Actress Dina Merrill had Lewy body dementia

Actress Dina Merrill died recently.  She was a guest on many TV shows, including “Bonanza,” “Mission: Impossible” and “Murder, She Wrote.”  Her son reported that she had Lewy body dementia.  The New York Times obituary says nothing about her LBD journey.

Here’s a link to the obituary:

www.nytimes.com/2017/05/22/movies/dina-merrill-dead-actress-and-heiress.html

Robin

Who converts from Pure Autonomic Failure to PD, DLB, and MSA?

There’s been quite a bit published this year about those with “Pure Autonomic Failure” converting to Parkinson’s Disease (PD), Dementia with Lewy Bodies (DLB), or Multiple System Atrophy (MSA).  (All three disorders are alpha-synucleinopathies.)

PAF is a disorder of the autonomic system.  The autonomic system controls things that the body generally handles automatically such as blood pressure, heart rate, eye blink, body temperature, sweating, digestion, etc.

This article, published in February 2017, is authored by the Autonomic Disorders Consortium, a group made up of the key autonomic specialists in the US.

In this study of 74 subjects at five US medical centers (NYU, Vanderbilt, Mayo Rochester, NIH, and Harvard), about one-third (34%) developed DLB (n=13), PD (n=6), or MSA (n=6) over four years. Overall, 14% of people converted from PAF to one of the three alpha-synculein disorders each year.  Many of those who converted had REM sleep behavior disorder (RBD).

Other symptoms were associated with who got MSA, DLB, or PD:

* “Patients who phenoconverted to multiple system atrophy had younger age at onset of autonomic failure, severe bladder/bowel dysfunction, preserved olfaction, and a cardiac chronotropic response upon tilt > 10 beats per minute.”  The “younger age” was early 50s.  On average, those in the PAF group who converted to MSA had PAF symptoms for fewer than five years.

* “Those who phenoconverted to Parkinson disease or dementia with Lewy bodies had decreased olfaction, a lesser chronotropic response to tilt, and a longer duration of illness.”  “Longer duration of illness” refers to the fact that, on average, those in the PAF group who converted to PD or DLB had PAF symptoms for nearly ten years.

And:  “The small group of patients retaining the pure autonomic failure phenotype had very low plasma norepinephrine levels, slow resting heart rate, no REM sleep behavior disorder, and preserved smell.”

Here’s a link to the full article (available at no charge online):

www.ncbi.nlm.nih.gov/pmc/articles/PMC5323269/

The abstract is copied below.

Robin

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Annals of Neurology. 2017 Feb;81(2):287-297.

Natural history of pure autonomic failure: A United States prospective cohort.

Kaufmann H, Norcliffe-Kaufmann L, Palma JA, Biaggioni I, Low PA, Singer W, Goldstein DS, Peltier AC, Shibao CA, Gibbons CH, Freeman R, Robertson D; Autonomic Disorders Consortium.

Abstract
OBJECTIVE:
To define the clinical features and biomarkers that predict which patients with pure autonomic failure will develop Parkinson disease, dementia with Lewy bodies, or multiple system atrophy.

METHODS:
One hundred patients who presented with pure autonomic failure were recruited at 5 medical centers in the United States. Seventy-four patients agreed to be followed prospectively. Patients underwent clinical evaluations including neurological rating scales, sleep questionnaires, smell test, and sympathetic and parasympathetic cardiovascular autonomic function tests.

RESULTS:
At enrollment, patients were 68 ± 12 years old (median ± interquartile range) and had had autonomic failure for 5 ± 7 years. Within 4 years of follow-up, 25 of 74 subjects (34%) developed dementia with Lewy bodies (n = 13), Parkinson disease (n = 6), or multiple system atrophy (n = 6). The presence of probable rapid eye movement (REM) sleep behavior disorder was strongly associated with the development of a manifest central nervous system (CNS) synucleinopathy (odds ratio = 7.1). Patients who phenoconverted to multiple system atrophy had younger age at onset of autonomic failure, severe bladder/bowel dysfunction, preserved olfaction, and a cardiac chronotropic response upon tilt > 10 beats per minute. Those who phenoconverted to Parkinson disease or dementia with Lewy bodies had decreased olfaction, a lesser chronotropic response to tilt, and a longer duration of illness. The small group of patients retaining the pure autonomic failure phenotype had very low plasma norepinephrine levels, slow resting heart rate, no REM sleep behavior disorder, and preserved smell.

INTERPRETATION:
Patients presenting with pure autonomic failure are at high risk of phenoconverting to a manifest CNS synucleinopathy. Specific clinical features predict future diagnosis.

© 2017 American Neurological Association.

PMID: 28093795
www.ncbi.nlm.nih.gov/pubmed/28093795

Lewy Body Dementia Info on Dementia Aide (website)

Dementia Aide, a relatively new website (dementiaaide.com), is focused on selling what it calls dementia-related products.  While a few things such as t-shirts are disorder-specific, most of the products are caregiving items.  They have pages on their website for Alzheimer’s Disease (AD), frontotemporal dementia, vascular dementia, and Lewy body dementia (LBD).

The LBD section, written in September 2016, won’t be added to our list of “Top Resources” but it’s worth checking out.  They seem to have pieced together information from lots of different resources (not always giving attribution every place they could.)  For example, the chart on the difference between LBD, Parkinson’s Disease (PD), and Alzheimer’s is straight from the Lewy Body Dementia Association but this is only pointed out in one place (not everywhere the chart is).

You might check out their infographic on what they say are the four stages of LBD (on the symptoms page).

The only obvious error I saw was that they don’t have an accurate description of “Lewy body dementia” within the Lewy body disease family.  They show Lewy body dementia is the same thing as Dementia with Lewy Body.  Actually, Lewy Body Dementia is an umbrella term that refers to both Dementia with Lewy Bodies and Parkinson’s Disease Dementia.

