“Cognitive Dysfunction in Parkinson’s” – Dr. Marsh presentation

Geriatric psychiatrist Laura Marsh, MD, gave a wonderful presentation at last
week’s APDA/NPF Young Onset Parkinson’s Conference on the topic of cognitive
dysfunction in Parkinson’s Disease.  The presentation is likely of interest
to those in our Lewy Body Dementia group as well.

Dr. Marsh reviews the prevalence, pathophysiology, clinical characteristics,
and treatment of cognitive dysfunction in PD. She also describes much of the
research into cognitive issues and PD. (Dr. Marsh is the author of much of the
research!)

The conference was recorded.  You can find the video/audio recording here:

event.netbriefings.com/event/youngparkinsons/Archives/jointconf1009/Viewer/wmpviewerP004c.html

You can find a PDF of Dr. Marsh’s slides here:

event.netbriefings.com/event/youngparkinsons/Live/jointconf1009/materials/youngparkinsonsyou002-04.pdf

I hope you enjoy the presentation as much as I did!

No strong environmental risk factors for PSP

This French study looked at 79 cases with a clinical diagnosis of PSP, and 79 controls who were relatives. They analyzed “demographic characteristics, occupation history, diet habits, anti-inflammatory drugs use, alcohol consumption, smoking habits, gardening and leisure activities, and exposure to pesticides.” They found no “strong environmental risk factors for PSP.”

According to the article, “only four case-control studies on risk factors of PSP” have been published. Dr. Golbe talked about these in his webinar on PSP and CBD several months ago.

The authors argue that: “These data also suggest that PSP and PD have no major risk factor in common. Similar conclusions can be drawn from studies about multiple system atrophy.”

You may note that the French used the “Golbe criteria” to diagnose PSP. I’d have to look this up again but I assume it’s those over 40, with a history of falls, cognitive issues, and supranuclear gaze palsy. (The citation is: Golbe LI. Progressive supranuclear palsy. In: Tolosa E, ed. Parkinson’s disease and movement disorders. 2nd edn. Baltimore: Williams & Wilkins, 1993:145­61.)

Robin

Journal of Neurology, Neurosurgery, and Psychiatry. 2009 Nov;80(11):1271-4.

Risk factors for progressive supranuclear palsy: a case-control study in France.

Vidal JS, Vidailhet M, Derkinderen P, de Gaillarbois TD, Tzourio C, Alpérovitch A.
INSERM Unit 708, Hôpital de La Salpêtrière, Paris, France.

BACKGROUND: The risk factors of progressive supranuclear palsy (PSP), a rare but severe Parkinsonian syndrome, are poorly known.

OBJECTIVE: To study the risk factors of PSP in a case control study among French patients.

METHOD: The study was conducted between April 2000 and December 2003. Cases were in- or outpatients of five large hospitals and fulfilled the Golbe criteria. Controls were relatives of patients from the same hospitals, free of Parkinsonian syndrome and dementia, and matched to cases for age, gender and living area. Data on demographic characteristics, occupation history, diet habits, anti-inflammatory drugs use, alcohol consumption, smoking habits, gardening and leisure activities, and exposure to pesticides were collected through a face-to-face questionnaire. A conditional logistic regression was used to analyse matched data and estimate OR.

RESULTS: 79 cases and 79 controls were included. Only a few comparisons were significant. Cases reached a lower education attainment than controls (odds ratio (OR) = 2.6 (1.3 to 5.3), p = 0.01). Analysis of diet habits did not show any major difference although cases ate meat or poultry more frequently. Conversely, controls ate fruits more frequently than did cases. No association was found between PSP and occupation, use of pesticides, gardening, alcohol consumption, smoking habits and anti-inflammatory agent use.

CONCLUSION: In this case-control study, we did not find any strong environmental risk factors for PSP.

PubMed ID#: 19864660 (see pubmed.gov for abstract only, available for free)

“Instead of Saying X, Trying Saying Y”

This is a nice article with a helpful chart on using techniques of validation and redirection to deal with memory loss, repetitive questions, delusions or aggressive behaviors, when speaking with those with dementia.

