“Treating PSP, MSA, and CBD – What can be done?” – Webinar Notes, Recording, and Resources

Brain Support Network and Stanford University co-hosted a webinar last Wednesday, February 27th about “Treating PSP, MSA, and CBD – What can be done?”  The webinar focused on what treatments are possible for progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration.  Most of the presentation and question-and-answer session apply to Lewy body dementia and advanced Parkinson’s Disease.

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RECORDING

The webinar recording can be found here —

https://www.youtube.com/watch?v=ePKXO1UPOlw

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SURVEY

If you listen to the webinar recording, please take LESS THAN TWO MINUTES to answer six questions on our survey.  See:

https://www.surveymonkey.com/r/2SX3KH3

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FUTURE WEBINARS

To be alerted to future webinars on PSP, MSA, or CBD, join one of Brain Support Network’s email lists –
www.brainsupportnetwork.org/join

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RESOURCES

For additional information on the topics addressed during the webinar, see:

PSP, MSA, and CBD (including a list of Top Resources from around the world) – 
www.brainsupportnetwork.org/psp
www.brainsupportnetwork.org/msa
www.brainsupportnetwork.org/cbd

Brain donation to support PSP, MSA, and CBD research –
www.brainsupportnetwork.org/brain-donation

Finding knowledgeable healthcare professionals (movement disorder specialists, physical therapists, occupational therapists, speech therapists, social workers, etc)  –
apdaparkinson.org/community

IF YOU ARE IN NORTHERN OR CENTRAL CALIFORNIA – Finding knowledgeable healthcare professionals (movement disorder specialists, physical therapists, occupational therapists, speech therapists, social workers, etc)  –
parkinsons.stanford.edu

Centers of Care for PSP and CBD –
www.psp.org/ineedsupport/centers-of-care

LSVT BIG and LSVT LOUD –
www.lsvtglobal.com/LSVTFindClinicians

Make an appointment with Dr. Bluett or other movement disorder specialists at Stanford –
Call 650-723-6469

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OVERVIEW

Our terrific volunteer, Denise Dagan, shared a summary of what Dr. Bluett discussed:

Dr. Bluett’s mother had colon cancer while he was a resident in medical school.  It gave him a lifelong lesson of the patient’s perspective.

Dr. Bluett discussed what can be done to manage symptoms of ‘parkinsonism,’ a term used to refer to disorders with symptoms which resemble Parkinson’s disease.

It can be confusing to understand what doctors are looking for when making a clinical diagnosis (used when there is no lab test or imaging to confirm a diagnosis), so I love that Dr. Bluett included a differential diagnosis chart for:

* corticobasal degeneration (CBD)
* Lewy body dementia (LBD)
* multiple system atrophy (MSA)
* Parkinson’s disease (PD) and

* progressive supranuclear palsy (PSP)

Dr. Bluett then outlined both motor and non-motor symptoms that can be experienced by someone with any of these five parkinsonism disorders (including PD and LBD) – and what to do to alleviate those symptoms.

Dr. Bluett shared these key points:

* There is unfortunately not currently a cure for atypical parkinsonism

* But there are several symptomatic treatments which can improve one’s quality of life

* Each individually may be minor, but in sum they can make a significant difference

* Seek out a movement disorders specialist, preferably one with expertise in atypical parkinsonism

* Be on the lookout for clinical trials
* There is always hope!!!
See more details below from Denise.

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DETAILED NOTES – PRESENTATION

Our terrific volunteer, Denise Dagan, took notes from the webinar.

Webinar
Treating PSP, MSA, and CBD – What can be done?

Speaker:  Brent Bluett, DO, movement disorders specialist, Stanford University
Host: Sharon Reichardt Walker, former PSP caregiver, Brain Support Network

February 27, 2019

This webinar will address these topics:
• What symptoms of PSP, MSA, and CBD are amenable to improvement?
• How can physical therapy, occupational therapy, exercise, and assisted devices help?
• What about eye movement training? 
• What about botox?
• What are some eating and drinking strategies?
• How can you ask your neurologist about these treatments?