Here’s a link to the LBD section:

www.dementiaaide.com/pages/lewy-body-dementia

Robin

Short descriptions of four atypical parkinsonism disorders on MJFF website

Looks like this webpage on the four atypical parkinsonism disorders — CBD, LBD, MSA, and PSP — was recently created on the Michael J. Fox Foundation website.  (It wasn’t there in July 2016, when we became one of their partners.)  Here’s a link to the new webpage:

www.michaeljfox.org/understanding-parkinsons/living-with-pd/topic.php?atypical-parkinsonism

Below, I’ve copied the summaries of the four disorders from the short webpage.  In addition to these summaries, the webpage also discusses treatment for these diseases.

Robin
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Excerpts from

Atypical Parkinsonism
Michael J. Fox Foundation Webpage
Un-dated

Corticobasal Degeneration (CBD)
Corticobasal degeneration (CBD) leads primarily to motor and cognitive (memory/thinking) symptoms. Motor symptoms mainly affect one arm and/or hand and include:
* slowness,
* stiffness,
* myoclonus (rapid muscle jerks), and
* dystonia (an abnormal, fixed posture).

The dystonic posture may cause the arm to be held close to the body and bent at the elbow and the wrist and fingers to be flexed toward the palm. Dystonia can cause pain and palm sores and interfere with regular daily activities (such as brushing teeth or preparing meals). Cognitive problems can affect speech, memory and/or behavior. Brain-processing difficulties can make performing complex motions, such as combing hair or turning a key in a lock, challenging or impossible. People with CBD may also experience “alien limb phenomenon,” which is involuntary activity of a limb and a feeling that the limb is foreign or has a will of its own. (An alien hand could take one’s eyeglasses off after the other hand has put them on, for example.)

Lewy Body Dementia (LBD)
Lewy body dementia (LBD), also known as dementia with Lewy bodies (DLB) is a form of dementia associated with PD, typically occurring early in the course of disease. LBD involves motor symptoms of Parkinson’s (usually stiffness and slowness) and significant impairment of thinking and/or memory abilities that interferes with daily activities. Additional symptoms may include:
* visual hallucinations (seeing things that aren’t there),
* unpredictable fluctuations in levels of alertness or attention, and
* mood, behavioral and/or personality changes.

REM sleep behavior disorder, in which a person acts out his or her dreams, and orthostatic hypotension (a decrease in blood pressure when changing positions that can cause dizziness or lightheadedness) are other common symptoms.

Multiple System Atrophy (MSA)
Multiple system atrophy (MSA) patients may experience:
* parkinsonism — usually slowness, stiffness and walking/balance difficulties (rather than tremor);
* cerebellar symptoms — incoordination, imbalance and/or slurred speech; and
* autonomic nervous system dysfunction — problems with the body’s automatic activities such as blood pressure regulation, bladder emptying and sexual functions.

Other features of MSA include abnormal postures (head and neck tilted forward, hand held in a grasping position, or foot and ankle turned inward); speech and swallowing problems; episodes of uncontrolled laughter or crying (pseudobulbar palsy); cognitive (memory/thinking) problems; and sleep disturbances, including REM sleep behavior disorder (acting out one’s dreams) or sleep apnea (breathing pauses during sleep).

Progressive Supranuclear Palsy (PSP)
Progressive supranuclear palsy (PSP) causes imbalance, gait difficulties and a tendency to fall backwards. It also restricts normal eye movements, which can lead to reading difficulties, falls when walking down stairs and visual disturbances (blurred or double vision, or light sensitivity). Involuntary eyelid closure (called blepharospasm); memory and behavior changes (such as decreased motivation and emotional fluctuations); and speech and swallowing problems also may occur.

Synucleinopathy: How Long You Live Depends on Which One You Have

We posted earlier this week about the Mayo Rochester research into lifespan for Parkinson’s Disease, Parkinson’s Disease Dementia, Dementia with Lewy Bodies, and Multiple System Atrophy, as compared to those without these disorders.

This is a good Alzforum explanation of the same research:

www.alzforum.org/news/research-news/synucleinopathy-how-long-you-live-depends-which-one-you-have

Here are a few excerpts from the Alzforum article:

* “Prior studies have reported survival rates for various parkinsonian disorders; however, most of these recruited from hospitals rather than the general population, and none compared α-synucleinopathies side by side.”

* David Irwin, University of Pennsylvania wrote to Alzforum:  “The comparison of survival…highlights the powerful effect of cognitive impairment and dementia to predict a poor prognosis across the PDD/DLB spectrum.  Further, there is limited data on the natural history of MSA, and this paper provides new insight into the relatively rapid progression of this disease.”

* “[Mayo Rochester researcher] Savica said his group has submitted one autopsy study for publication, and will expand on pathology in an upcoming project.”

Neurological Disorders Playlist? (Dysautonomia Playlist)

Dysautonomia or autonomic dysfunction is a set of symptoms that commonly occurs in multiple system atrophy and, to some extent, Lewy body dementia.  Here’s a playlist of 25 therapeutic music videos/songs from the Dysautonomia Support Network (dysautonomiasupport.org), which posts its blog on The Mighty:

https://themighty.com/2017/03/dysautonomia-songs/

Despite the playlist title — “The Ultimate Dysautonomia Playlist” — I think this is a great playlist for anyone coping with a challenging neurological condition.

Shorter life span in MSA-P, DLB, and PDD compared to PD and controls

This is more research out of the Mayo Rochester Epidemiology Project, looking at 461 people in Olmsted County, MN who were diagnosed with a synucleinopathy with parkinsonism between 1991 and 2010.  Synucleinopathies included were Parkinson’s Disease (PD), dementia with Lewy bodies (DLB), Parkinson’s Disease Dementia (PDD), and multiple system atrophy-parkinsonism (MSA-p).  These were matched with county residents without parkinsonism.

Those with MSA-p died 6 years earlier than others with synucleinopathies, and those with DLB (4 years) or PDD (3.5 years) had a shorter lifespan than normal controls.  And having PD took one year off a person’s lifespan.

Here’s a MedPage Today article about the research:

www.medpagetoday.com/neurology/parkinsonsdisease/65304

Higher Death Risk With All Synucleinopathies
Lowest with Parkinson’s disease, highest for multiple system atrophy with parkinsonism
by Kristin Jenkins
Contributing Writer, MedPage Today
May 15, 2017

(You can view the article once without signing up.  Signing up is free.)