This article is from care ADvantage, a free publication of the Alzheimer’s Foundation of America (alzfdn.org) for caregivers of people with AD and similar illnesses.
(Reprinted with permission from the Fall 2009 issue of care ADvantage magazine, a publication of the Alzheimer’s Foundation of America. For a free subscription, please visit www.afacareadvantage.org or call 866-232-8484.)

http://www.afacareadvantage.org/issues/ca_fall09.pdf –> article starts on page 9

Instead of Saying X, Try Saying Y
Use Validation and Redirection to Manage Daily Challenges
by Susan London, LMSW, QDCP
care ADvantage, Fall 2009

Let’s face it: caring for an individual with Alzheimer’s disease isn’t always a walk in the park; in fact, it can be downright stressful. Between memory loss, repetitive questions, false thinking or aggressive behaviors, each day often brings new challenges.

As a caregiver, your understanding of the disease as well as your attitude about the illness can have a great impact on the way you manage day-to-day caregiving responsibilities. And just because this illness has taken over someone close to you, it doesn’t mean you should let it conquer you as well. So what do you do when your loved one blames you for something you didn’t do or becomes paranoid that you are plotting against him or her? How about when someone sees objects that aren’t there or says things that simply don’t
make any sense?

If your gut reaction is to try to orient the person back to reality, you’re not alone. Many caregivers spend endless hours trying to prove who they are, where they are and what they are doing, but to no avail. They believe if they would just show a wedding album or have other family members confirm information that the person with Alzheimer’s disease will catch on and it will “click” for them. Even though a caregiver’s heart is in the right place, these efforts are often fruitless. Their loved ones may continue to press the issue, and sometimes even get angry or hostile at the “evidence” presented to them.

Fortunately, there are ways to manage daily challenges in order to minimize a caregiver’s stressful feelings and improve the odds that an individual with dementia will respond positively.

One of the best approaches to use with people with dementia is Validation—a technique that confirms their right to feel a certain way and express their emotions regardless of the situation. The Validation theory, developed by Naomi Feil, suggests that an individual
could be revisiting past events or trying to solve unfinished business. This helps explain why some people feel the need to go to work years after they retire or pay off a debt from decades ago. By validating their experience, you are meeting them where they are and sending a message that you still accept them no matter what. Another powerful approach to utilize is redirection—a behavioral intervention that shifts the individual’s focus, by distracting the person or moving away from an undesired topic or behavior to something more pleasant. See the chart on the following pages for some role-playing ideas that tap basic validation and redirection techniques to handle some of the most difficult situations.

When
Your mother says, “I want to go home!”

Instead of Saying
“This is your home! Don’t you remember? You’ve only lived here for 30 years!”

Try Saying
“Of course you want to go home! Your house was the prettiest on the block. Why don’t you tell me about those tulips you planted in your front yard?”

Why?
Memory impairments and disorientation can cause people to forget where they are. When they want to go “home,” it really signals a desire for a sense of safety and familiarity. Bring mom “home” by reuniting her with her favorite memories of what home represents.

When
Your wife says, “Get away from me, you’re not my husband!”

Instead of Saying
“But I am your husband! Look at our matching wedding rings. You know, you really upset me when you don’t remember who I am.”

Try Saying
“You must love your husband very much. I can tell by the way you talk about him. Why don’t you tell me about your wedding day?”

Why?
Memory loss can cause individuals to forget even their closest loved ones. When your wife becomes agitated, respect her space,
validate the love she feels for her spouse, and allow her to talk about “him” while you are sitting right beside her. Even though she may
have forgotten you today, hearing her talk about you will demonstrate that you are still very close to her heart.

When
During meals, your father refuses to eat and says, “You’re trying to poison me.”

Instead of Saying
“That’s ridiculous! Why would I ever do such a thing? Eat your meal and stop making up crazy stories!”

Try Saying
“Dad, I understand if you are feeling afraid, but I want you to know that I would never let anything bad happen to you. You are safe with me. By the way, this meatloaf is delicious. I am having a big plate of it myself. Let’s have some together and you can tell me all about
the fishing trip.”