PSP = progressive supranuclear palsy
MSA = multiple system atrophy
CBD = corticobasal degeneration

DR. BLUETT’S PRESENTATION

Overview:

* Parkinsonism is a term used to refer to disorders which resemble Parkinson’s disease (PD)

* “Atypical parkinsonism” includes four disorders: Progressive Supranuclear Palsy (PSP), Multiple System Atrophy (MSA), Corticobasal Degeneration (CBD), and Lewy Body Dementia (LBD)

* PD, MSA, and LBD are due to abnormal accumulation of the protein “alpha-synuclein”

* PSP and CBD are due to abnormal accumulation of the protein “tau.”  Tau also accumulates in the brains of those with Alzheimer’s.  Research into Alzheimer’s trickles down to assist research into PSP and CBD.

* There is currently no cure for parkinsonism, but the incidence of these disorders is increasing

* Today we will discuss what we can do to help manage the symptoms of PSP, MSA, and CBD, until we have a cure…


Who Should Be Managing My Symptoms?
* A movement disorders specialist!
* They are trained in the diagnosis and management of atypical parkinsonism.  In choosing a movement disorders specialist, look for atypical parkinsonism in their bio, training and experience.
* It typically takes three years before those with atypical parkinsonism see a movement disorders specialist
* Early intervention is very important for your overall health
* May be aware of clinical trials for your disorder  — https://clinicaltrials.gov/  Enter condition and it will show a list of clinical trials.  Look for Status: “Recruiting or Enrolling by invitation”


How Do We Diagnose Atypical Parkinsonism?
Clinical diagnosis = examination and observation only – no tests or imaging currently available
Dr. Bluett presented a slide showing a diagram of the differential diagnosis of atypical parkinsonism flowchart.  [Editor’s Note:  Probably best to view the webinar recording to see this slide!  It is protected by copyright so we aren’t allowed to reproduce it.]

The diagram begins with Bradykinesia and branches as to whether the patient exhibits:
A. Cognitive impairment before or shortly after onset of more symptoms
B. No or minimal cognitive impairment at onset of motor symptoms

A. further branches to:
a. No slowing of vertical saccades or downward gaze palsy
b. Slowing of vertical saccades or downward gaze palsy

B. further branches to:
c. Autonomic dysfunction (esp. orthostatic hypotension & urinary incontinence) + early postural instability + poor response to levodopa +/- cerebellar signs (difficulty coordinating fine motor movements)
d. 4-6Hz resting tremor + rigidity (limb > axial) + late postural instability + good response to levodopa

If the physician’s observation is b. there would also be noticed:
Postural instability with falls within the first 3 years of symptom onset + rigidity (axial > limb) + poor response to levodopa, resulting in a clinical differential diagnosis of Progressive Supranuclear Palsy.

If the physician’s observation is c. the clinical differential diagnosis is Multiple System Atrophy

If the physician’s observation is d. the clinical differential diagnosis is Parkinson’s Disease.  Parkinson’s disease is responsive to levodopa.

If, however, the physician’s observation is a. there is the further branching to distinguish between CBD and DLB (Dementia with Lewy Bodies), as follows:
e. Asymmetric motor symptoms or
f. Symmetric motor symptoms

If the physician’s observation is e. there would also be noticed:
Limb rigidity, akinesia, dystonia, or myoclonus (jerky tremor) + orobuccal (mouth & tongue) or limb apraxia (inability to use intended muscle) + cortical sentry deficit + alien limb phenomenon (your limb is not your own) + poor response to levodopa, resulting in the clinical differential diagnosis of Corticobasal Degeneration.

If the physician’s observation is f. there would also be noticed:
Visual hallucinations + fluctuating levels of cognition + poor response to levodopa, resulting in the clinical differential diagnosis of Dementia with Lewy Bodies.


