Webinar Notes – Sleep Issues in LBD, MSA, and PD

Yesterday, the Lewy Body Dementia Association (lbda.org) hosted a good one-hour webinar on sleep problems in Lewy Body Dementia (LBD), also known as Dementia with Lewy Bodies (DLB). Most of the webinar is of relevance to sleep issues in Multiple System Atrophy (MSA) as well.

The presenter, a sleep disorders neurologist at UCLA, addressed these topics:
* function of sleep
* how much sleep do we need
* obstructive sleep apnea (OSA)
* REM sleep behavior disorder (RBD)
* restless legs syndrome (RLS)
* insomnia
* conclusions about RBD and DLB

I was surprised that excessive daytime sleepiness was not addressed during the presentation. I suppose since the sponsoring pharmaceutical company is studying a drug for RBD, that was really the focus.

The highlight of the webinar was the question-and-answer session, which was well-facilitated by Angela Taylor of the LBDA. The questions were about:
* RLS and diabetic neuropathy
* excessive daytime sleepiness
* napping
* melatonin dosage
* neurodegenerative disease risk

My detailed notes from the webinar are below.

The presentation is here:
lbda.org/downloads/lbda-sleep-webinar-slides.pdf

The webinar recording is here:
youtube.com/watch?v=bnHQwduxGSA
(Note: there’s a problem with the slides for the first eight minutes or so.)

Robin

———————–

LBDU Webinar: Sleep Issues in LBD

Presenter: Dr. Alon Y. Avidan, MD, MPH, Professor of Neurology, Director of the UCLA Sleep Disorders Center.

Notes from presentation:

Theory that sleep is restorative. Memory is consolidated. If you don’t sleep well, your memory and cognitive abilities may decline.

Sleep is rejuvenative. Brain’s glymphatic system is most active during sleep. The brain “takes out the trash” while we sleep. Trash = byproducts and toxins. The function of the glymphatic system was only characterized in the last few years. Lack of good sleep puts the patient at risk for more disease and poor health.

14:13 Most adults need 7-8 hours. If less than 4 hours, you are putting yourself at risk for heart disease, depression, diabetes, and cardiovascular disease. Sleep needed varies by age groups. National Sleep Foundation recommends 7-9 hours for adults. Later, the American Academy of Sleep Medicine recommends 7-8 hours; less or more than that is not good. Healthy sleep duration is 7 hours or more each night (regularly). Good sleep on weekends is important.

Less than 7 hours/night regularly, puts you at risk for weight gain and obesity, diabetes, hypertension, heart disease, stroke, depression, increased risk of death, depressed immune function, increased pain, poor performance, increased risk, and increased accidents.

16:48 Sleep is often affected by aging process but it doesn’t have to be this way. As we age, we have more pain, more sleep apnea, more RLS, more comorbid disorders, and take more medications. Don’t be satisfied with 4-5 hours of sleep. Make an effort to improve sleep quality and duration.

OSA
18:10 Obstructive sleep apnea is affected by age, weight, alcohol. Alcohol can convert someone from simple snoring to sleep apnea. 20-80 times to stop breathing in one hour! CPAP therapy is gold-standard treatment.

RBD
20:20 This is the most important sleep disorder in those with LBD. REM sleep behavior disorder is a type of parasomnia (abnormal behavior in sleep). Muscles are supposed to be paralyzed when dreaming. In RBD, patients act out dreams. Concerns are self-injury or injury of bed partner. Incomplete transition from REM sleep to non-REM sleep (where you are nearly awake). REM sleep without atonia.

RBD is common in alpha-synucleinopathies (PD, DLB, MSA). RBD can present before the onset of neurodegenerative disease. Usually 2/3 of patients will develop neurodegenerative disease within 10 years.

Sleep neurologists should tell patients diagnosed with RBD that they are “at risk for dementia later in life.”

RBD is part of the diagnostic criteria for DLB.

Dreams in RBD are rarely pleasant.

26:10 Treatment focuses on safety: bedroom safe; remove hard/sharp objects; sleep in padded mattress; place mattress on floor; cover windows with heavy curtain; use pillow barricades. Until managed, sleep alone. Sleep in sleeping bag until treated. Medications: melatonin (he prefers because it’s the safesty; 5mg up to 15mg), clonazepam (.25 to .5mg; had side effects, such as grogginess).

RBD could be a window of opportunity in DLB. Nelotanserin clinical trial is ending at the end of June 2017. Lead institution is Mayo.

RLS
28:19 Restless legs syndrome. Urge to move the legs occur primarily in the evening. Many LBD patients have this condition. Very bothersome. Often physicians don’t know how to diagnose RLS. Symptoms get worse with inactivity. Difficult to relieve leg discomfort. Driving or flying long distances – especially difficult. Effective treatments available.

Insomnia
29:46 One-third of patients with neurodegenerative disease are affected by insomnia, particularly middle-of-the-night insomnia. Alcohol is not a good idea for insomnia. Get out of bed; avoid staying in bed awake. Talk to your MD about potential treatments.

30:40 Conclusions
* sleep disorder increases odds of DLB by 5x over Alzheimer’s
* RBD is strongest prognosticator of dementia, including DLB

Future research into RBD will focus on:
* benefit of exercise
* role of dietary factors (dairy products, saturated and animal fat, lower use of Mediterranean diet and of non-steroid drugs)
* role of melatonin as a neuroprotective agent
* establish guideline about agents that can help prevent phenoconversion from RBD to DLB

 

31:48 Notes from Question-and-Answer session:

Q: RLS and diabetic neuropathy
A: Common situation. The medication gabapentin can address both problems. Talk to a PCP. RLS diagnosis must be validated.

