“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.

 

Multiple System Atrophy and Cognition Webinar – Recording, Resources + Notes

Brain Support Network and Stanford University co-hosted a webinar last Monday, November 12th about Multiple System Atrophy (MSA) and cognition.

———————–

RECORDING

The webinar recording can be found here —

https://youtu.be/hr0TCWqBgUY

———————–

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/W23K9RW

———————–

FUTURE WEBINARS ON MSA

To be alerted to future webinars on MSA, join Brain Support Network’s MSA email list –
www.brainsupportnetwork.org/join

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RESOURCES

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

Multiple system atrophy (including our list of Top Resources from around the world) – www.brainsupportnetwork.org/msa

Brain donation to support MSA researchwww.brainsupportnetwork.org/brain-donation

Lewy body dementia – www.brainsupportnetwork.org/lbd

Parkinson’s Disease (PD) – parkinsons.stanford.edu

Orthostatic hypotension –
parkinsons.stanford.edu/orthostatic_hypotension.html

Cognition in PD – parkinsons.stanford.edu/cognition.html

Psychosis in PD – parkinsons.stanford.edu/psychosis.html

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

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SUMMARY

Our terrific volunteer, Denise Dagan, wrote a summary of the webinar:

Movement disorders specialist Kathleen Poston, MD discussed the diagnostic criteria for MSA with special attention on recent research showing memory and cognition problems can occur in MSA patients.

Here are some points Dr. Poston covered in this talk:

* Symptoms of MSA (now unofficially includes cognitive impairment).

* How to approach treatment of cognitive impairment based on how suddenly symptoms appear.

* How to identify if cognitive symptoms are associated with dopamine medications.

* Outline of six cognitive domains.

* In all variants of MSA executive cognition is affected, typically later in the disease process.

* The other five cognitive domains are affected to greater and lesser degrees, depending on the variant of MSA in question.

* Definition of psychosis.

* When sudden onset or worsening of hallucinations may occur, and how to treat them.

* When a diagnosis of MSA, Parkinson’s or Lewy body dementia (LBD) should be made — based on what symptoms present and the timing of those symptoms.

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NOTES

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

Webinar
Multiple System Atrophy and Cognition

Speaker:  Kathleen Poston, MD, movement disorders specialist, Stanford University
Host: Candy Welch, former MSA caregiver, Brain Support Network

November 12, 2018

This webinar will address these topics:
• What are normal cognitive problems in multiple system atrophy (MSA)?
• Can “cognitive impairment” be part of MSA?
• Can “dementia” be part of MSA?
• Are there MSA symptoms that can mimic dementia?
• When should a diagnosis of “Lewy body dementia” be given?  Does it make a difference?
• What’s the treatment?

DR. POSTON’S PRESENTATION

Diagnostic Criteria for Multiple System Atrophy (MSA)
From: the Second Consensus Statement on the Diagnosis of MSA (Gilman, et. al., Neurology 2008)
Every person has early, significant autonomic dysfunction (blood pressure fluctuations, urinary frequency or incontinence, constipation, sweating, sexual dysfunction, etc.) PLUS
* Parkinsonism (slowness, stiffness, muscle rigidity) = MSA-P
OR
* Ataxia (poor coordination) = MSA-C

Autonomic Systems (reiterated) =
• Blood pressure and heart rate fluctuations (orthostatic hypotension)
• Urinary difficulty and incontinence
• Obstructive Sleep Apnea and/or RBD
• Difficulty with sexual functioning

Parkinsonism =
• Bradykinesia (slow movement)
• Rigidity (stiffness in the muscles)
• Tremor (rhythmic shaking)
• Balance and walking problems

Cerebellar Symptoms =
• Ataxia (poor coordination)
• Can be either with limb movement or with the trunk and neck movements
• Speech problems
• Swallowing problems

Traditionally the diagnosis of ‘dementia’ has been considered a red flag that the diagnosis is not MSA.  However, it has recently been recognized that memory and cognition problems can happen in patients with MSA
2008 currently being considered for revision.

