“Things I have learnt” (message to caregivers)

Though this information was found on a PSP-focused online support group, I don’t think the content is PSP-specific.  I think all of the caregivers within Brain Support Network will find some value here.

The PSP Association — the UK-based advocacy organization in Europe for PSP (progressive supranuclear palsy) — hosts its online support forum with the website HealthUnlocked (healthunlocked.com).  A couple of days ago, a woman whose husband recently died with PSP, posted about “the things I have learnt.”

With the author’s permission, I’ve copied excerpts from her post below.  She asked that I remove her name and husband’s name from this post.  And she requests that no one contact her.

Her messages are for all caregivers.



Excerpts from post
The Things I Have Learnt
HealthUnlocked’s PSP Community
January 11, 2017

The things I have learnt? I suppose my main message is look after yourself. Those last few days, I did not take part in one bit of [my husband’s] personal care. I spent it, being his wife, loving him, snuggling up beside him in bed, telling him I loved him. It made it very special. In hindsight, that wonderful thing, I should have got others to take more of this responsibility off my shoulders, a long time ago. Spent more time, sitting and talking with him, not rushing around, in a mad whirl, trying to keep everything perfect, getting so, so tired, therefore, cross and being in total state of exhaustion, all the time. We all think, “nobody can look after their loved ones as well as me.” That I have to say, is rubbish!!! 99% of Steve’s carers, loved him and when I let them,(!!!!) could do everything, just as well as me, with the added bonus of a smile of their faces! The uniform of a Carer, takes away the embarrassment, that we all think, our loved ones suffer. This is my only regret, I tried to struggle, far too long, on my own.

The weeks [my husband] had in respite, helped in giving me a break, which meant I was able to carry on, that extra bit longer. Of course I felt guilty at the beginning, but now, oh, it was such a benefit to us both. Still, I am receiving the added bonus’s of it. I am use to being in the house on my own. I can go out with others, for a drink or a meal. I am able to walk into a supermarket and buy food just for me, without crying. I’m sure there will be moments, but not yet.

The hardest part of PSP? NOT now! I think it was more the middle bit, when his bladder stopped working, the constant clearing up of Urine. Falling. The long, slow loss of communication and the realisation, that PSP would win in the end. At the moment, it’s still a huge relief that [my husband] is no longer suffering from this evil disease.

You all know, I shouted, screamed, kicked and hated PSP with a vengeance. [My husband] did get this full frontal. But I won’t let myself feel guilty about this, I was just as much a victim of this illness as [my husband]. My only hope is, he understood.

I do feel very proud of myself, I looked after and cared for [my husband] until the end. Something every single one of you reading, has done, is doing and will do! Never, EVER doubt yourself, yes, you are tired, yes you are exhausted. The feeling of failure, is huge. We all feel that way, because, in the end, PSP does win. Its not because we are useless!

Copyright 2017, HealthUnlocked

Notes from “Pain in Parkinson’s Disease” webinar

Though this webinar was focused on Parkinson’s Disease, nearly all of the information shared applies to those within Brain Support Network.

Last Tuesday, the Parkinson’s Disease Foundation (pdf.org) hosted a webinar on “Pain in Parkinson’s Disease.”  The speaker was Dr. Jori Fleisher, a movement disorder specialist with NYU Langone.

The slides from the talk are here:


You can view the recording — which includes the 45-minute formal presentation plus a great 20-minute Q&A — here:


You’ll have to register first.  Registration is free.

Brain Support Network super-volunteer Denise Dagan listened to the webinar and wrote up some highlights.  Of course you can find lots more details by viewing the slides or, better yet, by listening to the recording.  See Denise’s notes below.



Notes from Denise Dagan

Pain in PD
Parkinson’s Disease Foundation Webinar
January 10, 2017

Dr. Jori Fleisher spoke for 45 minutes + 20 minutes of Q&A on these points:

Pain is common, under-recognized, under-reported, detrimental and manageable as a non-motor symptom of Parkinson’s disease.  By detrimental, she meant pain can keep you from exercising, thereby worsening stiffness, contractures, and balance, potentially falling and resultant injury.

Early, asymmetric stiff or painful shoulder (hip or knee) is a common, often misdiagnosed presenting symptom of Parkinson’s disease.  Talk with your neurologist or movement disorder specialist before you get surgery.

There are four categories of pain.  More than one may be present in Parkinson’s disease.

1. Musculoskeletal pain is most prevalent (45-75% of patients) and involves muscle cramps, tightness (especially in the neck), paraspinal (on either side of the spine), or joint pain (distinct from arthritis in unilaterality & lack of inflammatory changes).

2. Dystonic pain (8-50% of patients) is caused by both sides of a limb’s muscles spasming simultaneously.  It can occur early in Parkinson’s disease, even as a presenting symptom, or as a complication of treatment, either as an early morning off-dystonia or at the peak of medication effectiveness, especially in the neck and face.

3. Radicular or Neuropathic pain (5-20% of patients)

* Radicular pain is caused by a pinched nerve due to a herniated disk in the spine which may be due to postural abnormalities or dystonia.  Physical therapy should be tried to remedy those postural changes brought about because of Parkinson’s disease.

