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.”

http://www.dailymail.co.uk/health/artic … 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:

female
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.

Robin

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.

Abstract

Aims
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.

Methods
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.

Results
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.

Conclusions
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 pubmed.gov 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 gonefrommysight.com.) It’s a terrific booklet. 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. The presentation was recorded and edited; it aired on Community Television of Santa Cruz County. It is available on youtube. It’s 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.”

The online video is one hour and 36 minutes long. The last 10 minutes are not as strong as the first hour and 26 minutes but it’s all wonderful information to help take the fearful unknown out of the dying experience. Note that you may need a handkerchief nearby when you are watching this.

I’ve copied below notes that I took from the video.

Robin



www.youtube.com/watch?v=PPx-qpos57g

Gone from my sight: discovering grief, loss, and the dying experience
presented by Barbara Karnes, RN, Hospice Nurse Educator
for Heartland Hospice
3/10/11

Community TV Santa Cruz
1 hour 36 minutes

“Dying,” the word, is really a misnomer. Dying is really the last act of living or the final challenge of living. Dying is part of life.

When you are at the bedside of a loved one who is dying, you don’t really see what’s happening.

Two stereotypes we all get from TV and movies:
1- A profound message is given by the dying person and then he/she immediately dies.
2- The dying person looks magnificent and dies with his/her eyes closed.

Reality:
1- We often can’t hear the words that dying people are speaking. And, if we can hear their words, the words don’t always make sense.
2- Dying people don’t look magnificent and, usually, the eyelids are partially open. If a family member closes the eyelids, he/she is often horrified to see the eyes open again.

We approach a normal experience of our lives unprepared. We don’t know how to die. We don’t know how to grieve.

There are only 2 ways to die – fast and gradual. Gradual death comes in two forms — old age and disease. This talk focuses on gradual death. When it gets down to days, hours, and minutes, we all die the same, even fast death. When it gets down to weeks, someone dying of disease may look a certain way. Someone dying of old age may have these same signs for months before death.

This body is not who we are. All this body is is a vehicle to get around in on this planet. We can get out of this vehicle just as easily as we can get out of our car. The problem is that there is no “Dying 101” course.

Dying from disease and old age doesn’t just happen. There’s a process.

There are lots of similarities between birth and death.

Most people dying from disease and old age do certain things at certain times.

We are going to die the way we’ve lived, according to our personality.

We have limited control over the time that we die. Beware of anyone that would put a number on how long we have to live. It’s impossible to say that someone has 24 hours, 3 weeks, or 6 months to live. Yet, that’s what Medicare asks us to do with hospice — put a number on how long someone lives. They are asking us to do the impossible. The closest you can get to determining to prognosis is months, weeks, days, or hours.

(19 minutes) For some of us, dying will be our best work. For others of us, it will be our scariest, most terrifying work. It will depend on how we deal with other challenges in living. If I have run away from other challenges while living, then I’m going to be in denial and run away from this final challenge. If I have been controlling and very much of a doer, then I’m going to be in charge of my final challenge. I’m going to have my DNR in place. I will have my obituary written. If I’m a protective spouse or protective of my family, I will probably not die with my family in the room. If I am an A-type personality, and if I don’t die of a heart attack, then I will develop a disease and I will die quickly. It would be intolerable for an A-type personality to be in bed for 3 months. If I’m a more laid-back person, then I may die more slowly.

We can use manipulation in our dying also.

If you are with someone when they die, it is because they wanted you there. If you weren’t there when someone died and you wanted to be, get rid of the guilt and take the love and protection that they gave you.

(25 minutes) Any time we do something new, we are going to be at least nervous, if not downright terrified. That’s normal.

It’s easy to be dead but it’s hard work to be alive. I’m not really afraid of being dead. I am really scared about from right now until I am dead. I would relax if I knew the date of my death and the manner of death.

Look at people told by an MD that there’s nothing that can be done, go home and get your affairs in order. They go home and it’s as if they died at that moment because they stop living. All their energy goes into this disease and dying.

We want to live the best we can within the confines that our body and disease have put us in.

Now, I’m going to talk about the signs of dying from disease. If dying from old age, add more time to these guidelines.

(28 minutes) 2-4 months before death from disease, these things will happen: (you don’t have to know the diagnosis)

#1 a person’s eating habits change. They are going to stop eating meat (beef, chicken, and fish). Soon it’s fruit and vegetables. Then it’s anything that requires energy to digest. Then, soft things only are consumed. (Americans can do ice cream almost up until the end.) Then, Ensure-type supplements only are consumed. Then it’s water and Gatorade only that can be consumed. Then it’s sips of water or Gatorade.

