Dr. Boeve Webinar on PSP, CBD, FTD, and PPA – Notes

Tonight’s webinar was a great presentation but then I’m a huge fan of Dr. Brad Boeve so it’s hard for me to be objective.  I enjoyed learning about all the correlations between brain anatomy and symptoms.  The webinar’s question-and-answer session, organized by CurePSP, was not so good.

Did any of you pick up new things?  What Dr. Boeve had to say about experimental DBS (deep brain stimulation) was new to me. He indicated that it is showing some promise in PSP and for some CBS patients with dystonia.

There’s one important aspect of tonight’s webinar I’m still not sold on.  Though I’ve read a lot of articles about the clinical overlap in the four disorders discussed on the webinar (PSP, CBD, bvFTD, and PPA) and though I’ve attended an FTD caregiver education conference (when it was in SF several years ago), I don’t get the point of a PSP/CBD audience sitting through a Q&A that was nearly all about FTD.

What follows are the notes I took from Dr. Boeve’s presentation, the nearly-100%-focused-on-FTD Q&A, and a bit about Janet Edmunson’s presentation on CurePSP and Sharon Denny’s presentation on AFTD.  CurePSP and AFTD were the co-hosts of the webinar.

[Editor’s Note:  The archived webinar recording is no longer available on the CurePSP website.]


Topic:  Cognitive & Behavioral Issues in PSP, CBD, FTD and PPA
Speaker:  Dr. Brad Boeve, Neurologist, Mayo Rochester (MN)
4/29/10 Webinar Hosted by CurePSP and AFTD

Some acronyms:
PSP = progressive supranuclear palsy
CBD = corticobasal degeneration
CBS = corticobasal syndrome
FTD = frontotemporal dementia
bvFTD = behavioral variant frontotemporal dementia
PPA = primary progressive aphasia
FTLD = frontotemporal lobar degenerations

PSP and CBD have classically been considered parkinsonian disorders.  For many people, cognitive and behavioral symptoms do evolve.  On the flip side, FTD and PPA begins with cognitive and behavioral symptoms and, for many, parkinsonian symptoms evolve.  There is a lot of overlap.

PSP:  initially characterized in the 1960s
CBD:  initially characterized in the late 1960s; 20 years of no publications
PPA:  initially characterized in 1982; nonfluent variant and semantic variant
FTD:  coin termed in 1994

These four disorders have been classically considered to be distinct and separate.  But there’s growing evidence that these are strikingly similar, with one of the primary distinctions being what parts of the
brain are maximally affected.

Brain-Behavior Correlations:
* Frontal lobe (especially dorsolateral prefrontal cortex):  planning; judgment; insight; executive functions; problem-solving; multi-tasking
* Orbital medial part of brain:  socially-appropriate behavior (knowing what to say and when to say it); theory of mind; social cognition
* Left perisylvian region (frontotemporal, parietal regions):  language; verbal aspects of language
* Right perisylvian region (frontotemporal, parietal regions):  prosody
* Hippocampi:  memory (short-term memory, upcoming plans); severely affected in AD; we don’t see dense memory impairment
* Amygdala:  emotional valence; ex – seeing trash in the park vs. seeing someone sleeping in the park; memory traces of emotional events
* Anterior cingulate region:  motivation; spontaneity
* Right temporal occipital region:  visual recognition (ability to recognize objects and people)
* Bottom part of brain:  social disinhibition; loss of empathy and insight; change in food preferences (usually towards sweets); hoarding of food (especially stuffing food in mouth faster than it
can be swallowed); utilization behavior

A few more terms:
Poor verbal expression vs. Poor naming:  these are different regions
Aprosodia (monotone voice)
Visual agnosia (inability to recognize objects and people)

We don’t often seen atrophy on brain scans, but there can still be problems.  The symptoms come up because the networks are affected.

PSP – Clinical Features:  primarily subcortical parts of brain and the substantia nigra (SN is also affected in PD)
* Symmetric rigidity and bradykinesia:  axial > appendicular; levodopa unresponsive
* Postural/gait instability
* Extraocular movement dysfunction (saccadic pursuits: delayed, slowed saccades; supranuclear gaze palsy, particularly downgaze)
* Apathy (or lack of motivation is the term he prefers), bradyphrenia (the wheels are working but the gears are slow)

CBS is a clinical diagnosis.  CBD is a pathological diagnosis.  He prefers the term CBS.

CBS/CBD – Clinical Features:
* Progressive course
* Asymmetric findings
* Limb rigidity
* Limb apraxia

bvFTD – Clinical Features:
* Personality/behavioral changes
* Loss of empathy/sympathy
* Loss of insight
* Apathy
* Social disinhibition
* Change in food preferences
* Poor judgment and planning

We see the frontal-type behaviors in some with PSP and CBD.  This is because the same brain networks are effected.

PPA – Types and Associated Clinical Features:
Nonfluent:  changes in speech/language production
Semantic:  uncommon; loss of nouns and word meaning
Logopenic variant of PPA is almost always AD, with an atypical
presentation.  It won’t be covered tonight.