Robin

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Updated in July 2017:

The article described above has this citation:

Savica R, Grossardt BR, Bower JH, et al. Survival and causes of death among people with clinically diagnosed synucleinopathies with parkinsonism: a population-based study. [Published online May 15, 2017]. JAMA Neurol. Accessed June 8, 2017.

Recently, Clinical Neurology News published these five questions to test your knowledge of outcomes in synucleinopathies — dementia with Lewy bodies, Parkinson’s disease dementia, multiple system atrophy, and Parkinson’s disease:

www.mdedge.com/clinicalneurologynews/quiz/4815/movement-disorders/outcomes-synucleinopathies-parkinsonism

Most of the questions are about DLB, PDD, and MSA.  The questions are based on the JAMA Neurology article.

 

 

Urinary Problems in PD – Webinar Notes

The Michael J. Fox Foundation (michaeljfox.org) hosts webinars every third Thursday on various Parkinson’s Disease (PD) topics.  The April 2017 hour-long webinar was on urinary symptoms in PD.  The speakers addressed how PD affects the autonomic nervous system, including bladder functions; how urinary problems are diagnosed and managed; and the latest in research.

Certainly many in the Brain Support Network community cope with urinary symptoms.  During the webinar, alpha-synuclein is mentioned.  Both multiple system atrophy and Lewy body dementia are disorders of alpha-synuclein.

The webinar recording is available online here:

www.michaeljfox.org/understanding-parkinsons/webinar-registration.php?id=23&e=1389435&k=8EDACA15229E6F2DA1A8C61247716FDD

(You’ll need to register first to obtain access to the recording.)

Brain Support Network volunteer extraordinaire Denise Dagan listened to the webinar and took notes.  Her notes are copied below.

Sorry but the Fox Foundation doesn’t announce its webinar presenters in advance, and that information is not posted to its website.  So I’m unclear who all the presenters were.  One of the presenters is Dr. Maria De Leon, is a retired movement disorder specialist who also has Parkinson’s Disease.   Other presenters were Dr. Miyasaki and Dr. Juncos.  The moderator is always Dave Iverson, a journalist who has PD.

Robin

—————————–

Denise’s Notes

Urinary Problems in Parkinson’s Disease
Michael J. Fox Foundation Webinar
April 20, 2017

The Autonomic Nervous System Controls the Body’s Automatic Functions:
* Blood pressure
* Heart rate
* Temperature
* Digestion
* Sexual function
* Bladder control

Constipation can affect bladder control.  Urinary and sexual function are under treated because symptoms are attributed to aging, rather than to Parkinson’s disease.

Autonomic Problems are Common in Parkinson’s:
* Up to 80% of people with PD may experience an autonomic symptom during their disease course.
* Autonomic symptoms are likely due to the underlying disease process of Parkinson’s, but can be worsened by certain PD and other medications.
* The protein alpha-synuclein, which misfiles in PD, may play a role in autonomic dysfunction.

Alpha-synuclein not only collects in, and damages, the brain, but affects the periphery of the nervous system as well (ganglia and nerve roots of the autonomic nervous system) leading to the degeneration of those nerves.  Most of the symptoms caused by this degeneration can be managed, including by PD meds that treat motor symptoms.  Sometimes these meds make autonomic symptoms worse.  Tell your Dr. so he/she can adjust meds for best autonomic symptom treatment.

In general non-motor symptoms tend to cluster together.  Over time people accumulate more non-motor symptoms, including autonomic symptoms.  There needs to be a balance of symptom treatment with medications -vs- side-effects of those medications.

PD Urinary Problems May Include Difficulty Holding or Emptying Urine:
The bladder stores and empties urine.  In Parkinson’s, the brain’s control of the bladder is disturbed.
* Difficulty holding ruing may lead to:
— Strong urges to urinate
— Increased frequency of urination, especially at nighttime
— Accidental loss of urine (incontinence)
* Difficulty emptying urine could cause:
— Hesitancy when starting urination
— Weak stream
— Feeling of incomplete bladder emptying
* Difficulty holding and emptying urine can lead to urinary tract infections.

Dr. DeLeon initially experienced increased urgency.  10 years into her diagnosis she has discovered it is not one single factor causing bladder problems.  Not just worsening PD, or needing medication adjustment, but aging, diabetes, prostate enlargement, etc., comes into play.

Most common urinary symptoms in PD:
* Irritative symptoms – noctural frequency, daytime urgency, incontinence (leaking), daytime frequency
* Obstructive symptoms – hesitancy, poor flow, incomplete emptying
* Aging contributes to all of these symptoms.

How do you sort out what’s caused by PD and what’s due to aging, enlarged prostate, etc.?
* Best practice in diagnosis is building a multidisciplinary team to determine what is going on with the patient.
* Uro-dynamics is a test whereby the bladder is filled and its function is monitored.
– In overactive bladder any amount of content causes contraction, urgency, therefore frequency.
– In obstructive bladder there is difficulty in flow.  When caused by enlarged prostate, it can be treated with meds and/or surgery.
* Treatment begins with least invasive to more invasive.

Have an open conversation with your physician about urinary issues because most symptoms have a treatment if the cause can be determined.  Patients should not assume new urinary difficulties are associated with PD and/or aging, but mention it to your doctor and be persistent, especially if it becomes a quality of life issue for yourself or your caregiver/family.  Keep track of your urinary behaviors and symptoms to best help your doctor(s) determine the cause of your bladder and constipation issues.

(Dr. DeLeon found her constipation was causing bladder obstruction, so treating the constipation eased bladder issues).

Another issue is difficulty with movement impeding getting to the toilet in time, getting clothing closures undone in time, etc. due to increasing PD symptoms.