Motor Symptoms – Does Carbidopa-Levodopa Work?
* Carbidopa-levodopa 25-100 mg
* A sufficient trial is two pills three times a day (600 mg daily).  Gradual titration is recommended to avoid side effects
* 25-33% of atypical parkinsonism patients will have a significant, sustained response to levodopa therapy.  PSP-Parkinsonism (subtype of PSP) tends to have a more robust response, with greater improvement of symptoms.  Often a resting tremor when one has PSP-Parkinsonism

* There is no evidence for the use of dopamine agonists (pramipexole, ropinirole, rotigotine patch) in PSP, CBD, or MSA.  May cause side effects, esp. impulse control disorder, excessive daytime sleepiness, and worsen lightheadedness when standing


Postural Instability/Falls Prevention – Important in all 3 disorders (PSP, CBD, MSA)
* Physical therapy
– Do not underestimate the importance of physical therapy!  It can be more helpful and medications.
– Training focused on increasing amplitude of movements (i.e. LSVT-BIG or POWER)
– Focus on postural stability/gait training (walking)
– Balance, eye movement, and visual awareness training may improve gait in PSP

* Exercise – if you don’t use is, you lose it!
– Core strengthening
– Aerobic exercise (safe and as tolerated)

* Occupational therapy
– Home health assessment may be beneficial to understand how to make your home safer (fall prevention)
– Evaluation for lifting devices or wheeled mobility aides
– Optimize upper limb function

* Assisted walking devices
– Cane, walker, wheelchair
– Weighted walker may help prevent falls backwards (esp. important with PSP)


Orthostatic Hypotension (lightheadedness upon standing), especially with MSA

* Conservative measures:
– Increase water and salt intake
– Stand up slowly (most important! esp. with those who have impulsivity control issues.
– Exercises (i.e. leg crossing, squatting) to reduce lower body blood pooling
– Avoid alcohol
– Avoid autonomic stressors (i.e. long exposure to hot water, use a shower chair)
– Thigh-high compression stockings (with or without abdominal binder)
– Sleep with head of the bed elevated by 15 – 23 cm (15 – 30 degrees)

* If taking medications that can lower blood pressure
– Discontinue the medication if the risks are greater than the benefits.  Speak with your physicians about this.

If orthostatic hypotension is refractory to conservative measures
* Pharmacotherapy:
– Treat mild disease with pyridostigmine
– Use pressor agents (raise blood pressure) for moderate – severe OH
— Midodrine
— Droxidopa
— Fludrocortisone as a last resort (Also called Florinef).  Can cause leg edema -> more difficulty walking and increased risk of falls
– Treat postprandial hypotension with Acarbose

Freezing of Gait

* An inability to walk despite the intention to do so
* A feeling that one’s feet are glued to the ground
* Typically happens when starting to walk, turning, going through narrow spaces, reaching one’s intended destination, and with dual tasking.  Tune everything else, don’t even talk while you walk.
– Important to limit distraction while walking
* One of the most disabling symptoms of parkinsonism, and a frequent cause of falls
* Pharmacotherapy:
– Rasagaline (< 1 mg/daily) very few side effects.
– Amantadine 100 – 200 mg bid, works a bit better than Rasagaline in Dr. Bluett’s experience.  Monitor for hallucinations, leg edema, or impaired cognition, maybe take at bedtime.

* Cues
– Rhythmic auditory cues
– Focus on line on ground to step over
– Assistive devices project laser light on the ground to simulate a line to step over
— Weighted walker with laser light
— Cane with laser light
— Shoes with laser light

Cervical of Limb Dystonia in all 3 parkinsonisms (MSA, PSP, CBD)

* Dystonia = intermittent, sustained, repetitive muscle contractions resulting in abnormal posture
– Cervical area = neck stiffness, rigidity, tremor, limited range of motion
– Limb = Arm or leg stiffness or rigidity with abnormal posturing
* Pharmacotherapy can help, but side effects are common
– Trihexiphenidyl
– Baclofen
– Clonazepam
– All can cause sedation, imbalance, and confusion
* Botulinum toxin injections are best way to treat dystonia.
– FDA approved for cervical and limb dystonia
– Safe and effective but start with a low dose and increase as tollerated
– Recommend a physician with experience injecting for your particular condition