Motor versus Non-Motor Symptoms:
Classic Motor Symptoms =
• Ataxia
• Bradykinesia, Tremor, Rigidity

Mixed Motor/Non-Motor Symptoms =
• Hypophonia (soft speech) and Dysarthria (difficult or unclear articulation of speech)
• Gait and Balance Problems

Non-Motor Symptoms =
• Mild Cognitive Impairment and Dementia (rarely dementia)
• Psychosis (hallucinations and delusions)
• Sleep disorders (RBD and OSA)
• Depression/Apathy, Anxiety, Fatigue
• Constipation, Orthostatic hypotension, Urinary frequency, urgency, incontinence

Types of Cognitive Impairment:
1. Sudden
2. Intermittent (off and on)
3. Slowly progressive

1. Sudden Change in Cognition
* Most common reason for a sudden onset or change in symptoms, including cognition, is general medical illness or other medications
– Infection (such as a UTI or a cold/flu – elderly & those with neurodegenerative disorder may not run a fever or feel pain urinating)
– Medications (for pain, urinary frequency)
– Physical stress (constipation, poor sleep, travel, new physical environment)
– Emotional stress (anxiety)
– Being in the hospital (all of the above)

How to treat a Sudden Change
* Deal with the underlying cause (prior slide)
* Reduce or stop certain medications:
– Artane (any other anticholinergics)
– Amantadine
– Dopamine agonists
– Sinemet CR (carbidopa/levodopa CR)
– Comtan (entacapone)

2. Intermittent Cognitive Impairment
* Sometimes called fluctuations in cognition
* Can be associated with level of alertness throughout the day
– Determine whether cognitive impairment is related to dopamine medication by keeping a daily diary of medication dosages and times, symptom timing and duration, and meals.

Scenario 1: Non-motor symptom improves with dopamine
7:00a – Take 1, 25/100 Sinemet (carbidopa/levodopa)
9:00a – Symptom A starts
10:00a – Take 1, 25/100 Sinemet : Symptom A continues
10:30a – Symptom A ends
12:00p – Symptom A starts again
1:00p – Take 1, 25/100 Sinemet ; Symptom A continues
1:30p – Symptom A ends

Scenario 2: Non-motor symptom worsens with dopamine
7:00a – Take 1, 25/100 Sinemet
7:30a – Symptom A starts
8:00a – Symptom A ends
10:00a – Take 1, 25/100 Sinemet
10:30a – Symptom A starts
11:00a – Symptom A ends
1:00p – Take 1, 25/100 Sinemet
1:30p – Symptom A starts
2:00p – Symptom A ends

Scenario 3: Non-motor symptom is independent of dopamine, but related to meals.  This happens especially in orthostatic hypotension (fluctuating blood pressure).  Cognition can be reduced by low blood pressure.  A home blood pressure monitor can help determine if this is the situation.
7:00a – Take 1, 25/100 Sinemet
7:30a – Symptom A starts (Breakfast)
8:30a – Symptom A ends
10:00a – Take 1, 25/100 Sinemet
12:00p – Symptom A starts (Lunch)
1:00p – Take 1, 25/100 Sinemet
2:00p – Symptom A ends

3. Slowly progressive change in Cognition
* More likely to be part of the disease process

Cognitive Domains:
Each controlled by different parts of the brain.  Different parts of the brain are affected by pathology of different neurodegenerative disorders.
* Memory
* Processing speed
* Attention and working memory
* Executive function
* Visuospatial function
* Language

Memory:
* Example: On your way out the door your spouse asks you:  “Can you pick up some milk while you are at the store?”
* The process of memory includes:
– Encoding = storing information properly
– Maintenance = keeping it in storage long enough to be useful
– Retrieval = recalling the information when it is needed

Processing Speed:
Time it takes to absorb new information and come up with a response.

Attention and Working Memory
Example: A friend tells you a 10 digit phone number – you keep it in your mind for about 30 seconds while you look for a pen and paper to write it down or enter it into contacts on your phone.

Executive Function:
* The cognitive processes that dictate flexible and dynamic adjustment of performance in response to a changing environment.
* Examples:
– Planning/organizing
– Complex or novel problem-solving
– Shifting attention, keeping track of, or alternating from 1 task to another
– Not being susceptible to distractions
– Generating fluent sequence of thought or words
– Learning the rules of a new task (example: programming new DVR) without direction or explicit cueing

Visuospatial Function:
[Slide showing 4 complex geometric drawings and various trapezoids, rectangles and parallelograms in the center of each.]
Given an isolated parallelogram, an impaired subject would have difficulty finding a parallelogram of the same shape within the complex geometric drawings.
* Causes problems with parking the car or clipping corners when driving
* Causes problems with directions somewhere that is not familiar and getting turned around

Language:
* Names of objects and people
* Problems can be simple:  ‘Tip of the tongue’
* Problems can be more severe:  You can’t remember the name of something you use regularly.