This is a normal, natural progression. The hardest thing for me to get people to understand is that it’s OK for the dying person to not eat. Always offer food but it’s OK if they don’t eat. The family often thinks the person is going to starve to death. Most people dying from disease or old age starve to death. That is part of the normal, natural way that we die.

(32 minutes) We have so much emotional involvement around food. Consider Thanksgiving, Christmas, Valentine’s Day, Easter, Halloween, July 4th — look at all the food. We socialize over food.

(35 minutes) We eat to live. If the body is preparing to die, it doesn’t want the energy and grounding that food brings. All by itself, the body will cut back and stop eating. You’ll hear “I can’t eat.” They want to eat for us. They see what it does to us when they don’t eat.

When you are weeks from death, nothing works right. The body starts to shut down. People often talk about a feeding tube at this time. If you start pouring in protein supplement, you are going to have more complications than benefit. Diarrhea, constipation, aspiration pneumonia. The body doesn’t want the food; it can’t process the food.

When the person isn’t taking in enough fluids for hydration, people say “oh, my goodness, we better get an IV. Mom will get dehydrated, and that would be a terrible death of suffering.” This is a myth. Someone not taking in enough fluids for hydration is days or a week or so from death. Now their body really doesn’t work. If you start pouring in all this fluid, the kidneys aren’t going to be able to process it so they won’t be pee’ing out the water. The water builds up and up, resulting in drowning to death. This is not a gentle way to die. Very painful as the skin stretches to accommodate the water. People feel like they are buried in concrete.

(39 minutes) Visual a dry sponge. It’s nice and light. Fill the sponge with water. Now the sponge is heavy. That’s how a person’s body feels when you are pumping them full of fluids and they aren’t pee’ing it out.

What is your death of choice? Most of us would say: close my eyes, go to sleep, and not wake up. When you become dehydrated, your electrolytes get out of whack and your calcium goes up. When your calcium goes up high enough, you close your eyes, you go to sleep, and you do not wake up. God gives us an anesthetic to get us from this world to the next. We will get an anesthetic if we don’t interfere.

(41 minutes) The normal natural way to die is starvation and dehydration. This is controversial. We now have research behind this view.

#2 a person’s sleeping habits change. A person will start taking an afternoon nap. Then it’s a morning nap. Then they are sleeping in the evening in front of the TV. Then they don’t get out of bed. Then they sleep more than they are awake.

When a person sleeps more than they are awake, their reality changes. The dream world becomes their reality. If pain isn’t an issue, you can go in and visit with them, and they will go back to sleep. They won’t know or care if they dreamt your visit or if you were really there. They may talk to and about things that we can’t see.

In the weeks before death, our loved ones who have died before us, come to help us get to the next world. We do not die alone.

There is a symbolic language of the dying.

(46 minutes) The dying person says “I’m so tired.” It’s a tiredness they have never felt before. They can’t even move.

People who are dying are a window to the other world.

#3 a person starts to withdraw from the world around them. It starts far away, and then it includes family members. They go completely within. They are letting go of their hold on this world, and they are building their place in the other world.

(50 minutes) A person gets completely withdrawn. Some dying people actually start pushing people away. The dying person thinks that if they start pushing people away, it won’t hurt so bad…they won’t feel as much grief. It’s easier to leave home if things aren’t perfect.

(54 minutes) In order for me to get on with my life, my mother had to die. I don’t think I’m any different from anyone else. We can deal with just about anything if we think that the outcome is a positive one. In our society, death is viewed as a negative outcome.

There are similarities between birth and death. We go through labor to enter the world and we go through labor to leave it. The difference is that the labor to leave this world is harder on us, the watchers, than it is on the person that is in the labor.

The death is this world is a birth in the other world.

(58 minutes) Some labor to leave the body is fast and some slow. There are three things that affect the length of our labor:
* physical pain. This creates a tension that locks us into our body. This is why the hospice philosophy is to medicate until death. The key to getting out of the body is to relax. If you are in pain, you are not relaxed.
* fear. This creates a tension that locks us into our body. Religion often does a number on us in regard to fear. If your religion teaches heaven and hell, and you think you are going to go to hell, you are afraid. Hospice’s job is to neutralize the fear that goes with death and dying. Hospice is focused on letting the family have a sacred memory rather than a frightened memory.
* unfinished business. A woman died as soon as the unfinished business was addressed.