Eyelid Dysfunction

* Blepharospasm (contraction of eyelids) or apraxia of eyelid opening (miscommunication between brain and eyelid muscles)
* Botulinum toxin injections – Dr. Bluett recommends.  FDA approved and indicated for blepharospasm and apraxia of eyelid opening
* Oral medications are less effective
* Eyelid crutches may be of benefit
* Prism glasses may be of benefit for double vision in PSP

Non-Motor Symptoms – Constipation (prevention is better than treating after symptoms occur)

* Stay hydrated!  If urinating at night is an issue, slow down on fluid intake in early afternoon
* High fiber diet
* Daily metamucil or prune juice
* Miralax (17 g once or twice daily as needed)
* Stool softeners (i.e. Docusate or Dulcolax)

Dream Enactment Behavior (RBD) and Insomnia

* RBD = REM Behavioral Disorder.  Talking in one’s sleep or acting out one’s dreams
* Insomnia:  Difficulty going to sleep or staying asleep
* Melatonin.  5 or 10 mg (higher doses may cause paradoxical worsening of sleep).  Over the counter is 1-3mgs and often don’t work, possibly because the dosage is less accurate than prescription.  Recommend getting this by prescription.  OTC versions may have variable amounts of melatonin
* Clonazepam but side effects are bad so last resort.  Monitor for excessive sedation, impaired cognition
* Rivastigmine.  Evidence that it can ameliorate RBD

Daytime Somnolence

* First, treat insomnia and/or sleep apnea.  Sleep apnea may require continuous positive airway pressure (CPAP) or bivalve positive airway pressure (BiPAP).  Less intrusive and invasive models are now available
* Sleep hygiene
– No television in the bedroom
– No ambient light
– Warm milk or turkey (tryptophan)
– Warm baths (if safe)
– Maintain a regular sleep schedule
* Modafinil (Provigil).  May help but can cause irritability and/or impulsivity

Drooling (Sialorrhea)

* Extremely common and can be embarrassing or dangerous
* Botulinum toxin injections (very easy to treat!).  FDA indicated for sialorrhea.  Simple, safe, and effective
* Glycopyrrolate.  Need to monitor for impaired balance or confusion

Difficulty Swallowing (Dysphagia) – most common cause of morbidity or mortality are falls or choking

* Speech therapy
– Bedside swallow evaluation
– Modified barium swallow study
* Monitoring swallow function
* Eating and drinking strategies
– Tuck chin while eating/drinking
– Modified diet and fluids.  ie, “Thick-It” available at any pharmacy to thicken any liquid.  Ask a speech therapist before using.
– Maintaining oral hygiene.  Botox shots can help open the mouth wider to access the mouth and improve oral hygiene
– Assistive mealtime devices
* Ultimately, a discussion regarding placement of a Percutaneous Endoscopic Gastrotomy (PEG) feeding tube may be necessary. This is reversible and may be a temporary measure

Treatment of Neuropsychiatric Symptoms

* Depression/anxiety
– Antidepressant.  Recommend Selective Serotonin Reuptake Inhibitor (SSRI) or Serotonin-Norepinephrine Reuptake Inhibitor (SNRI).  Avoid tricyclic antidepressants due to potential side effects.  (i.e. Amitriptyline, nortriptyline).  Can cause confusion, sedation, imbalance
* Inappropriate laughing/crying (Pseudobulbar affect) esp. with PSP.  Dextromethorphan/quinidine (Nuedexta) is very effective for pseudo bulbar affect.

CurePSP Centers of Care

* A network of centers in the United States and Canada designed to increase access and improve care for PSP and CBD:  https://www.psp.org/ineedsupport/centers-of-care/
* Four Goals:
– To help PSP and CBD patients find the best possible local/regional care
– To increase awareness of PSP and CBD spectrum disorders providing educational material to patients, caregivers, and healthcare professionals
– To provide local/regional resources for physicians seeking information about PSP and CBD
– To promote collaboration between centers of care involved in order to optimize standards of patient care and establish hubs for PSP/CBD (pre-)clinical research

Dr. Kathleen Poston at Stanford has put together a center of excellence for MSA.

