimer’s patients who have suffered nerve damage.

When asked to stack plastic cups on the table in front of her, Jay knocks them over and finds the task of pouring water from a jug into one of them virtually impossible, as are tasks such as doing up a button on her dress or brushing her hair.

Jay tearfully removes the goggles, gloves and headphones. Composing herself, she says: ‘It’s so upsetting to know what it was like for Dad living so long in this muffled, confusing world. I remember once he was trying to pour a drink and knocked the cup over. His clothes were wet but he couldn’t understand what had happened or why. It was heartbreaking, as he had been so strong.’

But despite the raw emotions that surfaced during the experience, Jay was pleased she had taken part.

‘It seems to me that this is a very good form of basic training, and any project that would help dementia sufferers be better understood has to be a positive, valuable one.

‘I wish I’d had this experience when Dad was still alive because it would have unlocked the secret of why he – and so many other dementia sufferers – undergo a change of personality. I’m also reassured to know that those caring for dementia patients now have such an insight.’

One patient thought to have NPH but had CBD

I found this abstract and the full paper a bit hard to understand. It seems that 10 patients were diagnosed during life as having NPH (normal pressure hydrocephalus), “characterized with a clinical triad of symptoms: cognitive impairment; gait difficulty; and urinary incontinence.” Each had biopsies during life to try to determine what sort of disease they had. The biopsies may’ve been performed during the placement of a shunt. All patients had brain autopsies upon death.

The main thing I’m unclear on is whether any of the 10 patients had pathologically confirmed NPH, or whether there’s even diagnostic criteria for pathologically-definitive NPH. I don’t think a single one was thought to have NPH upon brain autopsy.

But one patient had autopsy confirmed CBD and even responded to the surgical placement of a shunt in the brain. Here’s that patient’s info:

leading symptoms (prior to biopsy): gait difficulty
age at biopsy: 75
cognition at biopsy: moderate dementia
MMSE: 20
shunt response: improved
clinical diagnosis or symptoms: DM (diabetes mellitus), HTA (arterial hypertension), CI (cardiac insufficiency), psychiatric
symptoms 30y, balance disorder (sudden falls), hypokinesia, apraxia, apatia [Robin’s note: I assume this is “apathy”], ocular movement disorder

age at death: 81
cognition at death: severe dementia
post mortem neuropathological diagnosis: CBD
final cause of death: pneumonia

According to the article, there is one other study that included 38 NPH patients. One of those also had CBD upon brain autopsy.

That’s about it from this article. The abstract is below.


Neuropathology and Applied Neurobiology. 2011 Jun 23. [Epub ahead of print]

Post-mortem findings in 10 patients with presumed normal pressure hydrocephalus and review of the literature.

Leinonen V, Koivisto AM, Savolainen S, Rummukainen J, Sutela A, Vanninen R, Jääskeläinen JE, Soininen H, Alafuzoff I.
Department of Neurosurgery, Kuopio University Hospital, Finland, etc.


Neuropathological features of idiopathic normal pressure hydrocephalus (iNPH) are poorly characterized. Brain biopsy during life may help in the differential diagnosis of dementia but post-mortem validation of biopsy findings is scarce. Here we review and report brain biopsy and post-mortem neuropathological findings in patients with presumed NPH.

We evaluated 10 patients initially investigated by intraventricular pressure monitoring and a frontal cortical biopsy for histological and immunohistochemical assessment as a diagnostic procedure for presumed NPH.

Out of the 10 patients, eight were shunted and seven benefited. Until death, six had developed severe and two mild cognitive impairment. One was cognitively unimpaired, and one was mentally retarded. Three subjects displayed amyloid-beta aggregates in their frontal cortical biopsy obtained at the initial procedure. One of these patients developed Alzheimer’s disease during a follow-up time of nearly 10 years. One patient with cognitive impairment and NPH suffered from corticobasal degeneration. In six patients various vascular lesions were seen at the final neuropathological investigation. Five of them were cognitively impaired, and in four vascular lesions were seen sufficient in extent to be considered as causative regarding their symptoms.

The frequent finding of vascular pathology in NPH is intriguing suggesting that vascular alterations might be causative of cognitive impairment in a notable number of patients with NPH and dementia. Brain biopsy can be used to detect amyloid-beta aggregates but neuropathological characteristics of iNPH as a distinct disease still need to be discovered.

PubMed ID#: 21696417 (see for this abstract only)

“Gone From My Sight” – terrific online video+booklet

Many hospice organizations distribute the blue booklet “Gone From My Sight: The Dying Experience” by Barbara Karnes, a hospice nurse. (You can order a copy online from It’s a terrific booklet.  Barbara notes that “When you are at the bedside of a loved one who is dying, you don’t really see what’s happening.”  The blue booklet is all about what we don’t really see.

When speaking with local support group members, I often refer to sleeping 22-plus hours a day as a sign that death is getting closer. And I note that family members who are dying often start to withdraw from the ones they are closest to.

One conversation I have had many times with local support group members is the anxiety and terror they feel when their family member stops eating or drinking. People worry about the pain associated with “starving to death.”