Listener question about his mother having frequent urinary tract infections.  In reply, an MD says incomplete emptying of the bladder is common in people with PD due to improper functioning of the bladder muscles, especially in older men due to enlarged prostate. Leaving urine in the bladder is the perfect medium for bacterial growth and resulting in frequent urinary tract infections (UTI). These can be treated with antibiotics, even chronic prophylactic antibiotics (although this puts you at risk of antibiotic resistance), and surgical intervention.  Elderly people can not realize they have a UTI, which can adversely affect PD symptoms, PD medications don’t work as well, and seem just as though they are having a bad day because the older you are the less prominent the symptoms of s UTI.  Systemic UTI (beyond the bladder) can cause confusion, hallucinations, and ER visits.  Because of this, UTI must be in the fore of your mind when and older person with PD is feeling under the weather.

Treatment Targets the specific Urinary System:
Difficulty holding urine
> Non-pharmacologic
– Pelvic floor exercises
– Limit fluids/caffeine, schedule bathroom breaks, use incontinence aids
> Pharmacologic
– Medication to relax the bladder
– Botulinum toxin injections

Difficulty eliminating urine
> Pharmacologic
– Medication to stimulate bladder emptying
– Evaluate current drugs to ensure none contribute (e.g., Artane/trihexiphenidyl)
> Non-pharmacologic
– Intermittent catheterization

* Consider seeing a urologist or other doctor with expertise in the urinary system to compete urodynamic testing and determine if symptoms are from Parkinson’s or other issues.

* Tracking symptoms can be useful in managing these problems.

Listener question: How do PD meds complicate urinary problems, particularly frequency?  MD answer:  Generally, PD meds do not cause bladder problems.  Used to use anti-cholinergics (for people with tremor), including Amantadine, which can result in urinary retention or inability to void.  Other meds for non-motor symptoms, like depression (Mertazapine) has anti-cholinergic affects, as well.

Also, low blood pressure during the day can result in having to get up frequently at night to pee because sitting and standing the kidneys don’t have high enough blood pressure to produce urine, and laying down at night increases blood pressure and allows kidneys to produce urine and fill the bladder.

How does one reconcile conflicting advice about staying hydrated to maintain blood pressure, and limiting fluids to compensate for difficulty in holding one’s urine?  Fluids help with constipation, which affects your ability to void.  After 6:00pm don’t drink a lot of fluids to minimize nighttime urination.

Dr. DeLeon contributes fluids are especially important during the hot months of the year, but during the daytime.  Also avoid caffeine, chocolate, and spicy foods which can all make you pee more often.

Pelvic floor exercises are often prescribed for women with respect to birth.  Try to stop the stream while you pee to find the muscles to exercise.  Don’t do this while you pee to prevent urine retention and UTIs.  Both men and women should do this exercise several times to a count of 10 throughout the day to strengthen pelvic floor muscles.  This prevents leakage and helps to void completely.

What medications can be helpful?
– What can be aggravating the situation so can be eliminated or modified to improve the situation, especially diuretics, opioids, amantadine, anti-cholinergics, calcium channel blockers.  Work with the physician team to adjust medications.
– Other medical conditions that can aggregate bladder symptoms, like BPHD, atrophic vaginitis, prior abdominal surgeries, how many children you have had, sleep disorders, diabetes, venus insufficiency, etc.
– Medications to help the bladder relax or minimize irritation and contracting before getting to the toilet.  These are anti-cholinergics but not those that stimulate the bladder.  There are many choices, like Detrol, or Vesicare, which has been studied on PD patients.  There are potential side-effects.  Beta3 receptor, Myrbetriq, works but may cause high blood pressure.
– Medications to improve emptying by relaxing the sphincter (Flomax, Rapiflow) and reducing the size of the prostate.  Some of these drop blood pressure more than others.
– In PD patients with motor fluctuations, minimizing OFF periods reduces urge to empty the bladder, especially when one cannot move well.

Dr. DeLeon commented about what’s been most helpful, personally. Many women tend to have greater risk of UTI and urgency from taking Azilect, but it helps her with pain so she has to find a way to work around balancing symptom treatment.  She was taking Myrbetriq and anti-spasmotic, but everything (even behavior therapy) only helps for awhile.  Dopamine can inhibit release of insulin and found she was becoming insulin resistant.  Even though she is not diabetic, she is on blood sugar medication, which stopped her bladder problems and she was able to stop taking Myrbetriq.

Dave asked Dr. Miyasaki about connection between blood sugar levels and bladder issues.  There is a close connection between the brain and the gut, including the pancreas.  Adding an endocrinologist to your care team is warranted.  PD patients have an increased risk of diabetes, statistically, but the reason is unknown.  Some diabetes meds increase kidney excretion of glucose resulting in urinary frequency.

Ongoing Research into Urinary Problems and Parkinson’s
* Trials are investigating the brain mechanisms involved in overactive bladder, as well as varied treatments.
> Medications = e.g., Melatonin
> Behavioral modifications = pelvic floor exercises, and Bladder routine/schedule
> Transcutaneous electrical nerve stimulation = Non-invasive stimulation of lower leg nerves through skin device.

Dr. Miyasaki agrees that starting with the least invasive treatments is wise.  Melatonin has multiple benefits to patients, especially for sleep.  It is difficult to determine the benefit of behavior modifications, but they are not harmful and can be beneficial so they are worth a try.  There are reports that transcutaneous electrical nerve stimulation help with both frequency and difficulty emptying.  People with PD can have a less common disorder where the sphincter of the bladder will not relax.  It can be quite painful and risks UTIs.  People who have had DBS report better sleep and less urinary frequency, especially at night.

Q&A
More questions about how much fluid and when it should be consumed?
8oz, 6-8 times daily until 6:00pm – depending on whether you are taking diuretics.

Any connection between bladder problems and development of kidney stones?
If you’re not able to void regularly you may develop kidney stones, but they have more to do with your body eliminating various minerals or whether you’ve had repeated infections.  If you are well hydrated, kidney stones shouldn’t be a problem.

Dr. Miyasaki feels strongly that your neurologist is connected with other specialists so each patient has a multidisciplinary care team, especially those who are interested in treating Parkinson’s disease within their specialty, like urology, and see a volume of patients to really develop an expertise in treating Parkinson’s patients overall.