* Peripheral Neuropathy refers to the bottoms of feet or fingertips and occurs more often than expected in Parkinson’s disease.  It is potentially related to dopaminergic therapy.

4. Central pain (10-12% of patients) is hard to describe, vague, constant, not localized to a specific nerve distribution.  It may have autonomic or visceral character in some Parkinson’s patients and present as reflux, labored breathing, or feeling flushed in the oral or general areas.

In communicating your experience of pain to your neurologist, consider using the OLD CARTS reference to be thorough.  Doctors can’t help at all if they don’t have specific information.  OLD CARTS stands for:

* Onset  (when did it start?)

* Location  (where does it hurt?)

* Duration  (how long does it last?)

* Character  (how does it feel?  Sharp, tired muscle, nauseating, etc.)

* Aggravating and alleviating factors  (exercising, resting, pain killers, next PD med dose, postural changes?)

* Radiation  (does it travel along the nerves from the point of origin?)

* Timing  (especially in relation to when you take your meds., mornings, after exercise or prolonged sitting)

* Severity  (completely pain free or child birth on a scale of 1-10)

Pain management in Parkinson’s disease requires attention to timing, quality of the pain, and relation to medication doses.  So, keep a diary of when you actually swallowed your medication and answer all the OLD CART questions in your diary with respect to your pain.  This should give your doctor enough information to determine how to help.

Multidisciplinary, customized approach to each patient’s pain should include:

– Physical therapy and exercise to improve mobility, prevent contractors, maintain range of motion.

– Pharmacotherapy tailored to the particular pain type(s).  May require adjusting Parkinson’s medications and/or adding anti-inflammatories, anti-depressants, anti-epileptics, or opiates, depending on the type and cause of pain.

– There are no proven benefit for medical marijuana or other alternative treatments (yet).  In fact, the effects of using marijuana include low blood pressure, dizziness, hallucinations, sleepiness, and confusion, which are similar to Parkinson’s symptoms and Parkinson’s medication side effects, so marijuana is not a recommended alternative therapy for any symptoms of Parkinson’s disease, including pain.

2017 Brain Support Network Caregiver-only Support Group Meeting Dates Set

One of Brain Support Network’s three missions is to coordinate the Northern California caregiver-only support group for those who have family members or loved ones with a diagnosis of one (or more) of four disorders:

  • LBD (Lewy body dementia).  This disorder is also called Dementia with Lewy Bodies or Parkinson’s Disease Dementia.
  • PSP (progressive supranuclear palsy)
  • MSA (multiple system atrophy)
  • CBD (corticobasal degeneration). This disorder is also called corticobasal syndrome.

These four neurodegenerative conditions have much in common.

We also welcome those who have family members with an atypical parkinsonism or Parkinson’s Plus diagnosis.

We have established our 2017 caregiver-only support group meeting schedule.  As always, we will be having nine meetings this year.

If you’d like to be added to the support group meeting reminder email list, please contact us and let us know what disorder you are coping with and which loved one has the diagnosis.


All caregivers are invited — primary, secondary, those giving hands-on care, those managing care, and those giving emotional and informational support.

Newcomers, casual visitors, and longtime attendees are all welcome!

Former caregivers — those whose loved ones have already passed away — regularly attend; these people have been through it all and are invaluable resources to those learning to cope.

If you are an active caregiver with a loved one at home, consider asking for a “respite care grant” from your county’s agency on aging or from your local caregiver resource center (see caregiver.org/californias-caregiver-resource-centers).  Such grants pay for a caregiver to be in your home while you attend support group meetings.  The local chapter of the Alzheimer’s Association (alz.org/norcal) also offers respite grants for those dealing with dementia.

We occasionally have guests.  Guests have included a neurologist, a family consultant from Family Caregiver Alliance, the president of the board of the Lewy Body Dementia Association, the co-founder of the LBDA, and the moderator of the PSP Forum.


We generally sit at one very large table, grouped by disorder.  For the last several years as our numbers have grown, some of the meeting regulars (one or two for each disorder) have become discussion facilitators.  They include:  Phil, Cristina, Ellen, and Sharon (PSP), Candy, Karen L., and Lily (MSA), Dick, Mindy, Val, and Cheryl (CBD), and Dianne, Alexa, and Bari (LBD).

We manage a lending library where books, DVDs, and videos get passed around.  And often at meetings group members bring items to give away.


At the beginning of 2012, BSN Board member Phil Myers suggested distributing a sign-up list so as to facilitate sharing contact info for anyone interested.  Phil emails out the updated directory after each meeting; it includes all the people who have attended a meeting in the past three years.  For privacy reasons, only those who are on the list may receive a copy.


One week before each caregiver support group meeting, we send out an email reminder and ask for RSVPs.  This reminder comes from the “BSN Support Group” email address.

If you’d like to be added to the support group meeting reminder email list, please contact us and let us know what disorder you are coping with and which loved one has the diagnosis.


If you don’t live in Northern California or aren’t able to attend our meetings, feel free to join our email lists.

Also, check out our webpage about online or phone-based support groups.

Support is critical!



Very brief overview of five Parkinsonian syndromes

On Kaiser’s website, there’s a very brief overview of five Parkinsonian syndromes – progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), and vascular parkinsonism.  The first four are the focus on Brain Support Network’s local support group and educational materials.