There is no perfect family. We are all walking wounded. There is no perfect anything. We are all dysfunctional. When we are diagnosed with a disease, we fall into a heap because we’ve used up all of our coping skills during daily life. Hospice can help us stand up again and use our coping skills.

(1 hour 6 minutes) We can’t see ourselves as being dead. We can’t comprehend our own death. There comes a point when a person says “they were right…I’m going to die.” When the person accepts it, he looks like he’s dying.

Hospice does its best work when they can help the person live the best they can. And the family can be helped through this process as well. Hospice does its best work in the months before death. When the person is dying, hospice can only address pain management.

The key change that says that a person is dying (because he looks different) is when the person is sleeping with his eyes partially open and mouth open. It takes energy to shut your eyes and shut your mouth. The breathing may start and stop. Or the breathing may be puffing…now the person is agitated and restless

(1 hour 9 minutes) 1-3 weeks before dying, the signs that someone is dying include:

#4 person is agitated or restless. They are picking at their clothes. They are picking at the air. You can’t get them settled. Now they know they are dying. They may not share this. They say to themselves “if I lay down and close my eyes, I may never open them again.” And they are right. They are fighting sleep. They are fighting death. This restlessness can be lack of oxygen. More like it’s fear.

#5 Remember that nothing works right. A fever goes with dying. Nothing bad is happening. This is normal. They may have a fever, and then in 30 minutes they are cold and clammy. It all comes and goes because the lightbulb is flickering. Congestion may come and go, depending on how hydrated they are. At this point, you don’t get a suction machine. Such a machine only makes them more uncomfortable. This congestion is part of the normal way people die.

(1 hour 12 minutes) signs that someone has days or hours to live:

#6 mottling. The fingers, the toes, the heels are going to look bluish and dark, almost black. Purple blotchy ring around the knees – more advanced mottling. If the back is entirely dark and blotchy, that’s more advanced mottling. If the person has a heart condition, they can be mottled for months, and it doesn’t count.

We can deal with anything if we know what to expect. Our imagination or fantasy of what this experience is going to be like, is far worse than reality. If we understand the process, we can see that “mom’s doing a good job…she’s dying just the way a person dies.” Neutralize the fear. Nothing bad is happening here. Yes, it’s sad.

#7 Our normal blood pressure is 120 over 80. A dying person’s BP may be 60 over 40. Maybe the RN can’t hear the BP at all.

The pulse is fast, weak, thready, and irregular. You can’t even count the beats.

Our normal breathing is 16-24 times a minute. A dying person breathes short, rapid, shallow breaths.

All of this together is saying that the body is in distress. It’s working hard to keep going. The heart cuts back circulation to the arms and legs. That’s all mottling is — a cutting back of circulation.

Now they are pee’ing and poop’ing in the bed. The body is releasing.

(1 hour 16 minutes) signs that a person has minutes to hours to live:

#8 person is non-responsive. They can be moving or talking. If they are talking, you can’t hear what they are saying. Most people are just going to be lying there. Some will be moving and agitated. They are non-responsive to their environment. They don’t respond to touch or their name.

It’s not like the movies: you are non-responsive first.

#9 Breathing changes; it gets slower and slower. They are taking 6-8 breaths per minute. They start breathing like a little fish. All of God’s creatures breath like this before they die.

#10 Some people sit up and flop back down to die. Some people fling an arm in the air and then die. In most people, it’s not that dramatic.

Some people frown and grimace, as if to say “no, I don’t want to leave.” Following a subtle movement, there will be one or three long spaced-out breaths.

People can still hear you. Even if a person is non-responsive, encourage each person who is significant to the dying person to go in individually and talk to the person who is dying. Say what’s in your heart. You are going to talk about the good times and the difficult times. The dying person has been processing his life — what has this been all about? Say “I understand you have to go.” (It’ll never be OK for someone to die, but I can understand it’s going to happen.) We are helping that person let go. “We’re going to miss you so much but we’ll make it. We’ll figure it out. We love you. Whenever you’re ready, you go.”

If the dying person protected you and you didn’t get there in time, you say what you would’ve said if you had been there in time. We don’t know when the dying person has left. What do you have to lose? You don’t have to say it out loud. You can say it in your heart.