Dr. Juncos doesn’t want people to forget Botox can be tremendously beneficial to urinary treatment (and other non-motor symptoms) in Parkinson’s disease and can be used repeatedly.  Also, men are offered prostate surgery to reduce urinary obstruction, but that will not treat the autonomic symptoms, so what level of benefit can they expect from the surgery?  Ask a lot of questions before you do the surgery.

Dr. DeLeon reminds people there are many treatment options for urinary issues and there is no reason to be embarrassed.  Bring it up with your doctor and be patient in determining the problem and treatment.  Keep the symptom diary for ALL PD symptoms.  It is infinitely useful in your own PD care.

Webinar on Sleep Issues in Parkinson’s, May 18, 9-10am (CA time)

May’s Third Thursday Michael J. Fox Foundation (michaeljfox.org) webinar on sleep issues in Parkinson’s might be of interest to those dealing with Lewy Body Dementia and Multiple System Atrophy as REM sleep behavior disorder (RBD) is a problem in all three disorders.  The webinar (no charge) is on Thursday, May 18, 9-10am California time.  You can register in advance to participate or register afterward in order to view the recording.  Details are below.

Robin
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“Sleeping Well with Parkinson’s”

Program:  Sleep disturbances are a common non-motor symptom of Parkinson’s disease that may cause difficulty falling or staying asleep. In this webinar, we’ll discuss sleep disorders that can occur in Parkinson’s, how to manage them and current research on sleep and PD.

Presenters to be announced at the time of the program

Hosted by: The Michael J. Fox Foundation for Parkinson’s Research

Register by clicking on the REGISTER NOW button at:
www.michaeljfox.org/page.html?hot-topics-webinar-series&navid=webinar-series

Notes from webinar on three non-AD dementias, including Lewy Body Dementia

There was a 90-minute webinar on April 6th, hosted by Resources for Integrated Care, was packed with lots of info on dementia with Lewy bodies (DLB) and frontotemporal dementia (FTD) with less info on vascular dementia (VAD).  (Note that throughout the webinar, with few exceptions, the term “Lewy body dementia” was used.)  The webinar, for both healthcare professionals and family caregivers, was fast-paced.  Presentations were for the first hour, and then there was a 30-minute Q&A.

You can find a link to the webinar recording here:

www.resourcesforintegratedcare.com/GeriatricCompetentCare/2017_GCC_Webinar_Series/Beyond_Alzheimers

You can find a link to the webinar slides here:

www.resourcesforintegratedcare.com/sites/default/files/Beyond_Alzheimers_Disease_0.pdf

VAD, DLB, and FTD are the most common dementia types after Alzheimer’s Disease (AD).  Here are short definitions:

* Vascular dementia – cognitive deficits most often associated with vascular damage in the brain, either micro or macro in nature.

* Dementia with Lewy Bodies – a dementia that also includes one or more of these core findings: recurrent and detailed visual hallucinations, parkinsonian signs, and fluctuating cognition.

* Frontotemporal dementia – a disease often seen in individuals with onset of cognitive symptoms at a younger age; these individuals present most often with executive and language dysfunction and significant behavioral changes.

The management strategies that are effectively in AD are less effective in these other three dementia types.

Brain Support Network volunteer-extraordinaire Denise Dagan listened to the webinar a couple of times and took extensive notes on the LBD content and some notes on the other two dementias.  She has provided the time stamp at various points so that you can fast forward through the recording, if you’d like.

Robin

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Denise’s Notes from

Beyond Alzheimer’s Disease, Other Causes of Prerogative Dementia in the Older Adult
Webinar hosted by Resources for Integrated Care
April 6, 2017

Presenters:
* Melinda S. Lantz, MD, Chief of Geriatric Psychiatry, Mount Sinai Beth Israel Medical Center, New York, NY
* Geri Hall, PhD, ARNP, CNS, FAAN, Banner Health, Phoenix, AZ
* Rebekkah Wilson, MSW, Dementia Care Consultant and Trainer
* Sharon Hall, Family Caregiver (to someone with FTD)

After many housekeeping comments and credits, introduction of the webinar presenters begins at time stamp 3:15.

DR. LANTZ SPEAKS….

DEMENTIA

After some audience polling, Dr. Lantz begins at time stamp 9:15 with how dementia is identified and diagnosed by evaluation as there are no clinical tests yet available to diagnose any type of dementia.

Dementia is a major neurocognitive disorder.  According to the American Psychiatric Associations Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (APA DSM5), to be diagnosed with dementia a person must exhibit significant decline from a previous level in the five domains of cognitive functioning, which include:
* Complex attention
* Executive function
* Learning and memory, language
* Perceptual-motor
* Social cognition

Diagnosis is based on collateral information including self-reporting, standardized neuropsychological testing or quantified clinical assessment.

Cognitive deficits interfere with everyday activities, social or occupational functioning.

MCI

Mild Neurocognitive Disorder [Mild Cognitive Impairment (MCI)], according to the APA DSM5, does not meed criteria for dementia.  MCI is described as:
* Subjective and objective decline in cognitive domain: primarily memory, language or motor
* No significant impairment in:
— Other cognitive domains
— Activities of Daily living
— Social or occupational functioning
* 10-15% of patients with MCI progress to develop dementia each year
* “Precursor of dementia” versus spectrum of normal aging
* Patients with MCI should be identified and monitored for cognitive and functional decline due to their increased risk for dementia
* There are no currently FDA approved medications for MCI

VASCULAR DEMENTIA

Vascular Dementia = dementia due to cerebrovascular disease.
* Cognitive loss due to cerebrovascular disease
* 2nd most common cause in late-life after Alzheimer’s.
* Risk factors:
— Hypertension (high blood pressure)
— Diabetes
— Hyperlipidemia
— Smoking
— TIAs (transient ischemic attack) or mini-stroke is a neurological event with the signs and symptoms of a stroke, but which go away within a short period of time.
— Stroke.
* Decline may be abrupt due to a stroke or series of TIAs
* Cognitive loss may be focal, with more awareness of symptoms
* Disturbance of emotions and mood is common
* Caregiving needs are due to medical and physical conditions (paralysis of one side of the body due to stroke, and multiple medical problems and medications).
* Decline may be step-wise with plateaus in symptoms.  They may recover after 1st stroke, but decline years later due to more strokes.
* Mixed variants of dementia (Alzheimer’s with vascular dementia) are common.
* More men than women because men have more vascular disease than women.