What is dementia?
* Comment: Alzheimer’s is the most commonly diagnosed type of dementia, but not the only one – by far.
* A person who can no longer do an activity of daily living because of a cognitive impairment has dementia.
* Examples:
– Can no longer pay the bills correctly
– Gets lost when driving
– Cannot shop for groceries

[Slide showing study results, “Cognitive Impairment in MSA: A Position statement by the Neuropsychology Task Force of the MDS Multiple System Atrophy (MODIMSA) Study Group,” Stankovic, et. al., Movement Disorders, vol. 29, no. 7, 2014.]

Autopsy verified diagnosis.
Table 1. Impaired cognitive functions in:
MSA (multiple system atrophy)
MSA-P (multiple system atrophy, parkinsonian variant)
MSA-C (multiple system atrophy, cerebellar variant)

MSA P+C
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous recall (immediate & delayed), recognition, visuospatial functions

MSA-P
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous immediate recall, visuospatial functions

MSA-C
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous delayed recall, recognition, visuospatial functions]

[Slide results from same study:]
* Prevalence of rates of cognitive impairment in autopsy confirmed MSA:
– 22% Mild Cognitive Impairment,
– 2% Moderate Cognitive Impairment,
– 0.5% Severe Cognitive Impairment
* 50% of those MSA patients with more than 8 years after diagnosis had some Cognitive Impairment
– Cognitive impairment seems to happen most frequently late in the disease process
* 14% of MSA patients had dementia in the last year before death
* 9% had well-formed visual hallucinations (one study)

[Slide showing study results, “Profile of Cognitive Impairment and Underlying Pathology in MSA,” Koga, et. al., Movement Disorders, vol. 32, no. 3, 2017.  Autopsy verified diagnosis.]

Table 2. Comparison of demographic and clinical features between 102 patients:
MSA-CI (multiple system atrophy with cognitive impairment) 33 patients
MSA-NC (multiple system atrophy without cognitive impairment) 69 patients

Data collected:
– Females with and without cognitive impairment
– Age at onset, in years
– Age at death, in years
– Disease duration
– Family history of dementia
– Family history of parkinsonism
– Having clinical diagnosis of MSA.  Only 70% accurately diagnosed.
– Clinical phenotype (both Parkinson’s and Cerebellar)
– Clinical symptoms of depression]

[Slide showing Characteristics of cognitive impairment in same 33 patients with MSA and cognitive impairment.  11 of the 33 had other pathologies that may have contributed to cognitive impairment, in addition to MSA pathology.]

What is meant by ‘psychosis’?
* Illusions – Misinterpretations of real stimuli. Can be unformed, such as a sense of presence or a fleeting false impression.  Example: see a jacket on a chair and misinterpret that it is a cat.
* Hallucinations – Spontaneous, fully formed. Most often visual, but can be auditory, tactile, olfactory (smell), or gustatory (taste).  Example: seeing there is a cat when there is not a cat.  With insight the person knows the hallucinations are not real.  Without insight they cannot know the hallucinations are not real; at this point, consider medication intervention.
* Delusions – False thinking. Can be paranoia.  Also, consider medication intervention.

Motor Symptoms – Sometimes Improved by dopamine medications
BUT
Hallucinations – Can be worsened by dopamine medications

Sudden Onset or Worsening of Hallucinations (Same as any worsening symptoms, including cognition.)
* Most common reason for a sudden onset or change in symptoms is general medical illness or other medications
– Infection (such as a UTI or a cold/flu)
– Medications (for pain, urinary frequency)
– Physical stress (constipation, poor sleep, travel, new physical environment)
– Emotional stress (anxiety)
– Being in the hospital (all of the above)

How to treat a Sudden Change
1. Deal with the underlying cause (prior slide)
2. Reduce or stop certain medications:
* Artane (any other anticholinergics)
* Amantadine
* Dopamine agonists
* Sinemet CR (carbidopa/levodopa CR)
* Comtan (entacapone)

What is the difference between MSA, Parkinson’s disease and Lewy body Dementia (LBD)?
Clinically:
Cognition = Memory and Hallucinations AND
Motor = Ataxia, Bradykinesia, Rigidity and Tremor WORSEN OVER TIME

Pathologically (only visible at autopsy):
[Slide showing Lewy body Pathology (image) in Both PD & LBD
Glial Cytoplasmic Inclusion Pathology (image) in MSA
Both Lewy Bodies and GCIs are make of up of the protein a-synuclein]

As is currently defined, all 3 would have autonomic dysfunction:
* The Diagnosis of PD with cognitive impairment or Dementia should be made when memory problems develops within the context of established PD.
* The Diagnosis of LBD is appropriate when the diagnosis of Dementia precedes or coincides within 1 year of the development of motor symptoms (parkinsonism).
* The Diagnosis of MSA is appropriate when the there is primary Autonomic Dysfunction, along with either parkinsonism or ataxia (with, or without memory problems).