I’ve copied the short overview below.  It’s odd that there’s so little on PSP yet there’s a lot on CBD, which is quite rare.
Parkinsonian Syndrome
Kaiser Northern California Website

Up to 20 percent of patients with clinical features resembling Parkinson’s disease actually have a Parkinsonian syndrome. They can be referred to as “atypical Parkinson’s” or “Parkinson’s plus.” These syndromes are clinically differentiated from “classic” Parkinson’s disease because they respond less reliably to medications and tend to progress more quickly.

Symptoms and Diagnosis
We might suspect that you have atypical Parkinson’s if you:

* Do not respond robustly to levadopa or dopamine agonists.
* Do not have a significant tremor.
* Experience early loss of balance.
* Have disproportionate rigidity.
* Experience prominent early speech difficulty and/or difficulty swallowing.
* Exhibit symptoms that progress rapidly.

We make a diagnosis based on your history, symptoms and physical examination. There are no tests or imaging studies to confirm a diagnosis.

Progressive supranuclear palsy (PSP)
PSP is characterized by early onset of balance problems, frequent falls, and (eventually) eye movement problems.

Corticobasal degeneration (CBD)
People with corticobasal degeneration experience nerve cell loss and shrinkage (atrophy) throughout the brain.  CBD is a rare progressive disorder marked by the following symptoms:

* Rigidity.
* Cramping and spasms of limbs that lead to painful unnatural positions.
* Uncontrolled movement of limbs combined with a feeling that the limb does not belong to you.
* Sudden muscle jerking.
* Early cognitive impairment.

Multiple system atrophy
Multiple system atrophy (MSA) is a neurodegenerative disease characterized by a variety of symptoms affecting movement, blood pressure, and other body functions.  Hence the label “multiple system” atrophy. All patients will exhibit some motor symptoms similar to Parkinson’s disease. However, most patients will develop additional symptoms.

In the Shy-Drager form of MSA, the most prominent symptoms are blood pressure control and urinary dysfunction. The Striatonigral Degeneration form of MSA is characterized by severe rigidity and may not respond to typical dopamine medications. The Olivopontocerebellar Atrophy form of MSA causes increased lack of muscle coordination, balance problems and speech difficulties.

Dementia with Lewy bodies
Lewy bodies are abnormal collections of protein that develop inside nerve cells. In Parkinson’s disease, patients do have Lewy body formations but only in select areas of the brain. When Lewy bodies are found throughout the brain, the patient is given the diagnosis of Dementia with Lewy Bodies.

Lewy bodies can only be confirmed by a post-mortem biopsy of the brain. For this reason, we use the patient’s clinical history to make a diagnosis. Symptoms we look for include fluctuating confusion, visual hallucinations, rapid cognitive decline and some motor symptoms of Parkinson’s disease. Patients with Dementia with Lewy Bodies do not respond to dopamine medication.

Vascular Parkinsonism
Multiple small strokes deep within the brain can cause symptoms that are similar to Parkinson’s disease. Patients with this disorder are more likely to experience difficulties with walking rather than tremor and they are more likely to have symptoms that are worse in the lower extremities than upper extremities.

Treatment focuses on preventing further strokes. Anti-platelet agents, such as aspirin, anti-cholesterol medications, along with treatment of high blood pressure, healthy diet and exercise, and cessation of smoking can significantly reduce the risk of future strokes.

“Pain in PD” webinar, Tues, Jan 10th, 10am CA time

This Parkinson’s Disease Foundation (pdf.org) webinar on Tuesday January 10th may be of interest to those in Brain Support Network who are coping with pain.  The slides for the webinar are already posted to the PDF website.  If you are interested in the webinar but cannot attend live, I encourage you to sign up.  Within a few days of the webinar, you will receive an email giving you a link to the recording.

Details are below.


Parkinson’s Disease Foundation (PDF) Webinar

Pain in PD
Tuesday, January 10, 2017
Start time:  10am California time
Duration:  One hour

Do you experience pain as part of your Parkinson’s disease (PD)? Are you looking for ways to better manage it? Learn more by joining a one-hour online seminar led by PDF and Jori E. Fleisher, M.D., M.S.C.E., Assistant Professor of Neurology and Population Health, The Marlene and Paolo Fresco Institute for Parkinson’s and Movement Disorders at NYU Langone.

Learning Objectives:
* Understand how common pain is in Parkinson’s disease
* Be able to identify and describe the different types of pain commonly experienced in Parkinson’s
* Learn about current treatments for pain in PD, including both pharmacological and non-pharmacological options

Sign Up Now:

Preview Seminar Slides (may take several minutes):

Problems families dealing with dementia may face

I recently stumbled across the website, patient.info, which is a resource for UK physicians about various disorders.  The website contains a helpful page about caregiving for someone with dementia.  Though it is written with physicians in mind (ie, how physicians can be supportive of families), I think the page provides a good summary for laypeople as well.

In addition, I think much of this article applies to caregivers who are NOT dealing with dementia.  Much of the information and advice is generic.

Here’s a link to the full article:


Supporting the Family of People with Dementia
Patient, a UK organization
Last reviewed May 2014

Note that in the UK “caregivers” are referred to as “carers.”  (It’s a superior term in my mind.)