She displayed a scan of vascular dementia at time stamp 13:45

She inserted a slide of two other types of vascular dementia at time stamp 14:00, but didn’t discuss them.

LEWY BODY DEMENTIA

Lewy Body Dementia
* Memory loss and other cognitive deficits that often have a fluctuating and variable course, and relatively rapid onset
* Motor rigidity, parkinsonism features
* Prominent hallucinations, usually visual
* Unsteady gait, syncope (fainting), unexplained falls
* More rapidly deteriorating course
* 3rd most common dementia after Alzheimer’s and Vascular dementias
* more common in men than women

She displayed a microscope image of a lewy body at time stamp 15:30

Sensitivity to all psychiatric meds, but definitely psychosis requiring meds, which makes it complex to treat.

FRONTOTEMPORAL DEMENTIA

Frontotemporal dementia at time stamp 16:12
* Significantly earlier onset between 40-60 yrs old because of slow and subtle onset
* Atrophy prominent in the frontal and temporal lobes of the brain (associated with personality, mood, behavior, impulse control)
* Slow onset with early changes in personality, impulse control and language
* Memory, arithmetic, copying figures often preserved until later in the course so difficult to diagnose early on
* Behavior often disinhibited, repetitive, socially inappropriate
* Prominent personality change very early
* Early onset with very slow, progressive decline
* Memory impairment later in the course
* Behavior changes: Disinhibition, impulsivity, apathy, depression, verbal outbursts
* Lack of recognition, agnosia (Inability to interpret sensations and hence to recognize things, people and/or sounds.  With agnosia they are particularly unable to recognize their own changes in behavior and capabilities, hampering diagnosis and caregiving.)
* Treatment is very symptom driven as there are no agents available for prevention or slowing progression

Two subtypes: Pick’s disease and frontotemporal dementia with parkinsonism at time stamp 17:52

She displayed a Pick body microscope image at time stamp 18:05

TREATMENT

Treatment of Cognitive Symptoms of Vascular and Lewy body Dementia:
* Cholinesterase Inhibitors slows the rate of decline:  Donepezil, Galantamine, Rivastigmine, for mild to moderate Alzheimer’s (AD), Vascular dementia (VAD), mixed the benefits relatively similar: +3 points ADAS-Cog, 4 to 6 Month delay in progression
* Individual response variable and difficult to measure:  20% of patients show “Greater than average” response, 20% “some response”, but 30-50% show no response
* Greatest effect appears to be delay in need for nursing home placement of 6 to 8 months
* NO ROLE for use in Frontotemporal Dementia (FTD), may worsen behavioral symptoms

Treatment of Cognitive Symptoms:
* Rivastigmine may be more helpful in LBD than the other available cholinesterase inhibitors
* Memantine is approved by the FDA for Alzheimer’s, but not enough evidence to show benefit for other types of dementia
* Cholinesterase inhibitors have no benefit in FTD and may worsen mood and behavior
* Cholinesterase inhibitors may cause nausea, vomiting, weight loss (due to GI upset) and bradycardia (slow heart rate)

Pharmacologic Treatment for targeted severe symptoms:
* The idea is to minimize symptoms without side effects, especially from poly-pharmacy.
Use of these is justified when patients are disturbed by hallucinations, especially if they try to act on them in dangerous ways.
* Not a substitute for good care and behavioral management.
* Psychosis (hallucinations, delusion) > Rx options: Antipsychotic Agents (risperidone, olanzapine, quetiapine, aripiprazole)
* Depression, Anxiety, & Irritability > Rx options: SSRI (sertraline, citalopram) or bupropion, trazodone, mirtazapine)
— These are particularly common early in these dementias, when mood treatment can be particularly helpful.

Pharmacologic Treatment for physical aggression:
* These are all off-label drug uses, but can be helpful.
* Severe Physical aggression (also helpful for severe impulsivitity and mood lability > Rx options: Mood Stabilizer (valproate, lithium, carbamazepine, gabapentin)
* Moderate Physical Aggression > Rx options: Mood Stabilizer

She put up three slides with Dosing Guidelines and Side-Effects charts for antidepressants and mood stabilizers at time stamp 23:37

DR. HALL SPEAKS….

Symptom presentation in dementia depends on these factors and directly affect care challenges:
* Location of the degeneration
* Function of the degenerated area of the brain
* Pathologic changes at the cellular level (e.g. presence of Lewy bodies)
* Comorbid conditions
* Environmental factors producing excess disability
* Premorbid personality

At time stamp 25:22 she put up PET scan imagines distinguishing dementias from each other (kind of amazing).

The location of damage is different, therefore so are care needs:
* Due to time constraints we will examine two more common non-Alzheimer’s dementias:
— Lewy Body Dementia (LBD)
— Frontotemporal Lobar Degeneration (FTD or FTLD)

LEWY BODY DEMENTIA

Lewy Body Dementia:  Three common presentations
* Regardless of the initial symptom, over time all three presentations of LBD will develop very similar cognitive, physical, sleep and behavioral features.
* Some individuals will start out with a movement disorder leading to the diagnosis of Parkinson’s disease and later develop dementia.  This is diagnosed at Parkinson’s disease dementia.
* Another group of individuals will start out with a cognitive/memory disorder that may be mistaken for Alzheimer’s disease, but over time two or more distinctive features of fluctuating cognition and psychosis (hallucinations and/or delusions) become apparent leading to the diagnosis of ‘dementia with Lewy Bodies’ (DLB).
* Lastly, a small group will first present with neuropsychiatric symptoms, which can include hallucinations (primarily visual, but also olfactory or auditory), behavioral problems, and difficulty with complex mental activities, also leading to an initial diagnosis of DLB.