Please take a 20 question online survey about MSA diagnosis and symptoms!!!
http://med.stanford.edu/poston-lab/msa-survey.html

QUESTION AND ANSWER
Lots of questions were received in advance.

Q. When will the diagnostic criteria be updated?
A. It is in the works.  So, probably the next year and a half.

Q. Why aren’t MDs able to determine if someone has MSA or LBD?
A. Even though in pathology the Lewy Bodies look different from the GCIs, there is no way to image them through an MRI, etc.

Q. Are there any specific assessments or screenings that you recommend for evaluating and monitoring an MSA patient’s cognitive status?
A. The screening a doctor would do during an office visit.  The Montreal Cognitive Assessment covers all the cognitive domains.  The problem is that is it fast (30 questions) so not detailed.  A neuropsychological battery, 2-3 hours of testing requires a PhD, clinical psychologist, to administer.

Q. Connection between NPH (normal pressure hydrocephalus) and MSA.
A. NPH can cause spaces in the brain to enlarge and press against other areas of the brain affecting urinary, cognition and gait.  The symptoms manifest differently from MSA and can be seen in an MRI.  That is one way to identify those with NPH and exclude MSA.

Q. What is sleep’s role in cognition?
A. A bad night sleep affects processing speed, executive function so address sleep apnea, have a nap, monitor blood pressure and minimize BP fluctuation to maximize cognitive function.

Q. How are executive functions affected in MSA?
A. This can come across as difficulty with multi-tasking because that requires a very high level of cognitive function.  Figuring out what to do in the right order is required.  When someone has trouble making a to-do list, there are some executive function issues.

Q. What is fatigue’s role in cognition?
A. Fatigue is very involved in blood pressure.  There is a lot of fatigue fundamental to each of these disorders.  Fatigue gets some improvement with napping or resting where blood pressure and dementia does not.

Q. How common are hallucinations in MSA?  Can there be hallucinations without Lewy body dementia?
A. Yes, recent studies have convinced clinicians.  That wasn’t known 10 years ago and Dr. Postonhas seen it in her clinic.

Q. How does dementia in MSA differ from Lewy body dementia?  Parkinson’s disease dementia?
A. It can be pretty darned similar.  The dementia, particularly the executive function issues, which is why the neuropsychological testing doesn’t differentiate between these.

Q. How does the cognitive impairment in MSA differ from that seen in Progressive supranuclear palsy (PSP)?
A. Patients with PSP can have a lot of executive function difficulty as well, but they often have poor judgement, even though they know their balance is terrible.  This can happen in other neurodegenerative disorders, but is more common in PSP,

Q. My husband is now very susceptible to TV ads and asks me to order products we do not need.  Is this executive function impairment?
A. Impulsivity can be part of the underlying disorder but can be made worse with dopamine agonists (Requip, Mirapex, Nupro patch).

Q. My husband is slow to respond, slow to make decisions, and has “on-and-off” periods.  Is this dementia?
A. It depends on the degree to which the on/off periods and indecision interfere with ability to complete activities of daily living.  On/off could be from medications or blood pressure fluctuations, so its hard to tell.

Q. Could MSA-related cognitive impairment be mistaken for vascular-related mild cognitive impairment?
A. The only study addressing that is the one showed where 4-5 had vascular disease found at autopsy that could have contributed to cognitive impairment.  It is impossible to tease out how much each contributes how much to cognitive impairment.

Q. How can you tell the difference between orthostatic hypotension and cognitive impairment?
A. The most common place you see this is when blood pressure stops and processing speed slows so much the person cannot answer questions. Blood pressure should be done during these episodes.  The tilt table test during cognitive testing can reveal whether the person has cognitive changes associated with blood pressure changes.

Q. In our local support group, people with MSA are taking Alzheimer’s medications such as Aricept and Exelon.  Does this mean they have dementia?  Lewy body dementia?
A. As far as medications to treat cognition, Alzheimer’s is the only disease FDA approved to treat.  Aricept, Exelon and Namenda are the most common.  These are not FDA approved for any other cognitive impairments.  The best data is behind Aricept and Exelon for Alzheimer’s and LBD.  They do help a little bit, but they can help for other cognitive and memory issues.