PSP Overview from UK Medical Professional Reference Website

Today, I stumbled across a medical professional reference website for UK doctors, called Patient (patient.info).  The overview of PSP seems rather up-to-date even though it was last checked in January 2014.  (There was some out-dated info on davunetide.)  These topics are addressed — diagnosis, epidemiology, differential diagnosis, presentations, management, complications, prognosis, and references.  Though the language is technical, many within Brain Support Network will have no difficulty understanding the text.

Coincidentally at Sunday’s local support group meeting, we discussed the two clinical subtypes of PSP.  For more info on these two subtypes (and several less common ones), see the Brain Support Network website, brainsupportnetwork.org.

Copied below are a few excerpts.



Progressive Supranuclear Palsy
Synonym: Steele-Richardson-Olszewski syndrome

Progressive supranuclear palsy is a neurodegenerative syndrome which was first described in 1964. It affects cognition, eye movements and posture. Characteristics include supranuclear, primarily vertical, gaze dysfunction accompanied by extrapyramidal symptoms and cognitive dysfunction. The cause is unknown.

Progressive supranuclear palsy is the most common atypical Parkinsonian syndrome. There are two main clinical subtypes:

* Richardson’s syndrome: prominent postural instability, supranuclear vertical gaze palsy and frontal dysfunction.

* PSP-Parkinsonism (PSP-P): asymmetrical onset, tremor and moderate initial therapeutic response to levodopa.

Expert Physical Therapist – Fall Prevention, etc. (Highlights of 48-min Video)

This email is of general interest to those in our Brain Support Network.

Though this talk by a physical therapist was delivered at a CurePSP family conference in Canada (mostly PSP and CBD families in the audience), all of the disorders in our BSN group will find value in this talk.

The speaker is Joellyn Fox, DPT, a movement disorder specialist physical therapist at the University of Pennsylvania.  Her focus is offering practical tips to preventing falls and keeping moving to “improve everyday life.”

You can find the December 2015 talk by Joellyn Fox, DPT, on YouTube here:  (48 minutes)


BSN volunteer extraordinaire Denise Dagan recently watched the video and wrote down a few highlights, which are below.  Denise encourages everyone to watch the video, however!



Methods to Improve Everyday Life
By Joellyn Fox, DPT, University of Pennsylvania
CurePSP Family Conference, Canada
December 4, 2015
48 minutes

Highlights by Brain Support Network volunteer Denise Dagan

Ms. Fox prefers to begin working with people the moment they realize there has been a change in their movement.  She calls this ‘prehabilitation.’  Clever, but her point is that until there’s a problem we don’t think about how we move.  Once there is a problem, she believes it is best to start motor learning and conscious movement training right away to prevent loss of balance and falls.

With Parkinson’s disease and parkinsonisms there develops a motor-sensory disconnect.  To compensate for that, one needs to:
– Think before moving
– Move with larger movements
– Finish one part of a task before moving on to the next by breaking tasks into parts.

For example; sitting requires approaching a chair, turning, and lowering oneself onto the seat.  When each part is done with thought, the risk of falling, sitting on the arm, or dropping onto the seat is minimized.

People with Parkinson’s often feel as if their ability to move has been lost, particularly when they suffer from freezing.  In actuality, it is a problem of activating the motion due to motor-sensory disconnect.  Compensating for this is primarily done through the use of cues to activate movement and improve gait quality.

Cueing the activation of movement can be internal (mental reminders, imagination) or external (verbal, visual or audible reminders, signs around the house, etc.).  Examples include:

– tape on the floor: to line up in front of a chair or bed before sitting, to step over going through doorways, etc.

– laser lines: light is emitted from the bottom of a cane (e.g. UStep laser cane) to break freezing and increase stride

– metronomes: help overcome freezing and maintain a regular pace or prevent slowing of stride.

Free apps are available for smartphones, even blue tooth so others can’t hear them.

– imagination: pretending to step over or on something that isn’t there (like step on a bug or over a threshold).

– tactile stimulation: tapping on the foot that’s frozen can often get it moving, or tapping on the side that needs to take the next step.

– RAS (Rhythmic Auditory Stimulation): People tend to step in time with music, whether they have Parkinson’s disease, or not.

Ms. Fox’s FOG (Freezing of Gait) Strategies start with the 4 S’s:
– Stop
– Stand tall
– Shift weight
– Step big

The key is to unweight one foot and make the next step a big one.  The cue in a physical therapy appointment is her saying, “Stop,” but remembering to say it to yourself is an internal cue, which doesn’t work for most people.  A solution at home can be STOP signs around the house where freezing often occurs (an external cue).

The rest of her FOG Strategies include:

For areas in the home (doorways, around furniture, etc.)
– Destination Estimation: estimate the number of steps to a destination and try to keep to just that many.  Works well for elevators because there is some anxiety over beating the door close.  Estimate 4 steps, take them big, and you’re in!
– Look through a doorway to the wall ahead.
– Stop, and step big over the threshold

For crowds (elevators, escalators, church, concerts, trains & buses)
– Allow others to go ahead of you to blaze a trail, and follow in their wake
– Move to the side of the crowd or against a wall and follow around the edge of the group

Turns require learning new techniques because you’re probably used to leading with your shoulders, but that causes legs to cross and may result in falling.  Instead, Ms. Fox offers specific techniques for maintaining balance while turning in both open areas and tight spaces.