Why?
It is difficult, if not impossible, to rationalize with people with dementia. Instead of trying to orient Dad back to reality, instill a sense of safety, and demonstrate it by eating the same meal as him or taking a bite from his plate. Once you’ve established trust, you can quickly refocus him by shifting the conversation to something more pleasant, such as a fun day he had by the docks.

When
Your sister says, “You stole my money! Give it back!”

Instead of Saying
“I’m sick and tired of you accusing me every time you hide your money. You stuffed it in your drawer five minutes ago. I saw you do it, so stop blaming me.”

Try Saying
“Oh no, your money is missing? I can see why you’re upset. Well, don’t you worry because I am going to help you look for it.”

Why?
It is common for people with dementia to hide items and forget where they are moments later. Since it can be embarrassing to admit this, individuals sometimes accuse others to take the focus off themselves. Rather than trying to deflect blame, simply let your sister know that you understand how she feels and that you want to help her resolve the situation. Then walk her over to the drawer and ask her to open it. When your sister finds her money, allow her to take pride in finding it all by herself.

When
Your grandmother says, “I have to leave now. I need to pick up Jimmy from school.”

Instead of Saying
“Grandma, Jimmy is a grown man. He’s 60 years old. You’re not going anywhere.”

Try Saying
“Oh Grandma, you have always been such a loving mother to Jimmy. Why don’t you come with me to get a drink of water and tell me all about what you love to do with Jimmy after school?”

Why?
People with Alzheimer’s disease often live in the past by re-creating experiences that happened long ago. Despite the fact that Grandma hasn’t picked Jimmy up from school in decades, her maternal instinct is still very strong, and she feels an urgency to follow the same routine she did while he was growing up. Take this as an opportunity to reunite your grandmother with those happy memories: while you are distracting her from going to the front door, she will tell you all about those wonderful times she had with Jimmy and will forget all about wanting to leave.

When
Your uncle believes he’s still at work. He treats you like an employee and tells you he needs the paperwork finished by the end of the hour.

Instead of Saying
“Are you starting with that nonsense again? You retired in 1985. How many times do I have to keep reminding you?”

Try Saying
“You really run a tight ship around here! I guess that’s what makes you such a good boss. I’ll make sure to get the paperwork to you, but I could use a little help. Can you assist me with one of the documents?”

Why?
It is not uncommon for people with Alzheimer’s disease to strongly affiliate with important roles they have taken on in the past. The fact that your uncle thinks he’s at work suggests that a job is still very meaningful to him. Use this moment to create an activity that can contribute to a sense of purpose. Bring him a phone bill or other document and allow him to “help” you interpret it. Support his efforts and remind him what a great boss he has always been.

SUSAN LONDON, LMSW,QDCP, is a social worker and project coordinator at the Alzheimer’s Foundation of America (AFA). As a member of AFA’s social services team, she regularly responds to calls and e-mails from caregivers across the country, walking them through both daily challenges and crisis situations.

Concomitant PSP and MSA (4 Cases)

This recently-published article is about one case of concomitant PSP and MSA in the Queen Square Brain Bank in the UK. (That’s one out of 120 cases of path-confirmed PSP.) And the article mentions that there are three other (independent) cases. One of these three is a Mayo Jax case; it was one case out of 290 path-confirmed PSP cases. So, that answers the question as to whether this is even possible!

Here are the highlights:

* “The apparent rarity of cases of coexistent PSP and MSA initially suggests they might be due to mere coincidence, rather than the result of any biological overlap.”

* “Grading of PSP tau pathology provided a score of 6, consistent with moderately severe tau pathology typical of Richardson’s syndrome [29].” [Robin’s note: this indicates that the RS form of PSP has more severe tau pathology than the other common form, PSP-P.]

* “Nevertheless, taking into consideration the rarity of MSA and PSP, in addition to the small number of cases that come to autopsy, the findings of 4 cases reported to-date is higher than expected.”

* “Whether these cases represent a pre-clinical stage of MSA, or simply a biological coincidence is yet unknown. The occurrence of pre-symptomatic MSA should be confirmed in prospective studies in which detailed neurological evaluation has been performed in the final months of life.”