Q. How is the care of a person with MSA different from someone with dementia?  Or different from someone with other
neurological diseases (such as PSP or CBD)?
A. Longitudinal studies show the care of any two patients with MSA is completely different.  Care must be specifically tailored to each person.  One may have severe urinary issues and another not.  This is true of all the neurodegenerative disorders.

Q. If someone with MSA has cognitive issues, is his/her prognosis (survival time) any different?
A. Chance of having cognitive problems is most likely if they live 8+ years after diagnosis.  Once someone has a diagnosis of Parkinson’s disease with dementia their prognosis is shortened.

Q. How can medications affect cognition?
Q. If opiates can affect cognition and cause hallucinations, what kind of pain reliever can I give to my mother with pain?
A. Anything that treats pain dulls all neurons in the brain, blocking the sensation of pain.  Therefore simultaneously slows cognition, worsens hallucinations, etc.  Start with over the counter pain killers.  If you have to go to prescriptions discuss with doctor about what to try and start with lowest dose possible.

Q. Can cognitive problems be prevented in MSA?
A. We don’t understand what causes cognitive problems so we don’t have a way of preventing them.

Q. How can caregivers respond to someone with MSA and cognitive problems?
A. It is difficult because patients can get frustrated.  Patients do improve with a certain amount of cueing, reminding, etc.  Unfortunately, spouses call this nagging.  Try to know what the problem is by getting neuropsychologist testing and refer back to the test or have the doctor tell him/her what they have difficulty with.

Q.Do people with MSA not live long enough for dementia to show up?
A. No.

Q. Can people with MSA *also* have Alzheimer’s disease?
A. Yes.

Q. Can people with MSA *also* have Lewy body dementia?
A. Yes, but very rare.

Q. When do we determine when the patient is unable to work because decisions are cloudy or cognition is impaired?
A. Depends on the job and flexibility the employer has to move employee into a different role and relationship between the two.  Most of the time once executive functioning is impacted, it is difficult to be employed because decisions are too hard.

Q. Once the patient is non verbal and unable to move on his/her own accord, is this considered a disability automatically?
A. In filling out disability paperwork people qualify long before someone is nonverbal or unable to move.

Driving
Q. When do we determine when the patient is unable to drive because cognition is impaired?
A. This varies by state.  In CA if someone has dementia the physician is required by law to report them to the DMV for evaluation.  Occupational therapists can do an assessment, even with a driving simulator, to see what concerns might be, especially visuospatial changes.

Q. Importance of brain donation to MSA research.
A. Critical.  Without autopsy diagnosis confirmation we would still be guessing.  Before research based on brain donations we used to think MSA was 3 different disorders.  We can only continue research into MSA and other neurodegenerative disorders with continued access to brain tissue donated for research purposes.

“Caring for Someone with Lewy Body Dementia” – Live Chat Notes

Recently, Home Instead Senior Care hosted a conversation with Angela Taylor, Director of Programs for the Lewy Body Dementia Association (lbda.org). She provided a brief summary of what distinguishes Lewy Body Dementia (LBD) from some other dementias, followed by a lengthy question and answer period.

Lewy Body Dementia (LBD) includes both:
Dementia with Lewy Bodies (DLB) and
Parkinson’s Disease Dementia (PDD)

Treatment and behavior management is the same for both.

According to the research criteria, someone is diagnosed with DLB when both cognitive changes (hallucinations and fluctuating cognition) and parkinsonian symptoms (gait and balance difficulty and/or tremor) appear at nearly the same time.

And someone is diagnosed with PDD the cognitive changes appear more than a year after parkinsonian symptoms or a Parkinson’s diagnosis.

Brain Support Network uber-volunteer Denise Dagan recently listened to the webinar, and shares her notes below.

For additional resources on LBD, check out Brain Support Network’s list of Top Resources for LBD.

Robin

================================

Caring for Someone with Lewy Body Dementia
Home Instead Senior Care
June 7, 2018 Webinar

Live Chat with Angela Taylor of the Lewy Body Dementia Association

Lewy Body Dementia (LBD) is most common type of dementia after Alzheimer’s.  Each type of dementia has different caregiving challenges.

Dementia is an umbrella term which is a change in a person’s cognitive skills that interferes with daily functioning. There are over 100 causes of dementia. Some are treatable, like hydrocephalus. Most are progressive.

LBD is the most misdiagnosed form of dementia and affects 1.4 million Americans. It is a disease of older age (50+).  LBD affects more than just cognitive skills. Biologically, there are the same changes as those with Parkinson’s Disease.

Changes that affect movement. Those changes are called parkinsonism because they resemble Parkinson’s Disease.

