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.



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.

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.

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.

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.

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 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:

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.



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

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
– 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 (
* 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

* 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. 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

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
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

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)

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?

“The bad news: I don’t have Parkinson’s”

Last week in the Birmingham News, a journalist named Mike Oliver revealed that he was recently given a diagnosis of Lewy Body Dementia (LBD).  He had previously written in September 2016 that his diagnosis was Parkinson’s Disease.  But new symptoms pointed to LBD.

Mike uses the term “Lewy body disease” and describes the condition as being “like Parkinson’s and Alzheimer’s combined.”
He says:

“The disease works and presents like Parkinson’s. In both cases, these proteins kill neurons. It’s just that with Lewy body disease the proteins are killing neurons over a wider swath of the brain. … The big difference is that with Lewy body disease, the dementia comes early, sometimes before any movement disorders are noticed. With Lewy body disease, dementia is often accompanied by hallucinations and REM behavior disorder, which cause people to physically act out their dreams, hitting, kicking or talking.”

Here’s a link to the full article:–_literally.html

The bad news: I don’t have Parkinson’s.
Birmingham News
By Mike Oliver | [email protected]
on February 20, 2017 at 12:04 PM



Blogger who has Lewy Body Dementia

Recently a gentleman named Robert Bowles spoke on a webinar about Lewy Body Dementia (LBD).  Robert is a retired pharmacist, who began having neurological symptoms in 2010.  After seeing several physicians, he was diagnosed with LBD in June 2012.  He lives in Thomaston, GA.  In January 2015, he created a website called “Lewy Body Dementia Living Beyond Diagnosis” (  He seems to post to the blog once a month though he hasn’t posted since December 2016.

Here’s his message on the home page of his website.



Robert Bowles
Lewy Body Dementia Living Beyond Diagnosis

This website has been created out of a deep love that I had for patients in my pharmacy practice, families, care partners, and others.  My prayer is that with me having a strong foundation in faith as a result of having two Christian parents, having a Mom and Dad with dementia and now with my diagnosis of Lewy body dementia that I will be able to offer insights from multiple perspectives as it relates to dementia.  The resources that are provided should help persons with dementia, families, care partners and others have a better understanding of what it is like to live with dementia and how to care for those living with dementia.  I have a deep passion for better education and understanding for care partners.

As you visit this website, you will be coming into my world of living with Lewy body dementia (LBD).  My goal is to transparently share my experiences with this horrific disease.  I will share the good, the not so good and the ugly in my personal blog.  In my faith blog, I will share how my faith has been my foundation and helped me the most in living with LBD.  There may be things that I write about that you might think are impossible and things that you do not agree with; but, remember you have come into my world with LBD.

June 2012, I received a diagnosis of dementia with Lewy bodies (LBD).  Actually, this gave me peace of mind because I had visited 7 previous physicians and had not received a diagnosis and treatment that was bringing closure for me in regards to the health issues I was experiencing. The peace that I felt was so great that I told my wife 10 minutes after diagnosis, “It is what it is and everything will be OK”

Soon after diagnosis though I begin to think about a patient of mine in my independently owned pharmacy who died with LBD.  I thought about the problems and difficulties that he and his family experienced as it related to his diagnosis.  It seemed as though my death sentence had been issued.  My thoughts were that my life seemed like that of a convicted murderer.  A physician had entered the charges against me, a trial had taken place, I had been found guilty and a judge had announced the sentence — without appeal.  During my initial research on LBD, I found reports of persons living from two to twenty years after diagnosis.

These statistics were not encouraging.  I decided, however,  that I was not a statistic. I acknowledged that I was a human being; born through a miraculous process and created by God with certain inalienable rights.  My decision to live and outdistanced the statistics was one of my God-given rights.  The decision did not mean that I would not have battles, trials and tribulations along the way.  Maintaining the desire to live has been a struggle at times. As I face the many struggles, I rely on my faith in God.  One of my favorite verses of scripture has been a constant source of encouragement to me knowing that I do not travel this journey alone.  Phillipians 4:19, “And my God will meet all your needs according to the riches of His glory in Christ Jesus”.  Also, my wife and entire family are with me every step of the way.

My choice to live with LBD well, drove me into dementia awareness and advocacy.  I wanted others to know that there was life after a diagnosis.  Realizing there was very little information that chronologically sets forth someones path with LBD, I wanted to share mine with the prayer and hope that others with LBD and their care partners might receive benefit.

I wanted others to know there was no stigma in having dementia. I dream for the time when there will be no stigma in having dementia.  I dream for a cure of dementia.  I dream of persons with dementia being better educated about their disease.  I dream of better education for care partners.  I dream for better education and understanding about dementia for family and friends.  I dream of memory cafes in every town and city.  I dream of dementia friendly businesses so that persons with dementia will not isolate because of how that might be talked to.