Some people with PSP present with florid FTD or PPA features.  Same thing with FTD.  Considerable overlap.  Many times the diagnosis changes or another possible diagnosis gets added.  This is typical.  Other symptoms evolve as other parts of the brain are affected.

FTLD = frontotemporal lobar degenerations

Management – Goals:
* Pharmacotherapy:  improve symptoms; manage behaviors; decrease injury risk (to patients – due to falls; to family members – due to behaviors)
* Non-Pharmacotherapy:  improve symptoms; manage behaviors; decrease injury risk; improve caregiving strategies

There are clearly ways to improve quality of life

Approach:  ask patients and caregivers to list and then rank which symptoms/problems are most bothersome, which allows the clinicians to tailor the management plan.  We try to focus on 3 issues to manage initially.

Problem list:  what are the most significant in these categories?

Management through Pharmacotherapy:
* No FDA-approved medications specifically develop to impact these disorders.
* There are a few well-designed studies that have shown that any medication improves symptoms in any of these disorders.
* There is considerable debate among clinicians whether any medications are worth using in patients with any of these disorders.
* Most of these medications are expensive and there are side effects.

Does your clinician’s approach fit with your approach?  (conservative, aggressive, etc)  No right or wrong way.

Non-medication management is more important that medication at the present time.  This will change in the future.

Education/Counseling/Behavioral Management of Cognitive Symptoms:
* Stay physically fit, mentally active, and socially active.  There is some evidence supporting this now.  If you are fortifying the brain networks by being active, more changes need to happen in the
brain for symptoms to worsen.  In theory, maximizing activity certainly can’t hurt and there is some evidence that it helps.
* Establish and maintain a daily routine
* Religious use of a daily planner (even for those who have memory problems!).  Mayo has a whole program on teaching people how to use daily planners.  It’s not just a matter of picking up a daily planner.  You need training.

Medication Management of Executive Dysfunction and Memory Impairment:  (Cognitive Symptoms)
* carbidopa/levodopa, dopamine agonists (Sinemet, Mirapex, Requip)
* cholinesterase inhibitors (Aricept, Exelon, Razadyne):  designed for people with Alzheimer’s; some experts will tell you that these should not be used; some clinicians think these medications are okay
* psychostimulants (Provigil, Nuvigil, Ritalin, Adderall, Desoxyn):  more controversial; some of the same circuits are involved with narcolepsy; expensive and have side effects
* Namenda:  used frequently in some of these syndromes.  Note that there is a placebo-controlled clinical trial of Namenda in the US in FTD and PPA.

Education/Counseling/Behavioral Management of Neuropsychiatric Symptoms:
* Develop tolerance and choose your battles!
* Acknowledge and redirect; refrain from arguing
* Employ therapeutic fibs:  sometimes the outcome is more important than the process; lies are appropriate in some circumstances
* Support group involvement (local support groups, even local PD and AD support groups; CurePSP; AFTD; sometimes even Huntington’s Disease support groups, if you are dealing with an inherited disorder)
* Work with local psychologists and psychiatrists:  not many are knowledgeable about these disorders

Medication Management of Disinhibition, Socially Inappropriate Behavior, Delusions:  (Neuropsychiatric Symptoms)
* SSRIs (Celexa, Lexapro, Paxil, Prozac, Prozac, Zoloft)
* atypical neuroleptics (including Seroquel, Risperdal, Zyprexa):  have gotten a lot of bad press but we feel that they have a role
* anticonvulsants (Depakote, Neurontin, Tegretol):  they do have side effects
* beta-blockers (Inderal)

Medication Management of Apathy:  caregivers view this as a big deal; clinicians don’t tend to view this as a big deal
* psychostimulants
* carbidopa/levodopa, dopamine agonists
* cholinesterase inhibitors

Management of Parkinsonism:  (Motor Symptoms)
* carbidopa/levodopa, dopamine agonists:  not very responsive to our standard agents; being aggressive with Sinemet is worthwhile
* DBS (deep brain stimulation):  this is experimental; it is showing some promise in PSP; in select cases with dystonia in CBS, DBS has provided a benefit with effects lasting for a year or two (so it’s
not prolonged)

Management of Gait Impairment and Falls (especially in PSP):  (Motor Symptoms)
* gait assistance devices and PT:  we need to do a better job counseling patients; Deena J. is a PT with considerable expertise in this area
* power-operated vehicle:  work with Medicare or other insurance

Management of Dysarthria/Nonfluent Aphasia/Apraxia of Speech:
* speech therapy:  can be helpful in some individuals; worth trying, particularly in nonfluent aphasia
* carbidopa/levodopa, dopamine agonists:  not usually helpful

Management of Dysphagia:
* swallowing study
* Thick-It, pureed food, PEG tube (no right or wrong answer)

Sleep issues can include:
* Insomnia
* Daytime hypersomnia
* Obstructive sleep apnea
* Central sleep apnea
* PLMS (periodic limb movements during sleep)

Management of Sleep Issues:
* Discuss with primary MD
* Consider formal sleep medicine evaluation and formal sleep study, in select cases
* This is a quality of life issue.  There are many sleep disorders; almost all are treatable.  Don’t overlook them.

