Category Archives: CBD

“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

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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)

“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

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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.

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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.