Individuals with dementia can still do a lot of things.  We all need to be getting this message across.  We just have to accept our diagnosis, find our new purpose and develop strategies of living with our disease well.

There is a lot of work to do in dementia advocacy and helping others understand there is life after diagnosis.

(c) 2015, Robert Bowles

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).

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.



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.

Difference between LBD and AD, and how is LBD diagnosed?

The Mayo Clinic has a large clinic in Rochester, MN. The local newspaper in Rochester has a health column where questions are occasionally answered by Mayo physicians. Here are recent questions where Mayo Jacksonville dementia expert Neill Graff-Radford, MD answers. The questions are — What’s the difference between Lewy body dementia and Alzheimer’s, and How is Lewy body dementia diagnosed?

Here’s a link to the article:

Many with Lewy body dementia also have Alzheimer’s disease
by Tom Jargo
Post Bulletin
Feb 6, 2017


Comprehensive Approach to DLB – Notes from Webinar for Healthcare Professionals

In December 2016, the Lewy Body Dementia Association ( hosted a one-hour webinar with Brad Boeve, MD, behavioral neurologist at Mayo Rochester, on the topic of management of Dementia with Lewy Bodies. Note that Dr. Boeve’s presentation was tailored to healthcare professionals.

Dr. Boeve detailed the pathology, neurotransmitters, six symptom groups (cognitive, sleep, autonomic, senseory, motor, and neuro-psychiatric), medications, disease progression, and current research. The webinar ended with a short question-and-answer period.

The webinar was recorded and the video is available on the LBDA’s youtube site:

Comprehensive Approach to DLB Management
Speaker: Brad Boeve, MD
LBDA Webinar, December 5, 2016

Note that Dr. Boeve uses the term Dementia with Lewy Bodies (DLB) throughout his talk. DLB and Parkinson’s Disease Dementia (PDD) are part of the spectrum of Lewy Body Dementia (LBD).

I watched the webinar, and thought it was terrific.

In my opinion, the best graphic is the busy slide shown at the 35:33 time marker. It lists “features” (symptoms) such as cognitive impairment, neuro-psychiatric features, parkinsonism, etc. And it lists medication classes such as AChEIs, memantime, SSRIs, etc. Dr. Boeve indicates whether there is optimism or caution about each medication class for each symptom.

Brain Support Network volunteer Denise Dagan took notes during the webinar but she thinks it’s faster if everyone listens to the webinar! I’ve copied her notes below.



Denise’s Summary of

Comprehensive Approach to DLB Management
Speaker: Brad Boeve, MD
LBDA Webinar, December 5, 2016

Dr. Bradley F. Boeve, MD, started with an overview of Dementia with Lewy Bodies (DLB): Lewy bodies are mis-folded alpha synuclein protein. In DLB, they are found in the brain, brain stem, spinal cord and, in some people, the retina, heart, intestines, bladder, sex organs. They affect nerve-cell structures throughout the body. There are no FDA-approved medications to treat DLB.

This ven diagram is a good visual of how symptoms overlap and, therefore, the complexity of treatment.

[Editor’s note: see webinar!]

Dr. Boeve asks DLB patients and caregivers to prioritize the top three most troublesome issues they seek to change at each appointment. When they resolve #1 to a satisfactory degree, #2 often moves up to the #1 position and, together they keep working through symptom relief, rather than tackling everything at once and confusing what’s a symptom and what’s a medication side-effect.

Brain-Behavior Relationships:

Dr. Boeve spoke with brain diagrams about these neurotransmitters and the part of the brain in which they are typically active. In DLB, they find the cells in these areas are living, but for the most part, are not working properly, so medications are helpful to support functioning.

He then went through each symptom group in detail with corresponding treatment options. General notes for each symptom group are above the charts, while notes on specific symptoms are in the ’Notes’ column of each chart.

Cognitive symptoms:

Cognitive symptoms are primarily the result of depletion of acetylcholine. Reductions in other brain chemicals contributes to cognitive impairment, as well as some degree of neuron cell loss.

Start with education & counseling to understanding what’s at the root of these symptoms. Non-medication approaches like, cognitive rehabilitation or occupational therapy (OT).

Neurophychiatric issues:

No medication is needed unless hallucinations are frightening, upsetting, or leading to delusions.

NEVER USE HALDOL, THORAZINE, MELLARIL, and others in this class of drugs.

Motor issues:

Start with physical therapy (PT), gentle exercise, dance, a personal trainer at the YMCA, etc. – as long as balance is okay.

Treating Autonomic Dysfunction

This chart from Dr. Boeve shows several groups of prescription medications and the symptom groups they are often prescribed to treat. Because medications tend to have side-effects (some serious), the chart uses a color scale with those medications having the greatest effectiveness and few ill-effects in dark green and those with the least effectiveness and most ill-effects in orange. Your doctor should have all this information, but its a good visual.