Cognitive changes are not so much memory initially, but in planning, paying attention, understanding how things relate in 3D, fluctuations in their cognition even from hour to hour.

Changes in mood and behavior commonly seen in mental health disorders, like hallucinations (commonly people, animals or children) and delusions (when you think something is true, even with no facts to support the belief), depression and anxiety.

REM sleep behavior disorder – physically acting out dreams (hit, shout, kick, fall out of bed), or insomnia, restless leg, sleep apnea

Changes in autonomic nervous system, including digestion, constipation, low blood pressure.

Q&A

Q. How to best deal with hallucinations?

A. Up to 80% of people will have hallucinations. Sometimes, it is the first symptoms families notice. It can be frightening for the family, even if the subject of the hallucination is not frightening to the person with LBD. Hallucinations are a huge indicator that you are dealing with LBD as it is not a symptom that appears early in PD.

Caregiver should listen with your heart before listening with your brain. Respond to what your family member is feeling because you cannot convince them they are having a very real hallucination. First comfort their emotional state. Enter their reality. Engage in ‘therapeutic lies’ to help them deal with what they are experiencing. Try moving them to another location/room to change their visual experience. Turn off TV. Remove mirrors.

Q. How do you convince relatives who only see LBD symptoms on a person’s good days that there really are challenges?

A. People with LBD can really rise to the occasion when they are visiting with someone they are excited to see. The next day they are depleted and their Sx are all worse. If you want a relative to see the aftermath, you really need to ask them to visit for a few days to see the fluctuations in person. Provide the relative with educational material or forward them web resources links explaining LBD symptoms.  Sometimes, the relative is resistant due to fear and grief that they don’t want to believe the person with LBD is declining so dramatically.

Q. How to best deal with delusions?

A. Every situation is unique due to individual experience. Angela’s father became convinced his wallet was stolen. Capgras syndrome = someone believes a person in their household has been replaced by an imposter. Respond that you will fetch the person who has been replaced, leave for a few minutes and return. Often someone with LBD will not see their home as being their home. Therapeutic lies to calm fears or tell them what they believe is going on here is not happening where they live, then relocate them.

For both hallucinations and delusions you may have to try a few different calming comments before finding one they can buy into. As long as you remain calm and not escalate the situation, you will eventually be successful.

Q. Are there treatments for LBD or detrimental treatments for mis-diagnosis of LBD?

A. LBD has a lot of symptoms. There are medications that help many of those Sx. We can’t make dementia go away, but we can improve quality of life for both the patient and family caregiver. You really need a specialist for this because when you treat one area, you may make another worse. Treating autonomic symptoms nearly any doctor can do. Speech therapy can help as those muscles are affected. Physical therapy can help to counter stiffness and rigidity of parkinsonism rather than medications.
Those that treat cognitive skills (Nameda, Exelon) can improve hallucinations, anxiety, etc.  Treating movement symptoms medications can worsen hallucinations in LBD. May not treat minimal movement symptoms.  Treating behavior and mood symptoms (depression and anxiety) can improve quality of life and make them cooperative to care.
Treating hallucinations & delusions medications can worsen parkinsonism symptoms.

5 areas of symptoms in LBD:
Thinking
Movement
Behavior/Mood
Sleep
Autonomic dysfunction

LBDA recently added 24 major medical institutions as centers of excellence for LBD treatment. Increases opportunities for families to participate in research trials.

Q. Importance of early diagnosis, but how?

A. Start with your primary care physician. They should rule out underlying medical conditions. If they can’t figure it out they should refer to a neurologist. 2/3 of accurate diagnosis are done by neurologists, neuropsychologist, geriatricians. To diagnose, they may do a brain scan, cognitive evaluation or other tests to narrow the diagnosis.

Q. Family member with LBD symptoms include jerking of the arms and hands as well as staring unresponsively.

A. Staring into space is a manifestation of fluctuating cognition or orthostatic hypotension (low blood pressure). Parkisonism symptoms include masked face, stiffness, tremor. Jerking can be caused by medications used to treat parkinsonism or may be a symptoms of LBD. You should have a doctor see her.

Q. How quickly do motor skills decline? Do some symptoms present before others?

A. LBD is an umbrella term. There are 2 specific diagnoses.  Patients w/PD that then develop LBD have PDD. They have the same Lewy bodies in their brain but heir earliest problems are motor. DLB initially has cognitive issues, and later develop motor symptoms. Each individual will have varying degrees of intensity of symptoms between the 5 areas of symptoms in LBD.