Two primary groups of disorders:
* Tauopathies:  Alzheimer’s Disease, Pick’s Disease; CBD, PSP, AGD; FTDP-17MAPT
* TDP-43opathies:  FTLD with MND; FTLD with ubiquitin/TDP-43-positive inclusions; FTDP-17PGRN

A huge percentage of those with AD also have TDP-43.  So any research into AD will help all these disorders.

Proteins found upon brain autopsy:
clinical PSP:  nearly all have tau; some have TDP-43
clinical CBD:  60% have tau; a small percentage have TDP-43; 10-20% actually have atypical AD (amyloid)
clinical FTD:  roughly half tau; roughly half TDP-43; a few have AD (amyloid)
clinical PPA:  60% have tau; a small percentage have TDP-43; 10-20% have atypical AD (amyloid).  NFA is more often a TDP-43 problem.

[Robin’s comment on the PSP data:  but what about the people diagnosed with PSP who actually have MSA, DLB, or AD upon brain autopsy?] [Robin’s note:  I may’ve posted here on 4/11/10 or so a recent UCSF article on the overlap in PSP, CBD, and FTLD-type disorders.  The article is useful if you want to drill further into what types of
protein-opathies those clinically diagnosed with PSP, CBD, FTD, and a few other disorders actually have upon brain autopsy.]

The challenge is we don’t have a test now that reflects the underlying protein.  In PSP, it’s less of an issue because most people have a tau disorder.  But it’s a problem for the other disorders.  We need to develop better biomarkers (MRI, PET, blood test, spinal fluid test, etc) so we can treat individual patients with the right therapies.  We don’t want to give a medication to someone that turns out to be toxic if they have a certain protein.

Tauopathy therapies being studied:  (short list; there are many others)
* microtubule stabilizers
* tau kinase inhibitors such as GSK3-beta enzymes
* Hsp90 inhibitors
* tau aggregation inhibitors such as Davunetide and a Rember-derivative:  clinical trials in these two over the next 1-2 years.  Davunetide trial is international.

[Robin’s notes:  One of the GSK-3 beta enzymes is Nypta.  The Nypta trial has already started in Europe.  Hopefully it will start by June at various US sites.  Regarding Davunetide, which is also called NAP or AL-108, there’s a small 12-person pilot trial of this in PSP and CBD at UCSF now.  Hopefully the full clinical trial will begin in September.]

TDP-43 therapies:
* in a genetic form of the disorder, too little PGRN (progranulin) leads to too much TDP-43

Plea:  please consider participating in research!  When we get any of these medicines (such as Davunetide), how are we going to prove or disprove the medicines are working?  These studies are typically free.  They are funded by our tax dollars, private foundations, and organizations (such as CurePSP).

What if we had a disease-modifying agent?  Even better is a slowed progression of symptoms with disease-altering therapy!

Future directions:
* Increase awareness among the public and healthcare professionals that these disorders exist, are recognizable disorders, and can be diagnosed and managed
* Develop networks of patients, families, friends, and healthcare professionals to improve managing the day-to-day aspects of these disorders
* Increase federal and private funding for research, education and counseling.  This is where you can help!  If anyone has ties to foundations or other funding sources, let them know of your interest in research funding for medication and non-medication approaches.  Lots of people are having fundraisers.  Anything that can be done for research is a positive advance.
* Refine the clinical, genetic, neuropsychological, radiologic, and pathologic characterization of these disorders
* Improve our understanding of tau and TDP-43 protein physiology and pathophysiology so promising therapies can be developed and tested
* Identify and develop clinical, laboratory, neuropsychological, and radiologic measures for future therapeutic drug trials

We want to ultimately prevent, delay the onset, and slow the progression of the disorders that cause PSP, CBD, FTD, and PPA!

www.brain.northwestern.edu/ppa/index.html –> best website anywhere on PPA

Questions & Answers:  (all answers were by Dr. Boeve, unless indicated)
[Robin’s note:  I’ve grouped these by topic and re-ordered them]


Q by Janet Edmunson:  Can someone have a diagnosis of PSP, CBD, and FTD?

A:  It is possible to have all three features.  Very uncommon would
be to have features of all four disorders (including PPA).

Some clinicians give the diagnosis of the predominant disorder.

Q by Janet Edmunson:  How do you deal with sexual behavior?

A:  This sort of behavior sometimes leads to people being dismissed
from a care facility, and they have to be placed in a psychiatric facility.

Consider SSRIs, atypical neuroleptics, sometimes even
anti-convulsive/seizure medications.  In on case of a gentleman with
these behaviors, hormone medication (estrogen) is being tried.

A by Janet:  Seroquel worked for Charles.

Have to remind yourself that it’s the disease talking and not the person.

A by Dr. Boeve:  There was just an announcement about the
manufacturer of Seroquel having to settle a huge dispute with the government.

I use this medication frequently myself.

Yes, direct the anger at the illness, not the patient.