[Editor’s note: see webinar!]

Best advice is to start slow, add one at a time, gradually adjust dose and evaluate the benefit against whether it is making something else worse.

The bright side is that if you try something and you don’t see a benefit, back off that one, change course, try another with the same caution.

It’s worth the effort.

He then went on to talk about the progression of the disease, the direction of research and, finally, questions & answers.

Progression of DLB can be 1-2 years or 10-15, or more, years. No idea why the range is so variable.

Current medications treat symptoms, but none show impact on rate of progression.

Research into Rx to slow progression, delay the onset so person dies before developing symptoms, or prevent DLB, altogether. Dr. Boeve believes medications to slow progression or delay onset will be available in the next 3-5 years. Biggest obstacle to research is lack of clinical trial participants, so get involved, of you can.

“Prodromal DLB” is defined as having early features of DLB


Q: Nuplazid?
A: Nuplazid is a new FDA-approved Rx for hallucinations & delusions in Parkinson’s disease. No evidence into the efficacy, safety, tolerability for people w/DLB. Currently, insurances are not covering this, so very expensive.

Q: When is it reasonable to consider discontinuation of cholinesterase inhibitors?
A: No right or wrong way to proceed. Most people, in later stages of illness, have no neurons making acetycholine so there is little effectiveness of the medication. Some people have increased cognitive impairment or hallucinations so doctors are inclined to maintain Rx to avoid the few who have negative effect of stopping the medication because there is no ill effects from taking it.

Q: Marijuana?
A: No research because illegal so no FDA approval. Medical marijuana prescription to treat pain in this doctor’s experience was ineffective, but no data pro or con.

Q: Pain management for arthritis, etc. when over the counter isn’t helping?
A: Ask your neurologist for prescription strength non-steroidal anti-inflammatories, other prescription with opioid effects. Use non-opioids first, then SNRIs have some pain modulating properties. Work with your doctor and keep trying!

Q: What’s in the pipeline with regard to monoclonal antibodies?
A: Monoclonal antibodies directed toward mis-folded proteins is a leading area of research for all age-related degeneration diseases. Phase I & II trials currently, but in the next 1-3 years will be in phase III trials. Challenge is that companies working on parkinsonisms find Parkinson’s disease patients easiest to have access to, so not as much research directed to DLB.

Q: Are seizures common? Are they part of DLB? What to do if they happen?
A: Relatively uncommon (less than 10% have one, or more) but disturbing to CGs and injuries can happen. Medications often have cognitive side effects, so if it is an isolated incident your doctor is unlikely to medicate, but if seizures are regular they will prescribe something, depending on the person’s medical history.

Q: Does the prevalence of lewy bodies in the gut help to understand DLB?
A: Current thinking is that alpha synuclein changes begin around the nasal cavity and along the brain stem from nerves coming from the stomach. Could it be a virus, toxin, or something ingested that starts the process of eventual changes in the brain stem. Avoiding this, if they could find what it is, would prevent infection, if the theory is accurate. Currently NO idea what that could even be!

Q: What are the milestones of the stages of DLB?
A: In recent research, MRI scans show if there is not atrophy in the brain, progression could be slower, but predicting course or progression is difficult, if not impossible.

Q: Is loss of speech communication related to muscle control or cognitive degeneration?
A: The cause is probably both. Use speech therapist because there are physical impairments that can be compensated for with training. Motor control could improve with a dopamine agent.

“Risky drugs: Antipsychotics, dementia can be lethal combination”

This article from the Pittsburgh (PA) Tribune-Review is about a gentleman with Lewy body dementia being given Haldol, an antipsychotic, at a hospital. He was agitated and the nurses wanted to calm him before a CT scan. Within ten minutes of receiving the injection, the gentleman experienced fever and seizures. He died several days later from neuroleptic malignant syndrome.

Here’s an excerpt from the article:

“‘[Antipsychotics] get used a lot in dementia despite the black box warning,’ said Dr. James Leverenz, director of the Cleveland Lou Ruvo Center for Brain Health and chairman of the Lewy Body Dementia Association’s scientific advisory council. Few other drugs are effective in treating psychosis, Leverenz said. He said he reserves the drugs for patients suffering the most acute psychosis. For example, he might prescribe them if a patient is calling police to report hallucinated break-ins at his or her home, he said. Leverenz said he opposes using the drugs to treat agitation except in very severe cases.”

Here’s a link to the full article:

Health Now
Risky drugs: Antipsychotics, dementia can be lethal combination
by Wes Venteicher
Saturday, Feb. 11, 2017, 1:24 p.m.

Hopefully everyone in the Brain Support Network knows about the dangers of antipsychotics in those with dementia and in those with parkinsonism!


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 (, 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.” (

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:

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.



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.


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.”

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.

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.

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.

“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 ( 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:

Delirium Makes its Own Mark on Cognitive Decline
03 Feb 2017