* “Clinically our patient fulfilled the NINDS-SPSP operational criteria for the diagnosis of probable PSP and the only feature that might have suggested MSA was the urinary incontinence which started 20 years before death and resolved. The lack of overt cerebellar features of MSA is consistent with mild olivo-ponto-cerebellar pathology, whereas the prevailing parkinsonian syndrome is in line with more severe striato-nigral-degeneration, or the baseline PSP pathology.”

* “The rarity of pathologically proven cases of combined PSP and MSA does not provide sufficient evidence to confirm a common pathogenic mechanism for both diseases, but our findings suggest that systematic screening for PSP and MSA changes in atypical parkinsonian patients and control brain bank donors is warranted.”

The abstract follows.

Robin

Neuroscience Letters. 2009 Oct 14. [Epub ahead of print]

Concomitant progressive supranuclear palsy and multiple system atrophy: More than a simple twist of fate?

Silveira-Moriyama L, González AM, O’Sullivan S, Williams DR, Massey L, Parkkinen L, Ahmed Z, de Silva R, Chacón JR, Revesz T, Lees AJ, Holton JL.
Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both rare neurodegenerative diseases.

In the Queen Square Brain Bank, from 2001 to 2008, we received 120 cases of pathologically confirmed PSP and 36 of MSA, and one had concomitant PSP and MSA pathology.

The clinical symptoms in this case were compatible with PSP and did not predict the dual pathology.

The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance.

PubMed ID#: 19835934

Concommitant PSP and MSA (4 cases worldwide)

This recently-published paper addresses the question of whether PSP and MSA can co-exist.  The answer is “yes” but it’s quite rare.

There is one case of concomitant PSP and MSA in the Queen Square Brain Bank in the UK.  (That’s one out of 120 cases of path-confirmed PSP.)  And the article mentions that there are three other (independent) cases.  One of these three is a Mayo Jax case; it was one case out of 290 path-confirmed PSP cases.  So, that answers the question as to whether this is even possible!

Here are the most interesting excerpts:

“The apparent rarity of cases of coexistent PSP and MSA initially suggests they might be due to mere coincidence, rather than the result of any biological overlap.”

“Grading of PSP tau pathology provided a score of 6, consistent with moderately severe tau pathology typical of Richardson’s syndrome [29].”  [Robin’s note:  this indicates that the RS form of PSP has more severe tau pathology than the other common form, PSP-P.]

“Nevertheless, taking into consideration the rarity of MSA and PSP, in addition to the small number of cases that come to autopsy, the findings of 4 cases reported to-date is higher than expected.”

“Whether these cases represent a pre-clinical stage of MSA, or simply a biological coincidence is yet unknown. The occurrence of pre-symptomatic MSA should be confirmed in prospective studies in which detailed neurological evaluation has been performed in the final months of life.”

“Clinically our patient fulfilled the NINDS-SPSP operational criteria for the diagnosis of probable PSP and the only feature that might have suggested MSA was the urinary incontinence which started 20 years before death and resolved. The lack of overt cerebellar features of MSA is consistent with mild olivo-ponto-cerebellar pathology, whereas the prevailing parkinsonian syndrome is in line with more severe striato-nigral-degeneration, or the baseline PSP pathology.”

“The rarity of pathologically proven cases of combined PSP and MSA does not provide sufficient evidence to confirm a common pathogenic mechanism for both diseases, but our findings suggest that systematic screening for PSP and MSA changes in atypical parkinsonian patients and control brain bank donors is warranted.”

Check out the abstract or full paper for more.  The abstract follows.

Robin

————————————

Neuroscience Letters. 2009 Oct 14. [Epub ahead of print]

Concomitant progressive supranuclear palsy and multiple system atrophy: More than a simple twist of fate?

Silveira-Moriyama L, González AM, O’Sullivan S, Williams DR, Massey L, Parkkinen L, Ahmed Z, de Silva R, Chacón JR, Revesz T, Lees AJ, Holton JL.

Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both rare neurodegenerative diseases.

 

In the Queen Square Brain Bank, from 2001 to 2008, we received 120 cases of pathologically confirmed PSP and 36 of MSA, and one had concomitant PSP and MSA pathology.

The clinical symptoms in this case were compatible with PSP and did not predict the dual pathology.

The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance.

PubMed ID#: 19835934