Ms. Fox also spoke about retropulsion, backward balance loss with reduced step size and increased cadence, and festination, forward gait with increased step cadence, a.k.a. “runaway train.’  She listed several specific triggers and preventions for both.

Ms. Fox made the point that moving doesn’t have to be boring or tedious, even during your physical therapy appointment.  You want a therapist with that same attitude.  Her last slide has several research based movement options shown to benefit people with Parkinson’s disease.  Pick some and keep moving!

Lastly, Ms. Fox offers some specific tips for caregivers to to help steady someone with Parkinson’s disease while walking together, as well as minimize quarrels about nagging over posture or gait.

To find a physical therapist close to you, start with these resources:

– Look for therapists with experience working w/people who have Parkinson’s disease & parkinsonisms

– National Parkinson Foundation : Centers of Excellence

– LSVT BIG Certification (Lee Silverman Voice Treatment, limb movement training)

– Parkinson’s Wellness and Recovery

Treatment/Management of PSP – Facebook Live, Mon., Dec. 5, 3pm PT

It looks like CurePSP has abandoned the webinar format and is opting for the Facebook Live format. The next event is as follows:

Monday, December 5, 3pm PT
Perhaps one hour in length
Speaker: Jori Fleisher, MD, movement disorder specialist, NYU Langone
Topic: Answering questions on treatment and management of PSP

If you are a Facebook member, you can access CurePSP Facebook page (unless they’ve fixed their permissions problems in which case non-Facebook members can access the CurePSP FB page) and leave a specific topic or question you’d like to suggest be addressed.

CurePSP indicates that “Dr. Fleisher specializes in treating patients with neurological disorders, and can address such issues as dealing with non-motor symptoms, palliative care vs hospice, and simple tips to manage daily activities.”

Unless CurePSP has fixed its permissions problems, only those who are Facebook members can access its Facebook page either live or to view the recorded version.

Here’s a link to CurePSP’s FB page:


After the recorded version is posted, Brain Support Network will post the recorded version to the BSN Facebook page. We open our Facebook page to anyone, regardless of whether you are a Facebook member or not. Here’s a link to BSN’s FB page:


If the talk is worthwhile, I might post a transcript as I did with the last talk.



Educational video for neurologists – diagnosing (mostly) and treating PSP, CBS, MSA, and DLB

This 60-minute video was put together by CurePSP with the main goal to educate general neurologists on differentiating Parkinson’s Disease from the four atypical parkinsonism disorders – PSP, CBD, MSA, and DLB.  A CurePSP letter with a link to the YouTube video was sent out to 14,000 general neurologists in the US in May 2016.  Though the YouTube video was produced for neurologists, I found it understandable and think most of you will as well.

If you are seeing a general neurologist — not a movement disorder specialist and not a dementia specialist — my suggestion and request is that you share this email with the general neurologist.  Encourage him/her to spend 60 minutes to gain some additional info on diagnosis, evaluation, pathology treatment, care pathology, and prognosis of the four atypical parkinsonism disorders.

Be sure to explain to your general neurologist the resources and services of Brain Support Network — we have local support group meetings in Northern California, email lists focused on the atypical parkinsonism disorders, and that we help people nationally with all neurodegenerative diseases with brain donation.

For everyone viewing this post, my suggestion is that you skip to the last ten minutes of the video and listen starting with the medication management section.  Most of you are beyond the diagnosis so reading about the symptoms, how to diagnose, imaging, and the pathology is probably less interesting.

This video is a great service to general neurologists.  And probably primary care physicians and other healthcare professionals as well.  It’s unfortunate that the four atypical parkinsonism disorders are compared to Parkinson’s Disease ONLY but that is the context of this educational video.  It was made by movement disorder specialists.  As a point of reference, no one would’ve thought my father had Parkinson’s Disease.  Alzheimer’s Disease seemed far more likely in his case but a video that compares PSP to Alzheimer’s ONLY wouldn’t be 100% right either.  We need better “blending” of the movement disorder communities and the memory disorder communities!

I’ve copied below some notes on the 60-minute educational video.  This is definitely not a transcript.  In many places, I say “see slide.”  But the notes at least give you the time markers and some idea of what is discussed at certain times.

By the way, I’ve heard great things about Dr. Stephen Reich, the main speaker in the video.  He’s been part of the PSP/CBD community for many years.  One of the other authors of the educational presentation is Dr. Alexander Pantelyat, a movement disorder specialist from Johns Hopkins I’ve had the pleasure of meeting at conferences.  The third author of the presentation is Dr. Shawn Smyth, who shares some helpful videos of specific symptoms as part of this larger video.

If you learn anything, let me know!  Or, if your general neurologist wants to know more about any of these disorders or get involved with Brain Support Network, let me know!