Common Symptoms of Lewy Body Dementia:
* Sleep disorders (can be present up to two years prior to LBD diagnosis)
— Acting out dreams while asleep
— Excessive daytime sleepiness
— Restless leg movement
* Impaired thinking
— Executive function (planning, processing information.)
— Memory fluctuates
— Ability to understand visual information fluctuates
* Problems with movement
— Tremors, stiffness, slowness and difficulty walking
— Anti-Parkinson medications often don’t work well and may cause initial hallucinations
* Altered sensory perception (particularly visual-spacial perception)
* Hallucinations
— Often of animals or children
* Behavioral and Mood Symptoms
— Depression, apathy, anxiety, agitation, delusions or paranoia
* Changes in Autonomic Body Functions
— Blood pressure control, temperature regulation, postural control (tend to fall a lot), bladder and bowel function
* Exquisite Sensitivity to medications, particularly those that affect the central nervous system.
— Don’t use old generation antipsychotics.  With new antipsychotics, start with a low dose and ramp up to an effective dose.
— Don’t use general anesthesia.

Care of Lewy Body Dementia:
* Similar to Alzheimer’s – decrease stimuli, increase rest, promote exercise (Big & Loud Programs) and balance
* Safety due to REM sleep disorder, fall precautions due to autonomic dysfunction, swallowing issues
* Supportive (self-care) activities of daily living (ADLs)
* Control of misleading environmental stimuli (especially TV) and medications that trigger hallucinations and delusions
* Prepare family for response to potential aggression
* LBD Association and support groups

Use of Therapies:
* Physical therapy – Design a program and teach to patient/family focusing on postural stability and core strength
* Exercise Programs – Big and LOUD Programs!!!
* Recreational Therapy
* Occupational therapy – ADLs
* Speech pathology – swallowing, spoken volume
* Pharmacist consultation – OTCs can be problematic and interact, so ask before taking anything.

Lew Body Dementia Support and Resources slide includes a list of resources, including encouragement to attend local support group meetings.

Robin’s note:  See Brain Support Network’s list of top LBD resources here, which includes the two resources Dr. Hall mentioned:

www.brainsupportnetwork.org/education/lewy-body-dementia/

Denise’s note:  If you are in the San Francisco Bay Area, please attend Brain Support Network’s LBD caregiver-only support group meetings!  We just met last Sunday.  Our next meeting is in June.  Email Robin Riddle to get on the meeting reminder email list.

FRONTOTEMPORAL DEMENTIA

She spoke about Frontotemporal Dementia (FTD) from time stamp 31:29 until her summary at 46:43.  FTD is often misdiagnosed for 10-14 years and is the most difficult to care for, because of youth and psychiatric issues.

SUMMARY

Summary:
* As diagnostic specificity improves, non-Alzheimer’s dementias will be diagnosed more frequently.
* A “one-side-fits-all” care program will not meet the needs of people with non-Alzheimer dementias.  They differ in terms of symptom presentation, behavioral responses, and ability to tolerate medications.  [When searching for a day program or caregivers for your family member with a non-Alzheimer’s dementia diagnosis, interviewing for their understanding of they type of dementia your family has, and their preparedness in caring for that type of dementia, is critical in finding the right program or caregiver.]
* Families and care providers are desperate for answers, ongoing support, and to seek out others suffering from similar conditions.
* Interdisciplinary care and research is essential for humane approaches to these vexing conditions.

MS. WILSON SPEAKS…

Rebekkah Wilson, MSW spoke next at time stamp 46:29

Beyond Alzheimer’s Disease, Impact on the Individual/Family System:
* Challenges with diagnosis
* Symptoms generally less recognized/understood
* Caregiver burden
* Younger onset considerations
* Community resources and considerations
— Legal
— Financial
— Emotional support
— Care options

Psychosocial Impact – Diagnosis
* Misdiagnoses common
— in FTD as late onset mental health issues (bi-polar), or marital problems
— in LBD when initial presentation is non-motor symptoms
* Rearview mirror clearer; but by then there are consequences of actions taken prior to diagnosis
— unnecessary treatments, or surgery
— impulse control issues in FTD can result in overspending of college & retirement funds, or loss of jobs & stability
* Sense of relief to get diagnosis
— Negative experiences prior to diagnosis
— Empowerment through learning about diagnosis (now I know what I’m dealing with)
* Present and future support – Programs are set up on an Alzheimers disease model.  Some areas of the country are more progressive and have more support groups and day programs/caregivers for non-Alzheimer’s dementia.

Psychosocial Impact – Symptoms
* Less recognized because Alzheimer’s disease is everyone’s reference point
– Memory may not be impacted, so dementia not recognized and symptoms not addressed
* Safety considerations due to brain changes (driving, wandering, judgement)
* Fluctuations in mental status misinterpreted (esp. in young patients misdiagnosed as bi-polar, marital discord, OCD, etc.)
* hallucinations/REM
* Impact of FTD symptoms
– Legal
– Financial
– Employment
– Social

Psychosocial Impact – Caregiver Burden
* Nature of Symptoms
— FTD – especially behavior and language changes
— LBD – especially cognitive fluctuations (can be moment to moment, rather than good morning & bad afternoon)
* High caregiver stress
* Isolation due to being a ‘rare’ diagnosis (need to find a support group with the same non-Alzheimer’s diagnosis)
* Age of onset impacts normalization (especially FTD & young onset Alzheimer’s)
— Expect memory and cognitive changes in older adults.
Younger people with dementia are misdiagnosed with mental health issues or arrested for unruly public behavior.
— Parents providing care for person with dementia (PWD)
— Care for PWD while caring for kids in household (sandwich generation)
* Ambiguous loss / anticipatory grief

Psychosocial Impact – Young Onset
* Developmental stage of the family
— Young kids at home, frequently in young onset families.
— Aging parents caring for their children with young onset dementias.
* Concerns about genetics
* Career and employment considerations
* Financial implications – primary caregiver may now have to work (maybe more than one job, depending on their skill set.)
* Challenges finding services because they are not trained to care for non-Alzheimer’s dementia

Legal and Financial Considerations
* Legal planning documents (maybe guardianship or conservatorship)
— Power of Attorney for Healthcare
— Power of Attorney for Finances
— Living Will
* Employment Laws
* Early Onset diagnosis impacts income and insurance (pre-Medicare age)
* Medicare
* Social Security Disability
* Social Security Compassionate Allowance to expedite benefits
* Additional option for financing care

The Compassionate Allowances (CAL) initiative is a way to expedite the processing of SSDI and SSI disability claims for applicants whose medical conditions are so severe that their conditions obviously meet Social Security’s definition of disability.  It is not a separate program from SSA’s two disability programs, SSDI and SSI.