If something has changed suddenly it is important to take your family member to see a doctor. It could be a medical issue that can be treated, but if it is physical changes it could be transition into a later stage of the disease. Only a doctor can determine this distinction. If so, it could be time to call in hospice to support the family.

Q. What should I look for in final stages of LBD and how to choose a good hospice?

A. There are no formal stages of LBD. Late stage is characteristic of being unable to care for themselves in all areas of daily living. Tell your Dr. if your family member having trouble swallowing so they can help you put services in place to support the patient and family, including hospice, speech therapy, feeding specialist, etc. Tell your Dr. if you think your family member may be in pain. Physical therapy & occupational therapy can bring in a hospital bed, special padding, etc. to make them more comfortable. Engage hospice and in-home care as early as possible to support the family so the family can do less hands-on care and focus more on bringing in experiences your family member will enjoy (music, art, movies, reading aloud, animals, family, etc.)

Q. How to deal w/LBD family member who has lost involvement in life?

A. LBDA has a private Facebook group so you can see what others are dealing with and share creative ways to cope. Talk to your Dr. about your family member’s change of activity and motivation. If it is depression it can be treated. Bring your family member’s passions to them or take them to their passions (Example: golf – can go putting, watch friends/fellow golfers at the driving range or a tournament, even watch a tournament on TV). Keep people socially stimulated, physically active and feeling they contribute value to society in addition to emotional support.

Dementia Resources:

– HelpForAlzheimersFamilies.com

– Facebook.com/RememberForAlzheimers

– HomeInstead.com

– LBDA Research Centers of Excellence: www.lbda.org/rcoe

– An Introduction to LBD Booklet: www.lbda.org/content/intro-to-lbd

– LBD Symptoms Checklist: www.lbda.org/go/comprehensive-lbd-symptoms-checklist

– LBD: State of the Science: www.lbda.org/go/lbd-state-science

 

Australian pop duo The Veronicas – mother with LBD and PSP

Australian pop duo The Veronicas were recently named ambassadors for Dementia Australia, after putting their music on hold last year to spend more time with their mother.  After four years of misdiagnoses, their mother was diagnosed with Lewy Body Dementia (LBD) and progressive supranuclear palsy (PSP). Their goal is to use their platform and their mother’s story to break down the stigma and shame surrounding dementia, and to build a social movement to advance community awareness.

Check out the article about The Veronicas here:

www.dailymail.co.uk/tvshowbiz/article-5892653/The-Veronicas-discuss-helping-mother-cope-dementia.html

Cancelled – “What If It’s NOT Parkinson’s?” Webinar on Tuesday, June 12th

Update on 6/11/18:  Unfortunately this webinar is now cancelled.

Brain Support Network group member Steven Russell shared with me today info on an upcoming webinar, which will give an overview of all four of the atypical parkinsonism disorders — MSA, LBD, PSP, and CBD.  Details are:

What If It’s NOT Parkinson’s?

In this webinar, Dr. Mihaela Alexander, Colorado Neurodiagnostics, will discuss atypical parkinsonisms such as multiple system atrophy and Lewy body dementia, among others (PSP, CBD). Dr. Alexander will cover what we know about these conditions and what treatments are available for each condition.

Tuesday, June 12
10-11am CA time

Organizer:
Parkinson Association of the Rockies, Denver, parkinsonrockies.org

Registration:
attendee.gotowebinar.com/register/3367947443153312002

General Information:  (same as what is above)
parkinsonrockies.org/programs-services/event-calendar/276/2018-06-12/what-if-it-s-not-parkinson-s-webinar

 

“Lewy Body Rollercoaster” – Alzheimer’s Weekly

Here’s an excerpt from an Alzheimer’s Weekly article from April 12, 2014 about Lewy body dementia (LBD):

www.alzheimersweekly.com/2014/07/lewy-body-rollercoaster.html

Excerpts from

Lewy Body Rollercoaster
April 12, 2014
Alzheimer’s Weekly

Attention, alertness and cognition have dramatic fluctuations in Lewy Body dementia. Caregivers call these ups and downs “The Roller-Coaster of LBD.”

“I watched my husband experience a decline in cognition followed by a period of what seemed like improved function only to plunge again into confusion with more frequent hallucinations,” says one caregiver newly acquainted with Lewy body dementia (LBD). According to the Lewy Body Dementia Association (LBDA), these ups and downs in function are sometimes refer to by family caregivers as the “roller-coaster effect” of LBD. Fluctuating levels of cognitive ability, attention and alertness are one of the core features of LBD.