Atypical Parkinsonian Disorders
60-minute tutorial for general neurologists
Produced by CurePSP, May 2016

Slide set put together by:
Stephen Reich, MD, University of Maryland
Shawn Smyth, MD, Parkinson’s and Movement Disorders Center of Maryland and Johns Hopkins University
Alexander Pantelyat, MD, Johns Hopkins


The importance of a correct diagnosis:
* avoiding repeated consultations, testing, and/or hospitalizations
* avoiding unnecessary diagnostic testing
* providing accurate prognostic information
* directing patients and families to appropriate resources/networking/clinical trials
* trying treatment strategies/care that may provide helpful

Parkinsonism is NOT Parkinson disease
* ParkinsonISM is a general term to describe movement (motor) problems that commonly appear together in certain illnesses
* The term parkinsonism comes from Parkinson’s disease, but these problems are also seen in other disordres
* The four main motor symptoms of parkinsonism: bradykinesia (slowness of movement), rigidity, tremor (rest), postural instability and gait dysfunction.
* Not everyone with parkinsonism has Parkinson’s disease though the majority do.

Differential diagnosis of parkinsonism in a flow diagram:
* 80% have primary parkinsonism or degenerative disease.  This breaks down as Parkinson’s disease (majority), atypical parkinsonian disorders, and heredo-degenerative parkinsonian disorders.
* 20% have secondary parkinsonism.  This breaks down as other brain conditions — vascular, hydroencephalic, infectious, traumatic, etc — or systemic etiologies — hypothyroidism, meds, toxins, etc.

(4:38) Degenerative causes of parkinsonism

More common presentations:
* Parkinson disease
* Atypical parkinsonian disorders
– progressive supranuclear palsy
– multiple system atrophy
– corticobasal syndrome
– dementia with Lewy bodies/diffuse Lewy body disease

Rarer presentations:  (this isn’t a complete list)
* frontotemporal dementia with parkinsonism (FTD-P)
* Alzheimer disease
* spinocerebellar ataxias (SCAs, often types 2, 3, 17)
* basal ganglia calcification (sporadic and inherited)
* Huntington’s disease (juvenile presentation)+
* Wilson disease (<50 years old)+
* acquired hepatolenticular degeneration

+ often have parkinsonism, dystonia, and tremor

Up to 25% of those who were thought to have Parkinson’s disease upon autopsy were found to have an alternative diagnosis.  The most common alternative diagnosis is another form of parkinsonism.  The most common of these are PSP, MSA, CBS, or DLB.

Degenerative parkinsonism and accumulating intracellular proteins
* In Lewy bodies: PD and DLBD
* In glial cytoplasmic inclusions (GCIs): MSA
* PSP, CBD, FTDP-17, Parkinson dementia complex of Guam

Secondary causes of parkinsonism
* Vascular: lower-half (waist down) parkinsonism; multi-infarct state; TIA history; step-wise progression; MRI scan indicative
* Hydrocephalus
* Space-occupying mass/lesion
* Endocrine (hypothryoid slowness)
* Toxic: manganese, carbon monoxide, cyanide, MPTP, carbon disulfide
* Drug-induced:  dopamine-receptor blockers (first and second generation antipsychotics; antiemetics, including Reglan); anticonvulsants/mood stabilizers (valproic acid, lithium); antiarrhythmics (amiodarone).  Most important category here.  Physicians are not very good at recognizing drug-induced parkinsonism.  This can last for a number of months, even a year, after the drug has been stopped.  It’s important to take a good drug history.
* Post-encephalitic
* Post-traumatic

(8:15) Steps to making a diagnosis
(See the slide)

Usually on PD and CBS are asymmetric.

Are there any symptoms that don’t fit with Parkinson’s?

Very important to take a careful drug history.

Atypical parkinsonian syndromes don’t respond at all to levodopa or have a short-lived response.  In PD, we are reassured that there’s a sustained response to levodopa over five years and when the patient develops dyskinesia.

(10:18) Dementia is typically a late symptom in PD.  If the symptom is early, this would suggest it’s not PD.

(10:36) PD diagnostic criteria – UK PD Society Brain Bank Criteria (Hughes et al, JNNP; 55: 181-184)
(See the slide)
Must have bradykinesia (slowness of movement)
Must have one or more of rigidity, tremor at rest, or postural instability but not at onset

In PD, postural instability usually begins 5-7 years in.

Patients with PD almost always survive ten years and often many more years than that.

(13:30) Atypical parkinsonian disorders
* Often confused with PD, AD, and other dementias
* Clinical diagnostic criteria for PD, AD, and the atypical parkinsonian disorders have imperfect sensitivity and specificity
* Diagnosis is made clinically as all diagnostic studies only support clinical suspicion
* ONLY WITH FOLLOW-UP can many “red flag” features be identified to improve diagnostic accuracy.  Ask about these at each visit.

(15:15) PD vs. atypical parkinsonian disorders (from Quinn, JNNP suppl: 78-89)

Clues for PD: asymmetric onset of movement dysfunction; significant and sustained benefits to dopaminergic medications (though tremor may not respond); classic resting tremor (or unilateral pill-rolling tremor)

Note: those with MSA may have a low-amplitude somewhat jerky postural tremor of the fingers or hand.