Q:  I have had PSP since 2004 and will be 80 in June.  Is this a form
of Parkinson’s?  Are all these diseases a form of PD?  What research
is being done and has there been any progress in finding a cause or
cure for PSP?

A:  Because the parts of the brain overlap with the parts affected in
PD, there can be overlap.  But PD and PSP are different, distinct
disorders.  PD is a Lewy body disorder.  PSP is a tau protein disorder.

If you are near a center that is participating in the Davunetide
trial, please participate!


Q:  Are you familiar with research on neuroplasticity?  Has any
research been done on neuroplasticity and FTD-type disorders?

A:  This refers to the ability of the brain to make up for problems
in another part of the brain.  Example:  a stroke patient may re-gain
use of a paralyzed limb over time by virtue of one part of the brain
helping other parts.  We need more research in this area!

Dr. Bruce Miller and UCSF researchers have found some rare
individuals with a particular neurodegenerative disorder who
developed artist talent after developing the disorder.  This
demonstrates neuroplasticity.  [Robin’s note:  I think Dr. Boeve said
semantic dementia was the disorder but I’m not sure.  SD is another
subtype of FTD.]

Q:  My brother was diagnosed with FTD at the age of 55.  He hasn’t
been able to talk for 18 months.  I suspect he now only understands
simple sentences.  What do we know about the way these patients
think?  Do they keep, at least for some time, the ability to think
with words?  Do they “lose” words in their minds as they “lose” words
in their mouth?

A:  Usually the speech production part of the brain is affected more
than the speech understanding part of the brain.  People seem to
understand far more than you would anticipate on the basis of their
spontaneous speech.  In advanced stages, it’s harder to know.

It’s a learning test for clinicians and family members.

Q:  Are there any alternative medications or supplements that might
enhance cognitive function in FTD?

A:  We don’t have any evidence.  Some believe in fish oil, CoQ10,
various vitamin preparations, blueberries, green tea, curry,
etc.  These dietary and supplementary agents…on a theroretical
level, we don’t know how they impact tau.  They might not hurt and
they might help.  Be cautious of expensive items.  There are some scams.

Q:  I have heard that Namenda may be harmful to the cognitive
function of those with FTD.  Is there any validity to this?

A:  All medications have side effects.  All side effects should be
reversible.  Not aware of any evidence of permanent side
effects.  It’s variable.

We are doing a Namenda study in FTD.

Q by Sharon Denny:  Is Aricept the same type of agent as Namenda?

A:  No.  They act on different neurochemical systems.  They can have
a role together because of this.

There is some early evidence supporting the use of Namenda in FTD,
but we just don’t know.  There needs to be a larger trial.

Q:  Any meds to control verbal outbursts often seen in patients with FTD?

A:  Most clinicians would consider the SSRIs.  Because of their
activity of serotonin and norepinepherine and because they are
well-tolerated, the SSRIs are tried.  Next, some clinicians will
consider atypical neuroleptics.

Physically aggressive behavior, hallucinations, and delusions can be
treated with atypical neuroleptics.

Q:  As the fulltime caregiver for my brother (age 53, Pick’s
Disease), one of the issues we are currently dealing with is his
disruptive mealtime behavior.  What are your suggestions on how to
deal with this?

A:  Hard to tell without more details.  Probably manners are not
being practiced.  There may be hoarding.  There may be inappropriate
statements said at the dinner table.

Honestly, this is very difficult.  This is difficult both in the home
and in restaurants.  Medicines tend not to work well.  This
circumstance probably requires tolerance by the family, as
frustrating as this may sound.

If there’s a chance of choking (due to hoarding), it’s important to
teach all caregivers the Heimlich maneuver.  Please learn it if you
don’t know it!

Q:  Are there techniques for diverting or minimizing behaviors
associated with FTD?

A:  This takes a real talent.  If there’s any way to work with social
workers, psychologists, and neuropsychologists, that is helpful
because these people are trained in re-directing.

For many, this disruptive behavior expresses something.  Try to
figure out what the person is expressing.  “I understand you are
upset.  Can you describe this more?”  Acknowledge it, and then re-direct.

Re-direct by saying “let’s go watch TV,” for example.

Q:  My wife was diagnosed with FTD and also has COPD.  She does have
difficulty breathing, but is fixated on the phrase “I can’t
breathe.”  She repeats this over and over again no matter what the
level of exertion and the actual being out of breath.  How can I
divert this behavior?  This seems to be more pronounced when I am present.

A:  Phrases can be repeated over and over.  There can be
humming.  There is often a restricted repertoire of verbal output.

This is hard to curb.  Any verbal expression or aggressive behavior
is a form of communication.  It may be that this person is trying to
indicate that she’s distressed or in pain.  The medicines don’t tend
to be helpful.  A lot of it comes down to what the person is trying
to communicate.  Can the individual write?

Q:  Is there a national list of neurologists who specialize or have a
particular interest in FTD?

A:  On the AFTD’s website, there’s a regional list of academic centers.

About CurePSP
Janet Edmunson
Chair, Board of Directors, CurePSP (psp.org)

Vision:  cure and prevent PSP, CBD, and related brain diseases

Genetics consortium:  announcing that they’ve discovered 6 new genes
that are related to PSP and CBD.