There is no special application or form that is unique to the CAL initiative.  Individuals with a CAL condition apply for benefits using the standard SSA process for filing claims for SSDI, SSI or both SSDI and SSI benefits.  SSA will expedite the applications of those with a CAL condition.  Applications for disability may be filed online, in the local field office, or by calling 1-800-772-1213.  To learn more about the CAL initiative, see:

www.ssa.gov/compassionateallowances/

Community Resources and Services
* Support Groups for both caregiver AND the person with a diagnosis
* Care models in programs, including day facilities and nursing facilities are based on Alzheimers, but expanding.
* Families are using online information & medical community to educate care models.
* Limited experience and understanding of non-Alzheimer’s dementia

Care Planning
* Establishing the care team (Neurologist, PT, OT, Pharmacist, speech therapist, and the primary caregiver.)
* Determining goals for care team, based on location of care (home or facility) (comfort & safety).  Focusing on the primary goal(s) helps to make decisions.  For example, when choosing a care facility for someone with LBD, focusing on fall prevention as the primary goal may eliminate several options.  Narrowing the number of choices makes the decision easier.
* Support for the person with the diagnosis
* Knowledge and support of the care partners
– Family/friends
– Professionals

Challenges with Home & Community Based Care in Non-Alzheimer’s dementias
* Facility care for FTD
* Cognitive fluctuations as a challenge for care in LBD
* Hospice / palliative care & denials of admission because guidelines are based on Alzheimer’s

Best Practices for Home & Community Based Care
* Recognize care for non-Alzheimer’s dementias is different than Alzheimer’s
* Structure very important
* “Failure free,” low demand engagement
* Group versus individual activities (For Alzheimer’s people, group activities work well, while for young onset individual and non-Alzheimer’s dementias, independent activities work better)
* Recognize preferences may differ from other residents if younger
* Risky or impulsive behavior may increase safety concerns.

MS. HALL SPEAKS…

Sharon Hall spoke last about being a family caregiver of two types of dementia simultaneously at time stamp 1:07:47

Cares for both her 90+-year-old mother with vascular dementia and her husband with bvFTD – yikes!

Feels most difficult aspects is changing role from daughter/wife to caregiver through ambiguous loss process.

Challenging to early onset patient in finding long-term care, especially day programs.  Her husband is physically robust so people don’t expect his behavior to be dementia, but think he’s joking or has mental illness.  Day programs don’t have activities he’s interested in.

Physical contact is ok for people with Alzheimer’s, but if you touch someone with FTD they WILL touch you back.  They also say inappropriate things.  She tried handing out cards excusing his behavior, but she finds talking with people about it, even including her husband in that conversation, educates people better.  Sometimes, they don’t believe her.

Meds are not the only way to manage behavior, especially in FTD, and can make things worse.  It is better to be their advocate and understand how to manage behavior without it.  You can’t expect someone with dementia to follow standard rules of behavior.  You have to go with whatever they’re doing, as long as it’s not dangerous or too disruptive.

Being their advocate, especially in understanding where difficult behaviors come from, is key.  Employing their assistance in as many activities of daily living as they can manage, throughout the day but including rest periods, is the best way to minimize behavior problems.  It occupies their attention, keeps them out of trouble and from being bored, and makes them feel they still are useful members of the family and society.

There’s a crazy financial burden with young onset dementia families, because they had to stop working early so they qualify for less SSI than people who retire later.  Especially people with FTD, because of spending compulsions, can spend through college and retirement savings so must be taken off all financial accounts as soon as diagnosis is made.

FTD has unusual eating habits, crave carbs, etc.  Hygiene can be an issue, but nobody ever dies from not taking a shower.  Forcing participation in conventional hygiene and other tasks on someone with non-Alzheimer’s dementias can bring on unwanted behaviors.  Understanding what you’re dealing with and working with them is the key.  If it’s not dangerous, let them do what they want to do.

QUESTION AND ANSWER

Q&A at time stamp 1:16

Sharon, what techniques work best to manage behaviors.
I apologize to him if she sees she’s done something to trigger a behavior.  Being on his side diminishes the behavior.

Rebekkah, can a healthcare surrogate with no family member present, place a dementia patient in assisted living or LTC if its in their best interest?
A medical decision maker can

Dr. Hall, do you have suggestions for alternatives when access to site services is a challenge?
Psychiatric services, but start with medical and neurological workup.

Dr. Lantz, FTD has been connected with concussion or head trauma?
No (listen for longer answer)

Dr. Hall, are sensory used to treat patients with challenging behaviors arising from dementia?
Sensory rooms are used to treat dementia.  (listen for longer answer)

Dr. Lantz, In early FTD what techniques do we use for early treatment and diagnosis?
monitor and evaluate symptoms and mitigate problems.  PET scan.  Team diagnosis approach, especially behavioral neurology training will better recognize FTD.

What do any of you know about a web based tool called WE Care Advisor geared to help family caregivers?
Nobody has heard of it.

You all stress the importance of interdisciplinary team supporting dementia and caregivers.  Non-medical support?
Area Agency on Aging, the Alzheimer’s Association, access to social services and medical help, as well.
FTD & LBD have national resources but those 2 have local resources.
Join a support group to share resources, moderators educate on resources in these groups.

Dementia friendly communities?  How do I find one?
They look at developing activities and resources places for people with dementia, including painting, coffee shops with dementia-firendly hours.  Locations are based on minimal stimulations, etc.  People with FTD have a harder time participating because their dementia peers with other diagnosis can’t communicate with them well.  Your local newspaper may have done an article on the opening of one in your area to help you find one near you.