Clues for atypical parkinsonian disorders (PSP, CBS, MSA, DLB): fairly symmetric onset of movement dysfunction (except CBS which is highly asymmetric); poor, transient or no benefit to dopaminergic medications

Red flags to atypical parkinsonian disorders: few are absolute; may not be present at presentation so re-evaluate for these at every visit

(17:20) ALERT for Atypical Parkinsonian Disorders

ALERT is a helpful acronym for approaching these syndromes.

A= atypical for Parkinson disease
* apraxia and/or myoclonus (CBS or AD with parkinsonism)
* saccade changes (slow saccades or vertical ophthalmoplegia is PSP, delayed/apraxic in CBS, and hypermetric in MSA)
* parietal/sensory dysfunction (extinction, cortical sensory loss, alien limb in CBS)
* cerebellar or upper/lower motor neuron signs (MSA)
* certain types of dystonia or dyskinesia:
– retrocollis, blepharospam/eyelid opening “apraxia” or trunk dystonia in PSP
– anterocollis or levodopa-induced facial/oral dyskinesias in MSA
– limb dystonia in CBS or MSA
* faster progression, including “wheelchair sign” (early use of wheelchair – within 3-5 years)

L= lack of response to medications (poor, transient or no benefit to an adequate trial of levodopa – 1000mg/day).  One small exception, if tremor-predominant PD, a trial of 1000mg/day may not be warranted.

E= early (compared to PD):
* falls/postural instability (PSP)
* dysphagia, dysarthria, bulbar dysfunction (PSP, MSA)
* dementia, executive dysfunction, impulsivity, apathy, personality change, or pseudobulbar affective lability (DLB, CBS, PSP)
* hallucinations/delusions (DLB)
* autonomic dysfunction such as constipation and orthostatic hypotension (MSA)

R= refer to resources (second opinion to movement disorder specialists, physical/occupational/speech and swallow therapy – ancillary services become mainstay of treatment; CurePSP)

T= treat symptomatically (even when unsure of the diagnosis)

(23:13) Autonomic dysfunction
(See slide for a list of symptoms/features)

Patients with orthostatic hypotension may not have lightheadedness but may have fatigue, confusion, visual blurring, and the “coat hanger sign.”

Those with erectile dysfunction rarely voice this to the physician.

These are problems that can often be treated and are disabling.

PROGRESSIVE SUPRANUCLEAR PALSY – symptoms, variants, imaging, pathology

(25:01) Progressive Supranuclear Palsy
(see slide)

Bradykinesia tends to be axial.  Difficulty getting up from a chair.  Or getting back into a chair.  Tremor is uncommon.

Rigidity is axial.  Rigidity is often at neck.

Supranuclear palsy – both up and down.  Key diagnostic feature.  Slow vertical saccades – this can be found before supranuclear palsy.  (26:00 – video and audio out of synch for several seconds.)  Frequent saccadic instrusions.


Prominent bulbar dysfunction early on.

Frontal dysfunction.  Prominent apathy.  Executive dysfunction.  Impulsivity.  Applause sign – shows perseveration (from frontal lobe dysfunction); not specific to PSP; rarely seen in PD.

(28:18) PSP Variants (from Williams & Lees, Lancet Neurology 2009.  from Dickson et al, Curr Opin Neurol 2010)

PSP-Richardson syndrome: classic presentation; accounts for only approx 25% of PSP pathology; originally described in 1964 by John Steele, who is still living, Richardson, and neuropathologist Olszewski

PSP-Parkinsonism: can have tremor and partial/temporary levodopa response (similarities to Parkinson disease); more benign course than PSP-RS; nearly as common as PSP-RS

PSP-Nonfluent Aphasia
PSP-Frontotemporal dementia
PSP-Pure Akinesia with Gait Freezing
Mixed pathologies

(29:50) Imaging features of PSP
(see slide)
Hummingbird/penguin sign in midbrain
Morning glory sign

(30:24) Pathological changes of PSP
(see slide)

CORTICOBASAL SYNDROME – symptoms, variants, imaging, pathology

(31:05) Corticobasal Syndrome
(see slide)

Corticobasal Degeneration now used for pathological confirmation.  During life, we call this corticobasal syndrome.  Only about 50% of those diagnosed with CBS during life are found to have CBD upon autopsy.  30% have Alzheimer’s pathology.  20% have Lewy body pathology.

Often presents unilaterally.

Important to see a saccade.

May present with early frontal dementia.

(33:21) CBS Variants (from Armstrong et al, Neurology 2013)
PSP Syndrome
Frontal-behavioral spatial syndrome
Nonfluent/agrammatic Primary Progressive Aphasia
Mixed Pathologies (with Parkinson Disease, with Dementia with Lewy bodies)

[Robin’s note: Isn’t “corticobasal syndrome” a variant??]

(34:00) Imaging features of CBS
(see slide)

(34:13) CBD Pathology
(see slide)

MULTIPLE SYSTEM ATROPHY – symptoms, imaging, pathology

(34:37) Multiple System Atrophy
(see slide)

MSA subtypes: MSA-P and MSA-C.  All patients have to have dysautonomia (orthostatic hypotension or a combo of urinary incontinence and, with men, erectile dysfunction).