Conclusions:
* There is unfortunately not currently a cure for atypical parkinsonism


* But there are several symptomatic treatments which can improve one’s quality of life

* Each individually may be minor, but in sum they can make a significant difference

* Seek out a movement disorders specialist, preferably one with expertise in atypical parkinsonism

* Be on the lookout for clinical trials

* There is always hope!!!

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DETAILED NOTES – QUESTION AND ANSWER

Over 200 questions were received in advance.  We narrowed it down to 50.

Some Advance Questions – Motor Symptoms

* Treatment for rigidity?

* Spasticity – treatment? pain relief?
* Are joint or muscle contractures the same thing as dystonia?  Are there any preventative steps?

Rigidity (stiffness) includes spasticity and dystonia (artane).  Medical treatments are Baclofen, for dystonia. Botox is most effective but there are medications.  You don’t want to let the condition get to the point of contractures, because then it cannot be treated with Botox.  There are orthotic devices (braces, etc.) that can aggravate rigidity.


* Can cerebellar ataxia (in MSA-C) be treated?

* CBD is one-sided.  Are there any robotic devices that can be used to strengthen the affected side?

Currently, no.  There is not weakness involved in these disorders, it is slowness and stiffness, best treated with Botox.

* What can be done about mutism? How can we communicate?

Try using a keyboard to communicate.


* Is there a treatment for the eye tracking problem?

There is specialized physical therapy to help overall coordination that can improve symptoms.


* How can we help someone clear his/her throat? Person is choking on phlegm and has a hard time coughing it up.

This is a risk for aspiration.  Suction devices are available through your Dr. or physical/speech therapist.

Some Advance Questions – Non-Motor Symptoms

* Pain management?  (lots of questions on this topic)

CBD and MSA have more pain issues.  Get a pain specialist involved.  Opiates may be required, but should be avoided due to side effects.  Botox is still his go-to if cause of pain is dystonia.


* What about urinary incontinence?  Botox for this?

It oftentimes does not work.  Overflow incontinence is usually the issue.  Undergarments and bedside commode is the best way to address this.


* Dealing with severe leg cramps at night

Tonic water has quinine in it that can relieve cramps.  Everything you take has side effects so discuss with your Dr.


* Any suggestions for dental care as my brother’s mouth won’t always open?

The problem is probably a mandibular dystonia.  Try Botox to relieve the jaw joint so you can provide dental care.


* How do we deal with stridor during the day?

Discuss with your doctor or pulmonologist.  It can be due to thin liquid aspiration so treat drooling. Suction devices can help as well.


* How can cognitive problems be treated?

There are many areas of cognition.  For Alzheimers they use Aricept, for parkinsonisms we use Exelon.  To stay alert, try keeping goals for the day and keep track of what you’re doing, stay focused, use mindfulness.

* Do patients with these disorders hallucinate?

Not usually, that is usually LBD.

Some Advance Questions – Specific Treatments

 

* Does cannabidiol (medical marijuana) help these disorders?  (lots of questions on this topic)
There are two parts of marijuana.  CBD is the medicinal part which is probably most useful for relaxation.

* Stem cell therapy?  (lots of questions on this topic)
Problem is getting stem cells into the right part of the brain.  There are some ongoing studies into how to do this.


* Immunotherapy?
* Hyperbaric oxygen therapy?

Be careful!  Always be sure what you are getting benefits you and not the therapy provider.  You can use those financial resources to useful treatments.  There is not benefit of Hyperbaric O2 therapy.

* Can vitamins slow the progression of disease?
Nope.


* What about the Duopa Pump?

This is not indicated for MSA, CBD, PSP because they are not affected by levodopa.

Some Advance Questions – Resources

* What is the process and value of brain donations for research into these disorders?

Sharon tells us that Brain Support Network helps families to accomplish brain donation to benefit research into neurodegenerative disorders.  About 50% of donating families found out through neuropathological analysis that their family member’s clinical diagnosis was incorrect.  See brainsupportnetwork.org/brain-donation

* Is autopsy the only way to confirm a diagnosis?  Can PSP, CBD, and MSA co-occur?
Yes, but with PSP the diagnostic accuracy is 80-90%, CBD and MSA also are about the same.  Problem is part of the diagnostic criteria is urinary incontinence and in older men urinary issues of aging can confuse symptoms.