(1 hour 24 minutes) Dying is not a medical event. It is a social, communal event. You don’t need doctors, nurses. You just need someone who can support and guide you through this time.

Story about being with Bonnie when she’s dying. “You are doing a good job, Bonnie. Forgive yourself for any feelings or failures in your life. We are far harder on ourselves than God will ever be. Take only the good with you, Bonnie.” Talk to a dying person this way only with permission of the family.

(1 hour 34 minutes) There are people who have had near-death experiences. 774 people a day are having near-death experiences. People who have been pronounced as clinically dead but have been brought back have reported on their experiences.

 

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.
Robin

————————-

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

Progressive supranuclear palsy: what’s new?

Levy R.

Abstract
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 pubmed.gov 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:

Letter
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

Abstract
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:

www.ncbi.nlm.nih.gov/pmc/articles/PMC3125476/

Fantastic!

Robin

 

Decision-Making Impairment May Precede Limb Apraxia (CBD)

This recently-published letter from two Italian neurologists describes two patients initially diagnosed with parkinsonism. They were both given tests, including the Iowa Gambling Task (IGT), that revealed cognitive dysfunction. About 18 months later, both patients showed limb apraxia (based upon a Movement Imitation Test), resulting in a CBD diagnosis.

The two neurologists conclude: “These neuropsychological findings suggest that, in CBD patients, decision-making may be impaired early in the course of the disease and may sometimes precede the appearance of upper-limb apraxia. These findings also suggest that the IGT is a useful task to detect early cognitive impairments in patients with parkinsonism whose clinical symptoms are suggestive of CBD.”

I’ve copied the citation below.

Robin

Journal of Neuropsychiatry and Clinical Neurosciences. 2011 Winter;23(2):E29.

Letters
Decision-Making Impairment May Precede Limb Apraxia in Corticobasal Degeneration

Michele Poletti, M.D. and Ubaldo Bonuccelli, M.D.
Neurology Unit, USL of Viareggio, Italy Neuroscience Department, University of Pisa.

6 Things Never to Say to a Sick Friend + 4 Things…

I ran across this article tonight, and it certainly resonated. Many people with neurodegenerative disorders report that they dislike being told “you look great,” particularly when most of the symptoms are non-motor ones. The article’s author, who dealt with bone cancer, details 6 things you should never say to a friend or relative who’s sick and 4 things you can always say…

http://www.nytimes.com/2011/06/12/fashi … -life.html
(access to the NYT is no longer free beyond a certain number of articles each month)

(Excerpts from)

June 10, 2011
New York Times

This Life
‘You Look Great’ and Other Lies
By Bruce Feiler

…So at the risk of offending some well-meaning people, here are Six Things You Should Never Say to a Friend (or Relative or Colleague) Who’s Sick. And Four Things You Can Always Say.

First, the Nevers.

1. WHAT CAN I DO TO HELP?

2. MY THOUGHTS AND PRAYERS ARE WITH YOU.

3. DID YOU TRY THAT MANGO COLONIC I RECOMMENDED?

4. EVERYTHING WILL BE O.K.

5. HOW ARE WE TODAY?

6. YOU LOOK GREAT.

So what do patients like to hear? Here are four suggestions.

1. DON’T WRITE ME BACK.

2. I SHOULD BE GOING NOW.

3. WOULD YOU LIKE SOME GOSSIP?

4. I LOVE YOU.

Bruce Feiler’s memoir, “The Council of Dads: A Story of Family, Friendship and Learning How to Live,” has just been published in paperback.

Song “Speak to Me” + Story Behind It

An online friend, Mike Guerrieri, works for the American Speech-Language Hearing Association. Speech therapists (speech language pathologists) are ASHA members, along with some other disciplines. Members of our local support group see speech therapists as the disorders in our group include speech and swallowing symptoms.

Mike’s mother Kathe died almost a year ago. She had autopsy-confirmed MSA (multiple system atrophy).

Mike was inspired to write a song, “Speak to Me,” because of his mother’s struggles with communication. Kathe’s voice and swallow were weakened by MSA. As a result, Mike was made “more aware of how critical human communication is.” The song celebrates speech-language pathologists and audiologists.

The beautiful song can be listened to here:
http://www.asha.org/Events/convention/2 … hive/song/

Mike plays guitar in the recording.

You can see photos of Mike and Kathe here along with more of the story behind the song:
http://www.asha.org/Events/convention/2 … ong-story/

Robin