Questions for CurePSP can be emailed to [email protected] or
[email protected]

There are three upcoming webinars:  (registration required)

5/13/10 with Laura Purcell Verdun, speech therapist, on “Speech &
Swallowing Therapy:  Q&A Session”
Email Laura with your questions at [email protected]

6/24/10 with Chris Debrelle (sp?) on “Tips for Caregivers”

7/15/10 with Dr. Irene Litvan on “Symptoms, Treatments, and Resources
Available for CBD”

About AFTD (Association for Frontotemporal Dementias)
Sharon Denny
Program Director, AFTD (ftd-picks.org)

FTD = cluster of progressive disorders caused by degeneration of the
frontal and temporal lobes of the brain (bvFTD, PPA, SD, PSP, CBD)

Vision:  a world where FTD is understood, effectively diagnosed, and
ultimately, prevented

5/21:  Caregiver Education Conference and AFTD’s Annual Meeting in
Fort Worth, TX

If you have questions about FTD or PPA, contact the AFTD.  Contact info:
[email protected]

Another haplotype discovered for PSP and CBD

These Italian researchers have been at the forefront of biomarker and genetic research in PSP. In this study, they find a certain haplotype to be over-represented in PSP, CBS, and FTD,as compared to healthy controls.

I’m sure this Italian research team is participating in the PSP/CBD genetics project overseen by UPenn. It will be interesting to see if the four new genetic mutations found by the UPenn-led project include this A-G-G haplotype.


Journal of Alzheimers Disease. 2010 Apr 22. [Epub ahead of print]

VEGF Haplotypes are Associated with Increased Risk to Progressive Supranuclear Palsy and Corticobasal Syndrome.

Borroni B, Del Bo R, Goldwurm S, Archetti S, Bonvicini C, Agosti C, Bigni B, Papetti A, Ghezzi S, Sacilotto G, Pezzoli G, Gennarelli M, Bresolin N, Comi GP, Padovani A.
Centre for Aging Brain and Neurodegenerative Disorders, Department of Neurology, University of Brescia, Brescia, Italy.

Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are in the spectrum of tauopathies and recognized to have a strong genetic background. It has been widely reported that MAPT tau haplotype H1 is a genetic risk factor in both conditions, but no other genetic determinants have so far been proposed.

Recently, vascular endothelial growth factor (VEGF) haplotypes were reported to confer risk to frontotemporal dementia (FTD). The aim of this study was to evaluate the role of VEGF genetic determinants in PSP and CBS susceptibility.

We evaluated a cohort of 687 unrelated Italian subjects, including 117 PSP, 108 CBS, 199 FTD, and 263 healthy controls. Genotype and allele frequencies of three well-known polymorphisms located within the VEGF promoter (-2578C/A, -1190G/A, and -1154G/A) were carried out. Genetic analysis revealed the presence of significant changes in terms of genotype and allele distributions in patients compared to healthy controls.

A-G-G haplotype (-2578C/A, 1190G/A, -1154G/A) was overrepresented in both PSP (OR=6.64, 95% CI=2.3-19.6, P=0.0003, CGG=reference) and CBS (OR=5.20, 95% CI=1.70-15.9, P=0.003, CGG=reference) compared to healthy subjects. No differences between PSP and CBS and FTD were found, and the A-G-G haplotype was also overrepresented in FTD.

Overall, these data suggest that VEGF gene variability represents a susceptibility factor for PSP and CBS. These data argue that additional genes may confer disease risk to PSP and CBS, and to FTD as well, beyond the MAPT tau haplotype. Further studies are warranted.

PubMed ID#: 20413880 (see pubmed.gov for abstract only)

Physical Therapy Q&A (Webinar Notes, 4-8-10)

Last Thursday’s CurePSP webinar with Heather Cianci, a physical therapist who is an expert in movement disorders, was very good.  We need more people like this in our communities!

Though the webinar was hosted by CurePSP, all of the disorders in our group will find useful information in the webinar.

Here are some key points from my perspective:

  • Start an exercise program immediately.
  • For fall prevention, consider gait and balance training, home modifications, acceptance that certain things cannot be done without assistance, and adaptations.
  • How should the caregiver help someone walk? Often caregiver will stand to the side and put an arm around the back.  Sometimes holding at the elbow is good enough.  Sometimes holding hands is good enough.  This should be practiced together, in front of a PT.  If someone is less stable but isn’t ready for a walker, consider using a gait belt.
  • As soon as falls or balance problems begin, have PT and OT assessments for “prehabilitation,” education and training.  Learn new ways of transferring, walking (maybe with equipment), balance exercises, turning, bathroom safety, etc.  If someone can’t remember these techniques, someone must be with the patient at all times.  Later in the disease, the patient can’t move safely without falling.  Even when someone is home-bound, all effort should be made to keep someone active.
  • Generally, a 4-wheeled rolling walker that has seat and brakes works the best for PSP.  Durable wheels that make turns (swivel).
  • Examples of devices for getting out of a chair:  chair risers (that attach to the bottom legs of a chair); firm cushion to raise the height of a seat; power chairs.
  • Examples of devices for getting out of bed:  bed rails; wedge pillows; electric beds.
  • Two good websites for equipment are 1800wheelchair.com and sammonspreston.com.

The webinar was entirely questions and answers.  My notes are below.



Heather Cianci, PT
Physical Therapy:  Questions & Answers
CurePSP Webinar

Q:  Falls play a very significant role in all of these disorders.  I am interested in treatment strategies to assist with managements.

A:  As soon as falls or balance problems begin, she recommends PT and OT assessments for “prehabilitation,” education and training.  Learn new ways of transferring, walking (maybe with equipment), balance exercises, turning, bathroom safety, etc.

If someone can’t remember these techniques, someone must be with the patient at all times.  Later in the disease, the patient can’t move safely without falling.

Even when someone is home-bound, all effort should be made to keep someone active.

Q:  Even with limited exercise or mobility, I get short of breath to the point of not being able to speak in more than a whisper.  How can I exercise and get any benefits if I can’t breathe?

A:  You may need to see a pulmonologist.  If you are cleared by this MD, you may need a supervised exercise program monitored by a respiratory therapist.

972-243-2272 Respiratory Care, www.aarc.org

Q:  I am experiencing extreme muscular soreness in my left quad.  Why?  I am in pain.  My internist provided no answers.

A:  Hard to answer since I can’t conduct an assessment.

The quad plays a role in straightening the knee.  See a PT for an assessment.  If this is a pulled muscle (and that sounds like what it may be), heat and massage may help.

Q:  I have pain above my eyes.  Is this due to muscle problems?  My left eye does not open all the way.

A:  Without being able to do a formal assessment on you, I’m not able to give an exact answer.

See an ophthalmologist about the pain and the eye not opening.  This may be a pulled muscle in the head!

The eye not opening may be due eye lid muscle weakness or blepharospasm.  Treatments for blepharospasm:  botox injections, eyelid crutches, or Lundie loops on the glasses.

Ptsosis can be a problem.

Q:  When my husband sleeps over 12 hours, his balance is much better.  Is there any relationship?

A:  It makes sense that a well-rested person can handle balance challenges.  There is no research supporting this.

There are lots of sleep problems in PSP.

Q:  Could you address the differences in the visual disturbances between PSP, LBD, CBD, and/or FTD?

A:  In LBD, a big problem is hallucinations.  This may come into play when a patient tries to get up and talk to someone they are hallucinating about.  Education of care partners that this is normal.  Don’t try to talk the patient out of the hallucination.  Is there a pattern to the hallucinations?

In FTD, there are no visual deficits.  There are behavioral problems.  There is disinhibition; the usual filter is not there.  Many times these patients experience apathy.

In CBD, many patients suffer from visuo-spatial disturbances.  In a study, CBD patients may not have seen the depth of something (so they missed a step) or they may think a dark spot on a rug is a hole in the floor.

In PSP, there is a marked problem with vision.  Many play into the functional role of people moving around.  First is the difficulty with vertical eye movements.  Some can’t see down.  Some can’t see up.  Obviously if someone can’t look down, they can’t see the floor.

Second, often times the eyes are fixed at a given target.  They experience square-wave jerks.  Third, there can be a misalignment of the eyes.  Fourth, there is a problem with cogwheel tracking of moving objects.  And, they lose the quick phase of movement.  They experience nystagmus.  They have blepharospasm (involuntary closing of the eyes and inability to close the eyes).  Many have a staring look or a look of surprise.  Many have photophobia or intolerance of bright light.  Some with PSP can’t stop blinking in bright light.

Q:  What exercise or therapy has an effect on balance or eye movement in PSP patients?

A:  Cris Zampieri gave the last webinar.  There are exercises to help with this.  Eye movement exercises and balance exercises did better with their mobility than those who did balance exercises alone.  (Zampieri has published two studies.)

There was also a case report on one mixed PSP/CBD patient.  Treadmill study.  Improvement demonstrated.

Another case report on body-weight supported treadmill training.  Improvement demonstrated.

Unanswered questions:  when do we start these exercises?  How do we change the exercises?

Q:  Is incontinence a symptom of PSP?

A:  Yes.  Seen more in the later stages.
Q:  Is it worth focusing on balance and eye gaze when the patient can no longer stand or straighten his head to see?

A:  Always important to continue an exercise program, regardless of the stage of disease.  Exercises can be done in bed, seated, standing while holding on to a chair, etc.  Exercise makes us feel better.

Q:  Is there any type of eyeglass lens that will help to focus things that are below the fixed eyeball?

A:  Consider prisms.  Different prisms are needed for different tasks (eg, reading vs. watching TV).  Bring things up to the level of the eyes (gaze level).

Q:  Is there anything that can be done to prevent nystagmus?

A:  As far as I know, there isn’t anything that can be done.

Q:  My mother is dragging her leg.  Any recommendations on strengthening?

A:  Without doing an actual assessment, it’s not possible for me to say what the exact cause of the leg dragging is, or the best approach.  She may need an assistance device.  She may need to be taught strategies to take a larger step and land on her heal.

Q:  Can anything be done to slow the progression of eye changes?

A:  Nothing can be done to slow down eye changes.  You might consider patching one eye.

Q:  I’ve had PSP for the last 6 years, and have fallen over 1200 times.  Physical therapy has been discontinued.

A:  If you have a medical reason for physical therapy, it should be covered.  If you are not reaching your goals, insurance may not pay.

Look for a fitness class or a fitness trainer.  Go to a local gym.

Maybe you need home modifications, more assistance in the home, and a scooter or wheelchair.  This is dangerous!  The falls must be prevented!

Q:  What is effective for treatment in early/mid stage?  Should we focus on vision training, balance, neck mobility?  What can we do to prevent falls — compensation vs. rehab?

A:  Start an exercise program immediately.

Fall prevention:  gait and balance training, home modifications, acceptance that certain things cannot be done without assistance, and adaptations.

Q:  What is the best walker for PSP patients?

A:  Generally, a 4-wheeled rolling walker that has seat and brakes works the best.  Durable wheels that make turns (swivel).

We like to use 1800wheelchair.com.  800-320-7140 phone.  Get a catalog.

PSP patients should NOT use an aluminum, straight, 2-small-wheeled walker.  You can ask for a replacement for swivel wheels for this walker.

Q:  Is it a good idea to put weights on the front of the walker?

A:  This is done to prevent backward falls.  That’s the theory.  If the person doesn’t know how to properly use the device, this walker won’t work.

It’s best to have a PT assess your walking and suggest the best device.

Q:  What is the best device for getting up from a sitting or lying position?

A:  There are many good devices.  It’s best to have a PT or OT try the devices with you to find the right one.

Examples of devices for getting out of a chair:  chair risers (that attach to the bottom legs of a chair); firm cushion to raise the height of a seat; power chairs

Examples of devices for getting out of bed:  bed rails; wedge pillows; electric beds

Q:  While walking, the feet become frozen.  How do you help the PSP patient “unfreeze”?

A:  These techniques come from the PD world.  Don’t fight the freeze.

1- stop moving and steady yourself
2- take a breath and stand tall
3- make sure the weight is on both feet equally.  (Often with a freeze, the weight is imbalanced.)

Focus on walking; do that activity well.

Don’t pull on or push someone who is frozen. Talk them through it.  Have them shift their weight.  Or count 1-2-3 and take a big step.  Or step over something on the ground.

Lots of auditory and visual cues can help.  Her Center has a handout on this.

Q:  Is PT beneficial for all stages?

A:  Yes!

Q:  Are reflexology and massage beneficial?

A:  Many patients do get temporary relief from pain and stiffness from massage.  There’s no research to support or refute reflexology or massage.

Q:  Why does PSP cause one to run into walls and doorways, even when being guided?

A:  Lots of different things going on with PSP.  Loss of balance.  Visual-perceptual problem.  Loss of ability to scan the environment (anticipatory scanning).  May have double vision.  May be from mental confusion.

Q:  During the Zampieri webinar, did she say that they are running tests with rats who have PSP?

A:  No, these studies were done on rats with chemically-induced Parkinson’s Disease.

Q:  Did these studies slow the progression of PD?  Does this apply to PD?

A:  Different exercises gave different results in the rats’ brains.  In some, the rats were protected from developing PD.  Reduction in symptoms and cell death before the rats were given the PD.  After the rats were given PD, fewer dopamine cells died.

PD – loss of cells in SN that produce dopamine
PSP – weaking of muscles that are controlled by nerve cells that are controlled in the brainstem; this results from tau accumulation in the SN

The medications that treat PD don’t help with PSP.  We don’t know if the effects on the brain of those with PD will be the same on those with PSP.

But it doesn’t hurt to exercise.  Any exercise can be helpful, when done correctly.

Q:  My wife cannot walk by herself.  She cannot push a wheelchair by herself.  What PT can I do?

A:  You already have a great exercise program established.  Without evaluating her, I can’t give specific exercises.

Practice techniques to make the transfers easier to both of you.

Keep working on both cardio and strength training.

Q:  Recent news on PD bikers.  Does this apply to PSP?

A:  We don’t know.

Q:  What is prehabilitation?

A:  When first diagnosed, ask the MD for a referral to PT and OT.  Don’t wait for balance problems.

Q:  Should and can an MSA patient with OH still benefit from PT and what should they be doing?

A:  Absolutely.  OH is a sudden drop in BP when people go from a lying position to a seated position or a seated position to standing.  PT can really help with this.  PT can teach you exercises to pump the blood better.  PT can talk about compression stockings.  PT can help you learn safe ways of moving.

Q:  What about neck mobility?

A:  It depends on the situation.  In PSP, patients can have dystonia so it can drop the neck forward.  Consider botox.

In some, it’s the opposite with patients looking up at the ceiling all the time.  Botox might help with the retrocollis.

Stretch what is tight and strengthen what is weak.