This topic of “starving to death” is addressed head-on by author Barbara Karnes in a presentation on March 2011 to hospice workers with Heartland Hospice. The 90-minute presentation was recorded and edited; it aired on Community Television of Santa Cruz County. It used to be available on YouTube. It was one of the best resources I can think of for helping families prepare for the dying experience of their loved ones.

Barbara shares that she will never say to a dying person “It’s OK to let go.” She says: “I understand you have to go.”

Barbara believes that “dying is really the last act of living or the final challenge of living.”

I took extensive notes from the 90-minute presentation and shared this with many hundreds of people over the years.  In early 2019, the Barbara Karnes folks have asked me to remove those extensive notes from this blog post as they are generally Barbara’s words.  And the YouTube link is no longer working so we can no longer direct you there.

If you are proficient in the web archive, you can still find the old blog post.

And there are probably some good substitutes available on YouTube or on the Barbara Karnes website.



PSP: “what’s new?” – 5 types

PSP folks –
A review article was recently published on what’s new in PSP.  I don’t have access to this full article.  It largely seems to be a summary of research published in 2009 by Williams and Lees regarding the five clinical types of PSP.  The most common type is Richardson’s syndrome.  In the abstract, the first paragraph describes the symptoms of Richardson’s syndrome but doesn’t use that term.


Geriatrie et Psychologie Neuropsychiatrie du Vieillissement. 2011 May 1;9(2):191-201.

Progressive supranuclear palsy: what’s new?

Levy R.

Progressive supranuclear palsy (PSP) has been described as a clinical syndrome characterized by an impairment of voluntary control of gaze (supranuclear palsy), postural and gait instability, and behavioral and cognitive deficits including a frontal syndrome and psychic retardation.

However, in the recent years, at least four other clinical forms of PSP have been recognized: PSP-Parkinsonism, “pure akinesia with gait freezing”, PSP with cortico-basal syndrome, and PSP with speech apraxia.

PSP-Parkinsonism mimics the signs and symptoms of idiopathic Parkinson’s disease, including a significant reactivity to levodopa.

“Pure akinesia with gait freezing” is characterized by a difficulty of self-initiation of motor programs, usually walking program.

PSP with cortico-basal syndrome mimics cortico-basal degeneration (CBD) in that unilateral or asymmetric limb dystonia and apraxia are prominent signs.

PSP with speech apraxia is an isolated syndrome of progressive anarthria.

All these clinical syndromes are due to brain accumulation of phosphorylated tau protein. The differences in clinical expression within the framework of PSP can be explained by the differences in the topographical distribution of the lesions. PSP is considered as a primary tau disease (“tauopathy”) such as CBD and some forms of fronto-temporal lobar degeneration. At the level of neuropathology, the pattern of tau abnormal inclusions differentiates PSP from other tau diseases, but some overlaps are reported. Moreover, several of the clinical forms of PSP partially or fully overlap with the other tauopathies. As a whole, the emergence of new clinical forms of PSP challenges the nosology of tauopathies and our understanding of these diseases.

PubMed ID#:  21690028  (see for this abstract only)

Nature Genetics – June 2011 Landmark Study PSP Genetics

Here’s the wonderful abstract from the PSP Genetics Study Group letter in the journal Nature Genetics:

Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy

Günter U Höglinger, Nadine M Melhem, Dennis W Dickson, Patrick M A Sleiman, Li-San Wang, Lambertus Klei, Rosa Rademakers, Rohan de Silva, Irene Litvan, David E Riley, John C van Swieten, Peter Heutink, Zbigniew K Wszolek, Ryan J Uitti,    Jana Vandrovcova, Howard I Hurtig, Rachel G Gross, Walter Maetzler, Stefano Goldwurm, Eduardo Tolosa, Barbara Borroni, Pau Pastor, PSP Genetics Study Group, Laura B Cantwell, Mi Ryung Han, Allissa Dillman, Marcel P van der Brug, J Raphael Gibbs, Mark R Cookson, Dena G Hernandez, Andrew B Singleton, Matthew J Farrer, Chang-En Yu, Lawrence I Golbe, Tamas Revesz, John Hardy, Andrew J Lees, Bernie Devlin, Hakon Hakonarson, Ulrich Müller & Gerard D Schellenberg

Nature Genetics (2011):10.1038/ng.859
Received 29 November 2010
Accepted 16 May 2011
Published online 19 June 2011

Progressive supranuclear palsy (PSP) is a movement disorder with prominent tau neuropathology. Brain diseases with abnormal tau deposits are called tauopathies, the most common of which is Alzheimer’s disease. Environmental causes of tauopathies include repetitive head trauma associated with some sports. To identify common genetic variation contributing to risk for tauopathies, we carried out a genome-wide association study of 1,114 individuals with PSP (cases) and 3,247 controls (stage 1) followed by a second stage in which we genotyped 1,051 cases and 3,560 controls for the stage 1 SNPs that yielded P <= 10(-3). We found significant previously unidentified signals (P < 5 × 10(-eight)) associated with PSP risk at STX6, EIF2AK3 and MOBP. We confirmed two independent variants in MAPT affecting risk for PSP, one of which influences MAPT brain expression.  The genes implicated encode proteins for vesicle-membrane fusion at the Golgi-endosomal interface, for the endoplasmic reticulum unfolded protein response and for a myelin structural component.

The full article is available online at no charge here: