Supranuclear gaze palsy occurs in more than just PSP

“Supranuclear gaze palsy” (SGP) refers to impairment of horizontal gaze and/or vertical gaze.  This symptom denotes “dysfunction in the connections responsible for conducting voluntary gaze commands to the brainstem gaze centers.”

As many of you know, SGP is a classic clinical feature of progressive supranuclear palsy (PSP).  In fact, it is part of the diagnostic criteria for PSP.  However, this symptom is not specific to PSP and can occur in many other neurological disorders, including parkinsonian conditions.

In this Washington University (St. Louis) study, researchers examined the clinical records of 221 parkinsonian patients who had visited the movement disorders clinic and who had donated their brains for research.  [By the way, Brain Support Network has been responsible for over 650 brain donations — quite a bit more than the WashU brain bank.]

Of the 221 parkinsonian brains in their brain bank, 27 had supranuclear gaze palsy noted in the clinical records.  The confirmed diagnoses of these 27 were:
* progressive supranuclear palsy (9),
* Parkinson’s Disease (10),
* multiple system atrophy (2),
* corticobasal degeneration (2),
* Creutzfeld-Jakob Disease (1), and
* Huntington Disease (1).

The researchers also looked at the 14 brains donated of those with PSP in their brain bank.  Nine of the 14 had clinical evidence of SGP but five did not.

Curiously, their brain bank doesn’t have many dementia with Lewy bodies (DLB) cases because their brain bank has a bias towards movement disorders rather than dementia.

This paragraph about MSA is interesting:

“In a study of oculomotor function in MSA, Anderson and colleagues suggest that the presence of clinically slow saccades, or moderate-to-severe gaze restriction, implies a diagnosis other than MSA. In contrast, our data indicate that SGP can be seen in patients who have subsequent autopsy-confirmation of MSA at a frequency similar to that seen in PD. Cognitive impairment is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. However, some patients with pathologically confirmed MSA have been reported to have dementia. Cykowski and colleagues have reported that the presence of Lewy body-like inclusions in neocortex in MSA, but not hippocampal alpha-synuclein pathology, was associated with cognitive impairment. We suggest that the association of SGP with MSA in some individuals provides further evidence for cortical pathology.”

The authors point out that other studies show that 90% of those with CBD develop SGP.

SGP is also reported in other disorders such as spinocerebellar degeneration, amyotrophic lateral sclerosis, Whipple disease, and Niemann-Pick disease type C.

I’ve copied the abstract below.

Robin

———————–

Parkinsonism Relat Disord. 2017 Feb 24. [Epub ahead of print]

Pathologic correlates of supranuclear gaze palsy with parkinsonism.
Martin WR, Hartlein J, Racette BA, Cairns N, Perlmutter JS.

Abstract
INTRODUCTION:
Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic.

METHODS:
We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St. Louis between 1996 and 2015. All patients with parkinsonism from this population who had subsequent autopsy confirmation of diagnosis underwent further analysis.

RESULTS:
221 unique parkinsonian patients had autopsy-proven diagnoses, 27 of whom had SGP documented at some point during their illness. Major diagnoses associated with SGP were: PSP (9 patients), Parkinson disease (PD) (10 patients), multiple system atrophy (2 patients), corticobasal degeneration (2 patients), Creutzfeld-Jakob disease (1 patient) and Huntington disease (1 patient). In none of the diagnostic groups was the age of onset or disease duration significantly different between cases with SGP and those without SGP. In the PD patients, the UPDRS motor score differed significantly between groups (p = 0.01) with the PD/SGP patients having greater motor deficit than those without SGP.

CONCLUSION:
Although a common feature of PSP, SGP is not diagnostic for this condition and can be associated with other neurodegenerative causes of parkinsonism including PD.

Copyright © 2017 Elsevier Ltd. All rights reserved.

PMID: 28256434  (see pubmed.gov for this abstract only)

Five E’s of empowered living with chronic illness

CurePSP hosted a webinar last year that focused on “patient-centered multidisciplinary management” of chronic disorders.  While the webinar was directed to families dealing with progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD), the concepts apply to all situations, regardless of disorder.  Very little about this webinar was specific to PSP, MSA, or CBD.

The speaker was Becky Dunlop, RN, with Johns Hopkins Parkinson’s Disease and Movement Disorders Center.

You can find an archived recording of the webinar here:

www.youtube.com/watch?v=BWoXJdkkV6I&feature=youtu.be

Your PSP, CBD, MSA Management Plan: Resources and Services
CurePSP Webinar
Speaker:  Becky Dunlop, RN, Johns Hopkins
March 6, 2016

Brain Support Network volunteer Denise Dagan listened to the webinar and said:  “[Becky] really paints a comprehensive picture that there is so much you can do to continue living even after what seems like a catastrophic diagnosis, if you’re willing to learn, and can surround yourself with supportive people.”

As part of her hopeful message, Becky shared five “E’s” of empowered living with chronic illness:

* Education:  learn about a disorder
* Expanding and building your team
* Effective communication:  utilize speech therapy, communication boards, etc.
* Effective coping
* Exploring options:  find a movement disorder specialist, participate in research, etc.

In terms of effective coping, these suggestions were offered:
* psychiatric services, professional counseling, social work services
* stress management
* meditation or exercise
* development of a support network
* support groups:  find or start one
* education programs
* develop and maintain your humor
* pet therapy
* maintain faith and hope
* get your rest
* maintain a healthy perspective
* find beauty in life
* don’t be afraid to ask directions
* recognize and celebrate your role and define the unique you

Denise’s notes about the webinar along with the question-and-answer session are below.  There’s more about PSP in the Q&A than there is in the presentation itself.

Becky referred to the WeMove organization.  This organization hasn’t been in business for several years so I deleted that reference.

Robin

===================================

Denise’s Notes

Your PSP, CBD, MSA Management Plan: Resources and Services
CurePSP Webinar
Speaker:  Becky Dunlop, RN, Johns Hopkins Parkinson’s Disease and Movement Disorders Center
March 6, 2016

Learning Objectives:
* Comprehend the need for patient centered multidisciplinary management of PDP, CBD, and MSA
* Identify available resources for individuals living with PSP, CBD, MSA and their families

Patient Centered Care:
* All these individuals strive to identify and meet the needs of the patient
* The Institute of Medicine (2001).  Crossing the quality chasm: A new health system for the 21st century.
* “Providing care based on patient’s needs and expectations is the key attribute of quality care.”

Bergeson & Dean Commentary on Patient Centered Care in JAMA (2006)
* Ensures access and continuity (access to psychiatry, PT, and other services, and continuity among and between service is ensured)
* Provides opportunities for patient and family participation (key take-home point of this presentation)
* Supports self management
* Coordinates care between settings

Individualized therapy involves not only the pharmacological, but also the appropriate use of allied health professionals, assistive technologies, educational and support resources along the chronic illness continuum.

The Es of Empowered Living with PSP, CBS, MSA.  Identify strategies in each of these areas that will help patients and families.
* Education
* Expanding & building your Team
* Effective Communication
* Effective Coping
* Exploring Options

Education:
Knowledge is Power
Lay Education / CurePSP offers
* Network of education and support groups
* Numerous publications
* Web resources
* Conferences and Symposia

Highlights of Lay Educational Resources
* Webinars on specific topics such as incontinence, aphasia, advance directives
* Resources like:
– CurePSP
– National Institute for Neurological Disorders and Stroke http://www.ninds.nih.gov
– Lee Silverman Voice Treatment website

Expanding and Building Your Team:
* Lay people (family, friends, disease community/support group, church)
* Healthcare professionals including:
– Primary Care Physician
– Neurologist, especially a movement disorder specialist
– Urologist
– Cardiologist (orthostatic hypotension)
– Ophthalmologist (double vision, difficulty focusing)
– Psychiatrist (depression, anxiety)

Visual Disturbances (Importance of Ophthalmologist)
* Dry eyes (natural tears and lubricants)
* Difficulty looking down (prismatic lens)
* Difficulty with eye movements, focusing
* Double vision (covering one side of glasses with tape)
* Sensitivity to light (sunglasses or tinted glasses)
* Blepharospasm (Botox around the eye muscle)

Psychiatric Issues (Importance of Psychiatrist, although PCP could help with some of these as well)
* Depression
* Inappropriate laughter or crying
* Impulse control problems (difficulty realizing limitations caused by disease so trying to do what they’ve always done may risk falling)

Role of Allied Team Members:
* Physical Therapy
* Speech Therapy
* Occupational Therapy (managing home safety, managing patient’s ability to participate in every day activities safely)
* Nursing
* Social Workers

Physical Therapy
* Maintain mobility
* Prevent falls
* Suggest walker and wheelchair as appropriate
* Instruct in safe transfer and walking
* Instruct caregiver to maintain caregiver health

Speech Therapy:
* Communication boards (www.givinggreetings.com/olderadults.html)
* Encourage patient to speak slowly
* Allow adequate time for response
* Prevent social isolation

Speech Therapy / Swallowing Issues:
* Place the chin in a downward or neutral position to close off the airway during swallowing
* Learn the Heimlich maneuver for use in the event of choking. (All family members.)
* Have suction equipment available for clearing the airway at some point in the disease.
* Thickening agents for liquids
* Consideration of a feeding tube (Verdun, 2000)

Example of a communication chart.  The person having communication difficulties just has to point.  [Editor’s note:  see webinar]

Occupational Therapy:
* Home modifications
* Home safety

Dietician:
* Unanticipated weight loss
* Maintaining a healthy weight
* Management of constipation

Developing your Personal Support Network:
* Educate family and friends
* Identify resources thru church or social networks
* Devise a plan by identifying needs
* Ask for help
* Consider creating an online care network.  www.caringbridge.org is a central place to keep family & friends up to date and ask for help.

Effective Communication

Effective Coping

Some Resources to aide Coping:
* Psychiatric Services (caregivers sometimes need an objective, trained person for our own needs)
* Professional Counseling ( “ )
* Social Work Services ( “ )
* Stress management (to improve quality of life)
* Meditation or exercise (stress busters)
* Development of a support network (list everyone who is there to help you, and call upon them, even if its just one meal weekly)
* Support groups & education programs (learn from each other)

Develop & Maintain your Humor
She used to hand out Sunsweet Prunes because constipation is a common issue.  Her motto was, “We keep you moving.”

Pet Therapy

Maintain Faith & Hope
Identify your faith community
Have hope knowing there are tools out there to help you.  Don’t lose sight of that.

Get your Rest
It supports your stamina.

Find or Start a Support Group

Maintain a Healthy Perspective
When you’re living with a chronic disease, take time to look up and experience the larger world to gain perspective.

Find Beauty in Life
View the sea or mountains, buy yourself some flowers, etc.

Don’t be afraid to Ask Directions
If you don’t stop asking, people will be available to help and guide you.

Recognize and Celebrate your Role and Define the Unique YOU

Caregiver Health is VITAL to the health of the patient !
The first rule of caregiving is to take care of yourself.  So explore all these options…

Exploring Options:
* Movement Disorder Specialists over a neurologist
* Participation in research
* Other specialists (urinary frequency, call a Urologist / unmanaged constipation, call a GI / depression not well treated, modify meds.)
* Occupational Therapy Assessments/Driving Assessments/ Home Safety & Fall Prevention
* Caregiver Resources (National Caregiver Resources, CurePSP)
* Assistive Devices
* Consider Palliative Care and Hospice

Walking aides
U-Step walker has red laser line between back wheels to prevent freezing.
laser cane
www.parkinsonshop.com/

Home environment.  Get an OT consult.
* Considerations for safety:
– Grab bars in bathroom (by toilet, tub, shower, sink)
– Railings on stairs/steps
– Adequate lighting
– Eliminate fall hazards:  Throw rugs, toys, clutter
– Entrance ramp

Gizmos and Gadgets
– Shoehorn with a long handle (medical supply stores)
– Bedrail that slides under mattress and helps get out and reposition themselves in bed.  (Must extend 3-4 feet under mattress for safety.)
– Swivel Seat (getting in/out of a car.  Plastic bags can work just as well on fabric car seats.)
– Ursec Urinal (this is a travel variety.  Good for preventing spills.)

More Gizmos and Gadgets
www.mtsmedicalsupply.com/pages/parkinsons-products.cfm
www.activeforever.com/flyers/Movement_Disorder_Catalog.pdf
Rollator (consult w/a PT before purchasing one.  Having a seat can be good on long outings.)
Stand Ease (helps one to stand from a low seat)
Turn Ease & Car Ease (help get in/out of car or bed.  Silk sheets and/or PJs can make it easier to reposition in bed)
Sock Aide (helps you put on a sock independently)

Complementary Therapies provide enjoyment and an outlet with a person living with a long term disorder.  Improves quality of life.
* Music therapy
* Art therapy
* Therapeutic horticulture
* Aromatherapy
* Animal-assisted therapy
* Spiritual care
* Massage therapy
* Healing touch
* Acupuncture

Hope
That elusive spirit in the heart of man,
With it, desires and fears will withstand.
When present and believed within the soul,
The thread of hope will keep you whole.
by Becky Dunlop, RN (2009)

Q&A
What county, state or federal agencies can help?
Contact your local Area Agency on Aging.  They are charged with helping individuals navigate the healthcare system when they are 60 years +.  If younger approach the state disability services agency.  Nurses and social workers at those agencies can provide direction.

Differences between Parkinson’s & PSP?
Main difference is Parkinson’s being a chronic progressive disease with good medicines for symptoms for many years so people are able to live full lives.  Advanced Parkinson’s disease is similar to PSP.  With PSP an individual will progress chronically and movement becomes worse because there are no medicines to reduce symptoms.  Falls and immobility increase over time.  In advanced Parkinson’s medicines don’t work as well because of brain cell loss.

As PSP progresses is it normal for the patient to become increasingly confused in unfamiliar surrounds outside the home?  What can a caregiver do to lessen the impact on the patient?
Yes, that is normal.  What a caregiver can do is create a routine and repeat verbally and in writing to the patient what’s going on.  Mainly, let them know what you’re going to do at the beginning of the day.  This may limit some of their anxiety and may help them be the best they can be.  Remember these individuals are not dealing with the same neurologic capacities we are as healthy people, therefore any change in routine is anxiety provoking and a disruption to them.  Anything you can do to aid and eliminate that will help them.

My husband can hear sound, but does not always understand what is being said.  Communication has become extremely difficult.  Is there anything to aid with this situation?
When someone can’t sort out verbal communication, it is very challenging.  Keep it simple.  Keep it slow.  Repeat yourself.  Give him time to process.  In many of these neurodegenative disorders people have bradyphrenia (slow thinking).  Their processing ability is much slower than a healthy person’s.  They may feel as though they understand what’s going on around the, but can’t get it out because what’s going on around them is happening so quickly.  Anything we can do to slow it down, simplify it, repeat, and give them time to reflect, will give them time to help that communication.

What palliative care services can be helpful to PSP patients?
Palliative care services may be engaging a home care agency that offers palliative care.  Identifying a depression may be helpful.  Identifying changes in vision.  Sometimes when a person has excessive saliva we provide medication to dry that and help them be more comfortable.  The whole aim is not to cure, but to keep the person as comfortable as possible, given the circumstances.

Will a discussion of facing the end worsen the depression of the patient?
Depends on the patient.  Many people want to know what lies ahead.  Even people with dementia, people can be concerned.  Reassuring someone and telling them the truth, but that everyone is there to help them is a comfort.  Facing the end may help the individual and allay their anxiety.  It may help them build their coping skills, knowing what to expect.

My PCP is treating my urinary frequency.  Should I see a urologist?
Sure.  A specialist may see something the PCP doesn’t know about.  Two heads are better than one.

Looking for a support group?
Contact CurePSP even for world-wide locations.

When should you consider palliative care?
Now.

Short podcast on Parkinson’s Plus conditions with Dr. Golbe

This is a worthwhile 28-minute podcast about Parkinson’s Plus conditions, including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), vascular parkinsonism, corticobasal degeneration (CBD), and dementia with Lewy bodies (DLB).

soundcloud.com/world-parkinson-coalition/07-parkinsons-plus-conditions

Parkinson’s Plus Conditions
Podcast by World Parkinson Coalition
Speaker:  Lawrence I. Golbe, MD, movement disorder specialist

Brain Support Network Treasurer Phil Myers listened to the podcast and reports: “This podcast was done a year ago in 2016 as preparation for the World Parkinson’s Congress in Portland.  This is good coverage of all variations of Parkinson’s Plus. It compares each of them to Parkinson’s. It does talk about the lack of direct treatment but does discuss symptom treatments. And it talks about the difficulty of diagnosis.”

I’ve shared an outline of the podcast and a few notes below in case you want to forward to your disorder of interest.

Robin

—————————

1:00 – Dr. Golbe begins a general overview of Parkinson’s Plus conditions

1:45 – PSP discussion begins

5:00 – terrific question and answer on why levodopa doesn’t work in PSP, MSA, etc.

6:00 – MSA discussion begins

8:00 – discussion of dementia in MSA

8:20 – is MSA a prion disease?  He can’t comment on any one study in this format.  He says many diseases are prion-like in that there is a toxic chain reaction.  “Can’t catch MSA.”  Key distinction is that in CJD, the key protein is prion.  Prion protein can be transmitted more easily than other proteins.

11:00 – treatment of MSA symptoms

11:52 – discussion of vascular parkinsonism, which isn’t a neurodegenerative disease.

13:43 – CBD discussion begins.  One-tenth as rare as PSP or MSA.  Those conditions are one-twenthieth as prevalent as Parkinson’s.  Life expectancy similar to PSP and MSA:  between 5 and 10 years, typically between 7 and 8 years.  Lots more tau in CBD than in PSP.

15:10 – MSA pathology

15:40 – DLB discussion begins.  Spontaneous fluctuations that happen in DLB don’t happen in PD.  Levodopa isn’t quite as effective in DLB than in PD.  There’s is a problem that levodopa can cause hallucinations in DLB.

18:00 – general discussion.  There is long-term hope via a double-pronged attack.  First, stopping the protein aggregation or misfolding for a group of diseases.  Second, focusing on each individual disease.

We are further along in PSP given the focus on Alzheimer’s – both are tauopathies.

CBD is more difficult to do research on — because it’s rare and because diagnostic accuracy is very poor (50%).

21:00 – general discussion.  Neglected because they are rare, hard to diagnose, and no biomarkers.  FDA can given these diseases the status of an “orphan disease.”

22:45 – general discussion about how to help these families.  First, accurate diagnosis is important.  Sometimes people are given fruitless tests on the way to a diagnosis.  Second, symptoms can be treated.  Palliative management of symptoms.  Referrals can be made.  Third, at academic centers, treatment trials or research projects are available.  These can give patients the feeling of “fighting back” to help future generations.

25:00 – Dr. Golbe stops speaking.  Podcast continues with comments from hosts Dave Iverson and Jon Palfreman.

Facial Masking – Blog and Podcast Notes

“Facial masking” is a common symptom in both Parkinson’s Disease and the atypical parkinsonism disorders (LBD, PSP, MSA, CBD). It is a symptom where facial muscles are immobilized and the person is expressionless.

The face is one of the primary means by which we communicate with each other; there’s loads of nonverbal communication in the face.

According to the Davis Phinney Foundation (davisphinneyfoundation.org), facial masking negatively impacts:
1- how healthcare professionals evaluate a person with Parkinson’s levels of sociability, depression and cognition
2- impressions and judgment of others
3- the enjoyment of a care partner in a relationship

If a person is speaking with another individual with this symptom, the person may believe that the other individual is disinterested or bored. This is true even if the people are married or in the same family.

And clinicians may believe the individual is disinterested or bored! Occupational therapist Linda Tickle-Degnen describes the difficulty this way:

“If a neurologist, speech therapist, or occupational therapist gets the feeling that the person they’re working with is disinterested or depressed, then this relationship can be affected. [Practitioners sometimes] fail to talk to the person about their feelings because facial masking consistently causes a perception of depression, deception, hostility, and apathy in practitioners — even when they are experts.” (tuftsjournal.tufts.edu/2010/01_1/features/01/)

Here are some tips for care partners and practitioners from the Davis Phinney Foundation:

Here are four tips for Care Partners and Practitioners (from a graphic related to the article):

1. Ask Positive Questions. Positive questions have been shown to bring out more facial expression than negative questions. For example, ask your loved one about what went right today or what instances brought them joy.

2. Trust verbal communication. We rely on facial cues to interpret others’ emotions. When a person has facial masking, it is important to trust what the person says is true, even if the facial expression does not match the sentiment.

3. Inform others. Tell new friends about your loved one’s facial masking and provide some tips for engaging with someone who has facial masking.

4. Examine your bias. Women and Americans are usually expected to be more emotionally expressive, making facial masking have a greater effect on how they are perceived. What biases might you have when it comes to how you believe people should express themselves?

Finally, this brings us to Kelsey Phinney, daughter of Davis Phinney, with Parkinson’s. In a podcast for the Davis Phinney Foundation, Ms. Phinney interviewed occupational therapist Linda Tickle-Degnan about her researcher on the social effects of facial masking.

You can a blog post by Ms. Phinney here along with a link to the 26-minute podcast:


www.davisphinneyfoundation.org/blog/facial-masking/

Brain Support Network volunteer Denise Dagan listened to the podcast and took some notes. The notes are shared below along with the related blog post by Ms. Phinney.

Robin

——————————–

Denise’s Notes

“How Facial Masking Influences Perception and Relationships”
Davis Phinney Foundation Podcast
by Kelsey Phinney
January 9, 2017

Kelsey Phinney was five when her dad was diagnosed with Parkinson’s disease (PD) and has been interested in learning more about the brain and ways to help people living with Parkinson’s ever since. Kelsey graduated from Middlebury College in May 2016 with a degree in neuroscience.

This is her first podcast for the Davis Phinney Foundation, and its pretty good work. She chose facial masking as the topic and explains why in the introduction below. She interviewed professor Linda Tickle-Degnen, from Tufts University, who’s done some research on the social effects of facial masking with some pretty interesting results.

In a well controlled study done with both Taiwanese and Americans, she found that no matter the level of training or years of experience a healthcare professional had, they all perceived people with facial masking as being more depressed, less sociable, and less cognitively capable.

– In Americans, who value sociability quite highly, participants viewed people with PD facial masking as being less sociable.

– In Taiwan, intelligence and social contribution are more highly valued and people with PD facial masking were more negatively viewed in those areas.

– In both cultures, women are expected to be more emotionally expressive, and were more negatively viewed in all categories assessed.

Dr. Tickle-Degnen’s team has not found a way to train healthcare professionals to overcome this bias. It it likely deeply rooted and has been evolutionarily beneficial. The question is, will this innate bias affect the care people with PD facial masking receive.

In another sample of older healthy adults tested in the same conditions as the healthcare professionals, the result was the same. And, further, people with PD facial masking were seen as being less desirable social partners. This is a huge barrier for those with PD, when it is known that social support is at least, if not more important than physical exercise to prevent motor decline, morbidity, and mortality in older adults, not just those with PD.

Finally, research with people who have PD facial masking and their care partners reveal that care partners tend to have less enjoyment in a relationship the more the partner is facially masked (on a continuum). The upshot is, the more facial masking you have, the less people want to hang out with you – even your care partner!

This is only part one of this interview. The second part will be about current research and tactics people use in personal relationships and social settings to help people with facial masking better communicate, especially with their partner, and ways the partner can better understand what’s going on with their loved one.

==========================================================

www.davisphinneyfoundation.org/blog/facial-masking/

How Facial Masking Influences Perception and Relationships
The Davis Phinney Foundation
By Kelsey Phinney
January 9, 2017

Earlier this fall, my parents came to visit me in Sun Valley, Idaho. During their visit, we had some of my new friends over for dinner. These friends had never met my parents, and they also had never met a person living with Parkinson’s.

Thanks to my recent work with the Davis Phinney Foundation, I’ve come to understand that it is important to be able to talk about the different sides of Parkinson’s with those close to you. With this in mind, I realized that the main thing I needed to tell my friends before dinner was that one of the more notable symptoms of my dad’s Parkinson’s is facial masking.

My dad has an amazing smile, makes goofy faces and gets that sparkle in his eye when he talks about something he’s passionate about. While those things haven’t disappeared with years of Parkinson’s, they unfortunately can be subdued. Particularly when he is tired, his face can appear to be “masked.”

THE EFFECTS OF FACIAL MASKING
My dad’s face, like many people with Parkinson’s who experience facial masking, is less expressive and more neutral than one would expect, given the content of his conversation.

Facial masking causes a loss of facial expressivity. The muscles of the face lose muscle tone, leading to a seemingly blank expression.

For people who don’t know facial masking, the blank expression can be misconstrued as a lack of interest, displeasure, low sociability or low cognition. We’re accustomed to a person’s face shifting and changing regularly with the conversation. With a masked face, there is often slowness and stillness in the muscles of the face.

The primary thing I notice is less blinking and less reaction, positive or negative. However, when someone with a masked face is passionate about a story or topic, you can often see the most genuine and beautiful smile!

Even though I know what facial masking looks like, having a conversation with a person with facial masking can feel confusing. We expect facial responses in conversation. A simple smile, nod, eyebrow raise or crinkle of the eyes makes the person who is talking feel heard. With facial masking, these small movements are not so simple.

It is easy to take for granted our ability to subtly communicate through our face and movements. It can be challenging to gauge how someone should react to our own facial expressions and stories. We may disregard someone with facial masking because their facial expressions, or lack thereof, go against our social expectations. Also, it can be difficult to trust verbal communication when the facial expression doesn’t match the sentiment.

From my dad’s point of view, I imagine it is incredibly frustrating to not be able to effortlessly express himself.

LISTEN TO KELSEY’S PODCAST
I wanted to know more about the implications of facial masking and the importance of facial expressions in social interactions, so that we can all better understand how to respond. I interviewed Professor Linda Tickle-Degnen, Director of the Health Quality of Life Lab at Tufts University. Listen to my podcast to hear about her research on the effects of facial masking, what it is and how it impacts how people are perceived by healthcare professionals as well as their own care partners.

LOOK FOR THE SECOND PODCAST – COMING SOON!
Dr. Tickle-Degnen had a lot of interesting information to share, so Part Two of my interview will focus on her current research along with ideas and tactics for people who experience facial masking.

Podcast on brain atrophy in PSP and CBS with Dr. Boxer

The journal Neurology hosts weekly podcasts with neurologists around the world. In this podcast from November 8, 2016, Adam Boxer, MD, neurologist at UCSF’s Memory & Aging Center, discusses brain atrophy in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The interview with Dr. Boxer is the first ten minutes of the podcast.

Brain Support Network Treasurer Phil Myers listened to the podcast and found it interesting.  He says Dr. Boxer discusses “using MRI measurements to speed up the time it takes to do a clinical trial for PSP or CBS potential drug trials.”

You can find the podcast here:

tools.aan.com/rss/index.cfm/getfile/AAN_3192.mp3

Neurology Podcast on brain atrophy in PSP and CBS
Neurology Journal
November 8, 2016

Robin

Blood Test to ID Who Has PD vs. PSP/CBD/MSA

Thanks to Brain Support Network volunteer Denise Dagan for passing this article in MedPage Today on to me.  This is progress on the way to a biomarker to discern if someone has Parkinson’s Disease (PD) vs. one of three atypical parkinsonism disorders — progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA).  Of course what we really need are biomarkers for each of the disorders — PD, PSP, CBD, and MSA.  But still, it’s progress.

The article is about a Swedish/UK study (published in the journal Neurology on February 8th) that shows that neurofilament light chain (NF-L) protein levels are increased in PSP, CBD, and MSA as compared to those with PD or healthy controls.   This was true for those recently diagnosed as well as those with had been living with PD, PSP, CBD, and MSA for several years.  (This also means that the blood test is not a biomarker for determining if someone has PD.)

And researchers determined that a blood test for the NF-L protein achieves the same results as a spinal test for this protein does.  Obviously it’s much easier to administer a blood test than a spinal tap.

In an accompanying editorial, authors pointed out that there is a need for autopsy confirmation of the PD vs. atypical parkinsonism diagnoses.

Here are two excerpts from the article:

* “These atypical parkinsonism disorders are rare, but they generally progress much faster and are more likely to be the cause of death than Parkinson’s disease,” [one of the researchers] said. “It’s important for patients and their families to receive the best care possible and to plan for their future needs.”

* “Distinguishing these major parkinsonian groups is crucial for best possible treatment and care, and not least for providing adequate information to patients and caregivers on their future needs and perspectives,” the editorialists pointed out. “When disease-modifying treatments become available, diagnosing parkinsonian disorders correctly at early, possibly preclinical stages will be even more important.”

Here’s a link to the full article:

www.medpagetoday.com/neurology/parkinsonsdisease/63007

Neurology
Blood Test Matches Spinal Tap to ID Parkinson Disease Types
Less painful diagnostic workup could help diagnosis
by Kristin Jenkins
Contributing Writer, MedPage Today
February 08, 2017

Robin

 

“Delirium Makes its Own Mark on Cognitive Decline” (Alzforum)

A comprehensive study of delirium and neurodegeneration was published last month in the journal JAMA Psychiatry.  The researchers wanted to learn “whether delirium worsens neurodegenerative pathology that’s already in the brain, or causes decline through a separate process, or both.”  Note that researchers relied on donated brains of those who suffered from neurological disorders — some of whom also had suffered delirium.  (If you are interested in brain donation, Brain Support Network can help your family make those arrangements!)

An Alzforum (alzforum.org) article about this research makes several key points about how delirium can hasten neurodegeneration and how delirium should be prevented:

* “[Delirium] contributes to cognitive decline independently of Aβ, tau, Lewy bodies, or vascular disease. But combined with any of these pathologies, delirium can quadruple the rate of memory loss.”

* “Delirium hastens cognitive decline in patients who have Alzheimer’s disease and increases the risk for dementia in older people who become delirious after surgery.”

* The findings suggest “delirium and pathology interacted to accelerate decline even further.”

* The “findings are a call to take delirium more seriously.”

* A clinician not involved in the study “said this study had tremendous health implications. ‘This creates an amazing impetus for public health agents to focus on delirium prevention as a way to reduce the negative burden on brain health.’ Almost half of cases are preventable by simple, inexpensive methods, ensuring people get optimal sleep, pain medication, fluids, and exercise in the hospital, he said (Hshieh et al., 2015).”

Here’s a link to the Alzforum article:

www.alzforum.org/news/research-news/delirium-makes-its-own-mark-cognitive-decline

Delirium Makes its Own Mark on Cognitive Decline
Alzforum
03 Feb 2017

Robin

Compassionate communication and managing difficult behaviors

Brain Support Network had an exhibitor table at the December 2016 Alzheimer’s Association “Circle of Care” conference in Foster City.  Steven Russell staffed our exhibitor table, where he talked to people about brain donation and our local support group for those with non-AD dementias.  He also had the opportunity to attend a few of the break-out sessions.  Copied below are his notes from the break-out session on “Managing difficult behaviors.”

During the break-out session, a handout on “Compassionate Communication” was reviewed.  The handout offers “do’s” and “don’ts” of communicating with someone with memory impairment.  You can find my previous post from 2008 about this terrific handout:

www.brainsupportnetwork.org/compassionate-communication-dos-and-donts/

Two key lines from the “Compassionate Communication” handout are:

“You can’t control memory loss, only your reaction to it.   Compassionate communication will significantly heighten quality of life.”

Words to live by as caregivers!

Robin

————————————

Steven’s Notes

Managing Difficult Behaviors
Session led by:  Alexandra Morris, Alzheimer’s Association
Circle of Care Conference, December 2016

Ms. Morris spent a good deal of time describing some typical dementia behaviors, why they occur and how to change or redirect these behaviors.

Ms. Morris reminded all of us that dementia behaviors are not deliberate. The care recipient has a disease and is exerting all of his or her energy to do the best they can with their disease.  Expending so much effort when trying to communicate and have the care recipient’s needs met can lead to “sundowning” at the end of the day.

Additional dementia behaviors can include repetitive questions, hiding/hoarding objects, delusions and hallucinations, suspicion, wandering and hitting. Besides exhaustion what leads to these behaviors? Here are some ideas.

The care recipient is a person who

– is an adult and doesn’t like being disrespected or under someone’s control

– is trying to get his or her needs met

– needs connection and purpose

– lacks insight into his or her condition

– is doing the best he or she can to deal with the cognitive challenges being experienced

– is  suffering from delusions or anxiety

– is irritated at something the caregiver said or did.

Ms. Morris gave us several tips to dealing with behaviors:

– Kindness versus Truth – “white lies” are better than anger, hurt or causing fear

– Appeal to the care recipient’s motivation (this may require practice to carry off successfully)

– Always say “yes” to a request.  If the outcome could endanger the care recipient, redirect to something safer as soon as possible.

– Don’t try to reason with or over-explain something to the care recipient

– If communication gets stuck, stop. Try physical movement to get unstuck (touch, gently direct the person towards where thy need to go).

– Tell a story – “I wondered about that…” “I’ll have to look into that…”

Probably one of the most difficult challenges happens when the care partner knows that a topic will agitate the person. Here are some tips:

– Determine if the conversation must be raised at all

– Interject with ” I have a question…”

– Change location

– Appeal to the person’s emotions, not the topic itself

– Use humor to defuse a charged situation

– Tell a “therapeutic fib” that helps move the conversation along without frightening the care recipient.

Every behavior has a purpose. You may have to put on your detective hat to determine what the purpose is.

– Let the issue go (“So what?”)

– Alter the situation to make it acceptable

– Keep responses and behavior slow, simple and calming

– Re-frame the issue/redirect to a more comfortable place

– Beg forgiveness rather than ask permission

Ms. Morris reminded the audience that as a care partner you can’t control memory loss, only your reaction to it. Remember that the care recipient’s disability is memory loss. Testing memory, lecturing the care recipient on their inability to find words or express appropriate emotions leads to suffering for all involved.  Instead refer only to the future (when the person with dementia mentions they want food, instead of saying “You ate an hour ago” say ” Why don’t I fix something for you in a bit?”).  Don’t use open-ended questions or try to initiate multi-step processes. Give the care recipient a simple choice between two items or direct their choice -“you look great in the green shirt.”

Finally, Ms Morris mentioned that the goal is to make life more calm and enjoyable for both you as care partner and for the care recipient. As you reassure the person remember that the care recipient cannot remember this reassurance. You’ll need to remind him or her each time.

“Medicare’s Coverage of Therapy Services Again Is In Center of Court Dispute” (KHN)

Incredibly, Medicare officials and consumer advocates are back in court again over the denial of therapy services (PT, OT, ST) to Medicare patients.

Here are some highlights from yesterday’s Kaiser Health News article:

* “Four years after Medicare officials agreed in a landmark court settlement that seniors cannot be denied coverage for physical therapy and other skilled care simply because their condition is not improving, patients are still being turned away. … The settlement affects care provided by a trained professional in a patient’s home, nursing home or the provider’s private office that is medically necessary to maintain the patient’s condition and prevent deterioration.”

* “Several organizations report that the government’s initial education campaign following the settlement has failed.”

* “‘We still regularly get calls from people who are told they are being denied coverage,’ said Peter Schmidt at the National Parkinson Foundation, based in Miami. Denials sometimes occur because physical therapy providers use a billing code that still requires the patient to show improvement. Although Parkinson’s is a degenerative brain disease, Schmidt said physical therapy and exercise can help slow its progress.

* “‘There was a long-standing kind of mythical policy that Medicare contractors put into place that said Medicare only pays for services if the patient could progress,’ said Roshunda Drummond-Dye, director of regulatory affairs for the American Physical Therapy Association. ‘It takes extensive effort to erase that.'”

Here’s a link to the full article:

khn.org/news/medicares-coverage-of-therapy-services-again-is-in-center-of-court-dispute/

Medicare’s Coverage Of Therapy Services Again Is In Center Of Court Dispute
Kaiser Health News
By Susan Jaffe
January 30, 2017

Robin

 

Care partner communication at all stages of dementia – workshop notes

Brain Support Network had an exhibitor table at the December 2016 Alzheimer’s Association (alz.org/norcal) “Circle of Care” conference in Foster City.  Steven Russell staffed our exhibitor table, where he talked to people about brain donation and our local support group for those with non-AD dementias.  He also had the opportunity to attend a few of the break-out sessions.  Here are his notes from the break-out session on “Care partner communication at all stages of dementia.”

Robin

—————————–

Steven’s Notes

Care Partner Communication at all Stages of Dementia
Session by:  Alexandra Morris, Alzheimer’s Association
Alzheimer’s Association Circle of Care
December 2016

EARLY STAGE

In early stages, where the diagnosis may be mild cognitive impairment, the person may be able to adequately express his/her thoughts, participate in and make decisions about future care but may also misinterpret what others say. People at this stage will have difficulty finding words, participating in/following conversations and struggle with decision-making or problem solving.  The care partner can connect with care recipient at this stage by using clear and straightforward sentences, leaving extra time for conversations (particularly responses), etc. Care partners should be especially careful to include the person in any conversation…related to future care decisions. Communicate in a manner that works best for the person (email, in-person, phone) and speak directly to him/her.

MID-STAGE

In mid-stage disease, language is reduced to basic words and sentences. The person receiving care is more likely to rely on tone of voice, facial expressions and body language to make a connection. At this stage, activities meaningful to the person with dementia are key to maintaining an emotional connection. Clues about cognitive changes include losing words (nouns go first), increasing trouble finding the right word and losing the train of thought or the thread of a conversation. Communication comes more through behaviors than words. Care partners can help by approaching from the front, saying who they are while calling the person receiving care by name. Care partners should move their level to match that of the care recipient, pay attention to tone of voice and take more time to let the conversation flow. Short sentences and basic words are best (one question at a time) and distractions should be limited. It is especially important to normalize experiences (for instance, if the care recipient is afraid, explain what is happening and show that you are not afraid).

Also at this stage, caregivers should join the care recipient’s reality. Keep respect and empathy in mind as you try to give the person multiple cues to help make and maintain a connection. Modeling behavior, keeping gestures fluid and overt (never sudden or coming from the side) repeating as necessary, avoiding “quizzing” about a topic and turning negatives into positives are great tools to help build trust. Writing things down, pointing them out or using photographs or pictures to convey meaning are also very helpful. One cue Ms. Morris mentioned is putting answers into your questions — “Would you like to wear the red shirt today?”  As verbal communication begins to decline, try and asses the care recipient’s needs (pain, bathroom, hunger, temperature, fear, boredom). People with dementia only receive half the pain medication needed compared to functional adults. In addition people with dementia are almost never treated for breakthrough pain (the person with dementia struggles to express this need so the care partner will need to always be alert to checking pain levels and advocating for patient comfort. Let the person with dementia know you hear his/her concern whether through words, behavior or both.

LATE-STAGE

In late-stage disease, the care recipient uses body language and his or her five senses to make a connection. The person may still respond to familiar words, phrases, smells or songs. Pain is often chronic at this stage . If the care recipient is agitated, always check first for pain, then bathroom, food, temperature, etc. The care partner should reply in a similar manner using all five senses to make a connection:

Touch – feel different fabrics, identify shapes by touch, give lotion hand massages, identify items in a bag by touch, visit animals, sculpt, hold the person’s hand;

Sight – brightly colored pictures to look at together, photo albums, paint with watercolors, go bird watching, sit at an open window;

Sound – particularly music (and personally meaningful music-the movie Alive Inside shows this very movingly), traditional or native language music, poems, whistling, singing and humming are all helpful;

Smell – baking (cookies are always great), aromatherapy with essential oils, flowers, grass clippings, fragrant lotions for hand massages;

Taste – favorite foods, popsicles, flavored drinks, ice cream.

At all stages of disease the care partner needs to understand and accept what can’t be changed. The person receiving care retains a sense of self, despite the many losses caused by dementia. You are visiting their world — join them there to make a connection. Always treat the person as an adult, worthy of respect and empathy. Try and decode what need the person is trying to express and help meet that need with soothing and calming words and actions. Recognize the effects of our moods and actions on the person receiving our care.