Q:  What specific knowledge about PSP, if any, do you think a PT needs to have to do a stellar job at providing PT to a PSP patient?

A:  Great question!  In school, we might’ve received two minutes of training on atypical parkinsonism disorders.  This is not necessarily a bad thing.  Find a PT who is willing to do some research.  A PT is going to test you — walking, your balance, up and down stairs.  PTs can’t treat the deficit in the brain but they can treat the functional issues.  Are you falling backwards?

We are starting to have people who are specially-trained in PD and atypicals.  See wemove.org for PTs and OTs.

Q:  Problem of restless legs when sleeping.

A:  There is no good PT for this problem.

Q:  Are there any illustrated books available that show the exercises and assistance techniques specific to PSP for the caregiver?

A:  Fabulous question!  We are currently working on getting The Guide re-done.  It will have pictures.  We are also intending to make videos that are downloadable.

There are lots of exercises on the web.  Check with your MD, PT, or OT to be sure they’re safe.

Q:  Is there a catalogue for equipment?

A:  Two good ones:  1800wheelchair.com, sammonspreston.com

Any PT place should have catalogues of equipment.

Q:  How do we locate PTs throughout the US or other countries?

A:  Usually a movement disorder specialist is linked up with specialized therapists.  If you are not with an MDS, check out wemove.org, lsvtglobal.com (LSVT PT/OT or LSVT ST…they are trained in PD and will know some about the atypicals).

In the tri-state area (PA, MD, NJ plus DE), she has recommendations.

European Association Parkinson’s Disease Physio Therapists

Q:  Are ankle or foot weights beneficial or a hindrance for PSP patients?

A:  For balance, this is not helpful.  To strengthen muscles, this is helpful.  Not a great idea to put them on and walk with them.

Q:  How should the caregiver help someone walk?

A:  Often caregiver will stand to the side and put an arm around the back.  Sometimes holding at the elbow is good enough.  Sometimes holding hands is good enough.  This should be practiced together, in front of a PT.

If someone is less stable but isn’t ready for a walker, consider using a gait belt.

Q:  What are some ways to make the home more safe?

A:  Big topic!  You can actually go room through room.  An OT or CAPS certified aging in-place specialist) can evaluate the home.

Q:  Diagnosis of PSP.  The dementia seems to be causing the patient to exclaim they have only a balance problem and not PSP.  How does one overcome the symptoms to convince a patient they have the disease?

A:  Schedule another appointment with the diagnosing neurologist or one of the team members.  Ask for another appointment with the patient and care partner for the purposes of medication.  Seek help also from a social worker, counselor, or cognitive behavioral therapist for the patient.

This is a clinical diagnosis.  Best to find a movement disorder specialist.

Q:  Are there any new methods for treating MSA-C?

A:  Nothing new in the PT realm for MSA-C.

There is a study ongoing at UPenn for Azilect in MSA-P.  See pdtrials.org to learn if the study is happening near you.

There is a 2008 study comparing the cognition of those with MSA-P and MSA-C.  Those with MSA-C have less severe cognitive dysfunction than MSA-P.

Q:  Can you give information about support groups or volunteers that could go in and help people all over the world?

A:  CurePSP, psp.org
Facebook page “Miracles for MSA”
MSA National Support Group, shy-drager.org

Find volunteers through:
* local church or synagogue
* hospitals
* universities with PT, OT, and ST programs
* local clubs (eg, Rotary, Kiwanis)
Medical Education Advisory Board of CurePSP is putting together a series of pamphlets on PSP, CBD, and MSA (three separate booklets).  Information for PTs, OTs, and STs.  These will be available online soon (May).

Hypothesis that King Lear had Dementia with Lewy Bodies

An article titled “Portrayal of Neurological Illness and Physicians in the Works of Shakespeare” was recently published.  I’ve copied the abstract below.  Part of the article addresses the hypothesis that King Lear had Dementia with Lewy Bodies.

There’s a chart in the article that indicates what characters in what plays had what neurological diagnosis, and who made that (modern) diagnosis.  Two examples are:  Achilles, a character is “Troilus and Cressida” had signs of parkinsonism and Lord Say, a character in “King Henry VI, part 2” had signs of tremor.

The abstract is below.



Frontiers of Neurology and Neuroscience. 2010;27:216-226. Epub 2010 Apr 6.

Portrayal of Neurological Illness and Physicians in the Works of Shakespeare.

Matthews BR.
Indiana University School of Medicine, Indiana Alzheimer Disease Center, Indianapolis, Ind., USA.

William Shakespeare was arguably one of the most prolific writers of all time. The topics explored in his works include both physicians and neurological illnesses. In addition to a review of the portrayal of neurological diseases such as dementia, epilepsy, parkinsonism, and parasomnias, this article describes the roles of physicians in Shakespeare’s plays. Furthermore, a novel hypothesis that King Lear, one of Shakespeare’s more tragic figures, suffered from dementia with Lewy bodies is explored based on evidence from the dialogue of the drama.

Copyright © 2010 S. Karger AG, Basel.

PubMed ID#: 20375533   (see pubmed.gov for the abstract)