Important Diagnosis
“The combination of the motor signs of Parkinson’s disease (slowed mobility, stooped posture and tremor) and mental confusion, especially if the degree of confusion fluctuates day to day, should raise a red flag for suspicion of LBD,” says Howard I. Hurtig, M.D., Chair, Department of Neurology, Pennsylvania Hospital, and Elliott Professor of Neurology, Perelman School of Medicine, University of Pennsylvania. “If those symptoms are accompanied or preceded by REM sleep behavior disorder (vivid dreams, dream enactment, hitting bed partner, falling out of bed) and visual hallucinations (seeing people, animals, etc., that aren’t truly there), then the diagnosis of LBD is almost certain. Even when all symptoms of LBD are present, it is critical for the evaluating doctor to look for underlying, TREATABLE causes of dementia.” A thorough assessment will include an inventory of daily medications (drugs can produce a “chemical” state of pseudodementia), CT or MRI scan of the brain, blood tests for thyroid function and vitamin deficiency and other causes of dementia that can be identified by routine study.

Following Alzheimer’s disease, LBD is the most misdiagnosed form of dementia but the second most common cause of progressive dementia, affecting 1.3 million Americans. LBD is associated with abnormal protein deposits in the brain, called Lewy bodies, that impair thinking, movement, sleep and behavior (causing people to see hallucinations or act out dreams, sometimes violently). Also, it affects autonomic body functions, such as blood pressure control, temperature regulation, and digestion. Recognizing symptoms early can help people with LBD get comprehensive and appropriate treatment and help caregivers get much needed support. It’s difficult to diagnose LBD, because its early symptoms resemble symptoms found in Alzheimer’s and Parkinson’s disease.

Man found joy in completing puzzles that were family photos

This article from last year is about twin daughters who discovered a company that created puzzles from family photographs, and that their father with Lewy body dementia (LBD) really enjoyed putting together these puzzles.

This activity might be of interest to more than those with dementia.

www.brainandlife.org/the-magazine/article/app/13/1/23/puzzle-power-as-lewy-body-dementia-narrowed-their-fathers-world

Congratulations to “Sue’s Story”

Congratulations to “Sue’s Story” — winner of the “Audience Choice Award” for the best documentary at last weekend’s Jasper Poppy International Film Festival in Morgan Hill, California. When Sue Berghoff was diagnosed with Lewy Body Dementia (LBD), her life was turned upside down. Like most people, she had never heard of the disease even though it is the second most prevalent form of dementia after Alzheimer’s. She encountered a society in which the existence of dementia is burdened by misconceptions, fear and shame. What she did next created a ripple effect of hope and change. Sue’s husband Chuck Berghoff is a member of Brain Support Network’s  LBD caregiver support group. Brain Support Network’s CEO Robin Riddle appears in the documentary, speaking about the importance of brain donation for LBD and the value of a caregiver support group. Stay tuned to Brain Support Network for more news about the distribution of “Sue’s Story.”

Capgras syndrome – almost 20% of people with LBD have

This is a sad but helpful article on Capgras syndrome, a specific type of delusion where a person believes that loved ones have been replaced by identical duplicates. They often believe that the loved one has been kidnapped and the “imposter” is a bad person. This is very common within our local Lewy body dementia support group. According to the article, one report showed a prevalence of 16.6 percent of those with LBD have Capgras syndrome.

Here’s a link to the article in today’s Washington Post:

 

www.washingtonpost.com/national/health-science/this-strange-syndrome-causes-people-to-think-their-loved-ones-have-been-replaced-by-identical-impostors/2018/04/06/0091f168-1be6-11e8-9de1-147dd2df3829_story.html

Health & Science
This strange syndrome causes people to think their loved ones have been replaced by identical impostors
Washington Post
By Meeri Kim
April 7, 2018

Robin

 

Anosognosia – lack of awareness (not denial) of one’s own dementia

This short article from Next Avenue (nextavenue.org) is about anosognosia, or the lack of awareness of one’s own dementia.  This is not denial but being unaware.  “This lack of awareness can cause major stress and heartache for caregivers.”

Here’s a short excerpt:

Both of Kathy Kling’s parents, who are divorced, have Alzheimer’s. Kling recently talked with her mother, Karen Kelly, about her father’s disease. “Oh, I hope I never get it,” her mother replied.  She was diagnosed six years ago.

The full article is here:

www.nextavenue.org/parent-doesnt-recognize-dementia/

When Your Parent Doesn’t Know He Has Dementia
It’s a common aspect of the disorder, but tough on caregivers
By Emily Gurnon, Health & Caregiving Editor
Next Avenue
March 28, 2018

Robin