* How to find:  movement disorder specialist or neurologist; PT, OT, or exercise instructor trained in LSVT BIG or POWER; speech therapist; social worker

LSVT Find a Clinician.  LSVT focuses on amplitude of vocal sounds and gross motor movements.  Also the APDA Information and Referral Center at Stanford.


* What is a DO?  How is that different from an MD?

DO is doctor of osteopathic medicine.  It is an MD with more of a holistic approach to medicine.  They are very similar, overall.

Some Advance Questions – Specific Problems

* Can bladder training work if there is dementia?

Yes, it is more difficult.  If there is dementia there is impulsivity.  The frontal lobe communicates with your spinal cord, communicates with your bladder.  In dementia the frontal lobe is impaired.  Use absorbent undergarments.


* Can breath support be improved? (using the expiratory muscle strength trainer, for example)

Definitely, also consult a pulmonary specialist.

* Can mindfulness or meditation help with anxiety or depression?  Even if there is dementia?
If there is dementia it will be difficult to get the person to cooperate.  Dr. Bluett’s motto is to start with things that are less invasive with the fewest side effects, so try it!

* Is it possible to recover from impaired mobility via PT?

Yes, but it depends on the level of severity.  If there is impaired mobility, augment PT with Botox.  Botox weakens muscles because it can cause weakness and falls.

* If opiates affect cognition and cause hallucinations, what kind of treatment can be offered for pain relief?

Pain in these disorders is usually due to dystonia so Botox is first treatment, then other agents besides opiates.

* What are some common and easy exercises that the patient can do without fear of falling?

Here’s a simple one.  Stand with your arms crossed.  Someone should spot you so you don’t fall.  Ask physical therapy.

* Which of these therapies are appropriate in the late stages?  (Or, which of these therapies are not appropriate for late stages?)

Every treatment plan should be custom designed to accommodate their individual abilities.  Speak with a physical therapist about this.

* Do all of these treatments apply to advanced Parkinson’s Disease? Lewy body dementia?
No.  Parkinson’s and LBD show more response to levodopa.

* Are there any new drugs for non-motor symptoms in the pipeline?

Yes, there are lots of things – for REM sleep behavior disorder.  There is not as much research into these, but there are some.

Some Advance Questions – Insurance

 

* Hospice and palliative care do not want to approve speech therapy or physical therapy.  Should I argue with them about this?
Once you go into hospice they don’t want other interventions.  It may be futile to argue with them.  Let your doctor argue with them about it.  We’re used to it.

* What can we do if the insurance company denies eye therapy?
We/your doctor can write a letter of appeal, PT can help as well.

* How to optimize therapy (OT/PT/ST) if we only get a certain number of sessions approved?  What are the most important goals?

Prioritize falls and choking.  Everything else is troublesome, but not life threatening.

Some Advance Questions – Other

* When should my family member stop driving?

With these diseases, early.  There is concern about slowness of movement/reaction time, cognitive issues. There are driving evaluations at occupation therapy centers.

* Can medications affect cognition?
All of them.

* What matters more–my precise diagnosis or how I treat my condition?
Treating your condition.  The precise diagnosis is academic.

* What can a caregiver do to help?
Caregivers should not put too much on yourself.  He sees people literally breaking themselves.  Talk to your care recipient’s doctor and a social worker to find resources to help.

* Is there hope?

Yes.  Huntington’s disease is an excellent example where there may be a cure involving gene-modifying therapy.  Research into all of these neurodegenerative disorders impacts all of them.

* What about vital-stem for swallowing?

It really does help!

* How is Pisa syndrome treated?

Try Botox into the extensor muscles of the back.  It is very difficult to treat.  Posture is hard to maintain, but caregivers should understand it is part of the disease.

* Are there assistive devises for speech?

Yes, going back to the keyboard.  Speech therapists have great tips up their sleeves and great recommendations for this.