(36:15) Red flags suggesting MSA
(see slide)


Inspiratory stridor or expiratory sighs

Pseudobulbar affect

(38:00) Imaging features of MSA
(see slide)

Hot cross bun sign

(38:15) MSA Pathology
(see slide)


(38:51) Dementia with Lewy Bodies/Diffuse Lewy Body Disease
(see slide)

Symmetric parkinsonism (often no tremor) with early dementia


Sensitive to antipsychotic medication

Greater extent of Lewy bodies in brain (especially in cortex)


(40:32) Overlapping symptoms in neurodegenerative disorders
cognitive – emotional – sleep/wake cycle – autonomic – sensory – movement

(41:30) Non-motor symptoms
(see slide)

cognitive – emotional – sleep – fatigue – autonomic – sensory

These can be very disabling.


(43:00) Shawn Smyth, MD presents some videos that illustrate symptoms, which are useful during evaluation and diagnosis.

Woman with anguished look with PSP.

(43:20) Evaluating saccades in woman with PSP.  Evaluating optokinetic nystagmus.

(44:24) Evaluating retropulsion in PSP.

(44:37) Evaluating rigidity in neck and arms in PSP.

(44:50) Evaluating bradykinesia of PSP (unusual).

(45:40) Applause sign in PSP.

(46:04) Clenched-fist dystonia with irregular postural tremor in CBS.

(46:24) Asymmetric bradykinesia and lefthand dystonia in CBS.

(46:36) Apraxia in CBS.

(47:10) Squeaky hypophonia and dysarthria in MSA.

(47:29) Cerebellar signs in MSA.

(48:10) Wide cadence and irregular gait in MSA.


(48:27) Stephen Reich, MD discusses ancillary testing
(see slide)

MRI is worthwhile unless the patient seems to be classic for PD

DaT scan cannot distinguish between any of the PD and atypical parkinsonian disorders

Not challenging: distinguishing between PD and essential tremor.

Routine lab tests: vitamin B12, thyroid


(51:09) Medication management of these syndromes
* Levodopa: test 1000mg/day immediate release (optimally received on an empty stomach during the waking part of the day)
* Cholinesterase inhibitors (rivastigmine) in DLB
* Quetiapine or clozapine for hallucinations, delusions or agitation, used cautiously
* SSRIs or TCAs for depression or anxiety.  Beware of medication interactions with other antidepressant, antipsychotic, or dopaminergic.  Shy away from TCA if constipation or OH is present.
* Levetiracetam or clonazepam for myoclonus, especially in CBS
* Try non-pharmacologic approaches to orthostatic hypotension first.  Review meds that might contribute to low BP.  Adequate salt intake.  Avoid hot baths/showers.  Lots of water.  Fludrocortisone, midodrine, droxidopa, or pyridostigmine for orthostatic hypotension.  Stockings, head of bed elevation, and abdominal binders can be considered.
* Botox injections for sialorrhea, dystonia, and blepharospasm/apraxia of eyelid opening

The atypical parkinsonian syndromes are NOT untreatable.  Medication does play a small role.


(53:40) Supportive Measures
PT, OT, speech/swallow therapy
Social work/case management consultation
Support groups for patients/families, in-person or online
Palliative care


(54:18) Management of PSP
(see slide)

Trial of levodopa up to 3 months.  Taper or discontinue if unsuccessful or not tolerated.

Weak evidence for amitriptyline and amantadine.

He prefers UStep walker.


(55:22) Management of CBS
(see slide)

Try levodopa if significant bradykinesia.

Consider cholinesterase inhibitors for cognitive impairment


(55:50) Management of MSA
(see slide)

If MSA-P, judicious trial of levodopa.  Watch that orthostatic hypotension isn’t worsened.

See a urologist.

Not many therapies for MSA-C.


(56:42) Management of DLB
(see slide)

Not very responsive to levodopa but worth a trial.  Watch that mental status isn’t worsened and hallucinations aren’t caused.

Cholinesterase inhibitors can be helpful.  Worth trying them plus memantine.

Avoid antipsychotics unless necessary.


(57:32) Prognosis for atypical parkinsonian syndromes

* Quite different from PD, where there’s a normal lifespan.  Cause of death is often unrelated to PD.
* Highly variable
* Most common causes of death due to disease itself — complications of immobility (DVT, infections with spesis), dysphagia (aspiration pneumonia), or injury from falls
* Generally have a lifepsan of <10 years from onset of symptoms, with variability.  Usually 6-10 years.


(58:15) Conclusions

* Atypical parkinsonian disorders have protean manifestations and can be challenging to diagnose at the front end

* ALERT acronym:
A= atypical for Parkinson disease
L= lack of response to medications
E= early falls, cognitive impairment, personality change, autonomic dysfunction (pay close attention to non-motor symptoms)
R= refer to resources (movement disorder specialists, CurePSP)
T= treat symptoms (even if you don’t have a diagnosis yet)

* It is important to consider these disorders in all middle aged or older patients, as early detection can improve quality of life for the patient and their caregiver/family

* Supportive care and a variety of symptomatic therapies can be offered.  Pay particular attention to the caregiver throughout the disease.


(58:48) Resources

CurePSP, curepsp.org
AFTD, theaftd.org

[Robin adds: Brain Support Network, brainsupportnetwork.org – educational materials on PSP, CBS/CBD, MSA, DLB]