 

Q: Excessive daytime sleepiness is common. How do you know if sleepiness is excessive?
A: Well-validated measures of EDS to assess what is abnormal and what is not. Epworth Sleepiness Scale (ESS) can be used. You can find the scale online. Falling asleep immediately upon watching TV at any time of day, for example, is excessive.

 

Q: What is the maximum number of nap-time that will not disrupt nighttime sleep?
A: If “hours,” it’s already not good. Naps should be short (15-20 minutes) and strategic (1-3pm). Sleep is not like a bank account.

 

Q: What treatments are there for EDS?
A: Stimulants should not be the focus. We should use good sleep to give us energy. Exception to the “avoid medications” rule is narcolepsy, which is very rare. Shift workers or patients with sleep apnea who are still fatigued could be given stimulants. But I would never give someone with EDS a stimulant because this doesn’t address the problem of poor sleep. First find out what is causing the sleep disruption. One thing that can be helpful in treating LBD sleepiness is light. Light exposure, especially early in the day, is important. We don’t have good data on wake-promoting agents in LBD.

 

Q: Dosage of melatonin for LBD for sleep or RBD?
A: Melatonin for RBD – high-dose melatonin (3mg, increasing by 3mg every two weeks up to 12mg). We have good data on RBD. Could consider 5mg sustained release melatonin. This increases by 5mg every two weeks up to 15mg. If 12mg or 15mg don’t work, consider adding clonazepam. If that doesn’t work, look again into the cause of RBD. Often RBD is due to other substance patient is taking but forgot reporting initially to MD.

Low-dose melatonin (.5mg) – circadian rhythm problems. Middle-dose melatonin – insomnia.

 

Q: Is RLS or sleep apnea associated with neurodegenerative diseases?
A: No data whatsoever that RLS puts you at risk for neurodegenerative disease. Nor is it a prognosticator.

Untreated sleep apnea puts you at risk for accelerated neurodegeneration, if you already have a predisposition for development of Alzheimer’s.

 

Q: If you have RBD and receive treatment for it, can you reduce risk of neurodegenerative disease?
A: We don’t know. If you use clonazepam, you are probably not going to reduce risk of disease. We don’t know for sure about melatonin. Some believe that melatonin is neuroprotective. But patients taking melatonin don’t have slower progression towards neurodegeneration. Disease process isn’t reversed.

RBD is a great biomarker. This means that we can use it to test neuroprotective agents.

 

Angela Taylor, LBDA:
LBDA research page — lbda.org/participate-in-research

Enrollment for one RBD study has been extended.

 

Sleep issues in LBD and MSA, Thursday 6/22, webinar

The Lewy Body Dementia Association (lbda.org) is hosting a webinar this Thursday 6/22 at 11:30am California time on sleep issues in Lewy body dementia (LBD).  The content also applies to those in the multiple system atrophy (MSA) and Parkinson’s Disease (PD).

Sleep issues to be addressed by a UCLA sleep disorders specialist include REM sleep behavior disorder, daytime sleepiness, restless leg syndrome, insomnia, obstructive sleep apnea, and periodic limb movement.  There is no charge to attend.  Details below.

Updated, 6/23/17:  See our blog post of the notes from this webinar:

www.brainsupportnetwork.org/webinar-notes-sleep-issues-in-lbd-msa-and-pd/

Robin

—————————–

lbda.org/sleep

Webinar – Sleep Issues in LBD
Thursday, June 22, 2017
2:30 pm Eastern Time

Did you know that most people with LBD have at least one sleep disorder?
From REM sleep behavior disorder, which causes frightening dreams that sufferers often act out, to daytime sleepiness, restless leg syndrome, insomnia, obstructive sleep apnea, and periodic limb movement, those with LBD often have sleep issues that dramatically effect their quality of life and can lead sometime lead to injuries to themselves and others. In addition, the sleep disorders associated with LBD can begin years to decades earlier than other common LBD symptoms such as memory loss or confused thinking.

Join LBDU and Dr. Alon Y. Avidan, MD, MPH, Director of the UCLA Sleep Disorders Center for a free, informative webinar on sleep issues in Lewy Body Dementia.

Dr. Avidan will explain changes in sleep patterns with aging specific to Lewy Body Dementia. He also will share information about the management of LBD-related sleep disorders, as well as treatment strategies, ongoing research and clinical trials.

Presenter:
Dr. Alon Y. Avidan, MD, MPH
Professor of Neurology
Vice Chair Clinical and Educational Affairs
Department of Neurology
Director of the UCLA Sleep Disorders Center
David Geffen School of Medicine at UCLA

“Dreams and Brain Disease: REM Sleep Cells Linked to Disorders”

This article on last week’s Live Science (livescience.com) is about REM sleep behavior disorder (RBD), which is acting out dreams. This symptom in common in Parkinson’s Disease, Dementia with Lewy Bodies, and Multiple System Atrophy. The vast majority of those with RBD have one of these three disorders and often one of these neurological disorders comes to light years (or decades) after the first signs of RBD.

Here’s a link to the full article:
http://www.livescience.com/59300-brain-cells-linked-to-dreaming-found.html

Live Science
Health
Dreams and Brain Disease: REM Sleep Cells Linked to Disorders
By Tracy Staedter, Live Science Contributor
May 30, 2017 07:06pm ET

June 2017 Parkinson’s Support Group Meetings – Guest Speakers – NorCal + Central CA

Here’s a list of guest speakers at many Northern California and Central California PD support group meetings for June 2017.

With my Brain Support Network atypical parkinsonism (DLB, PSP, MSA, CBD) hat on, these meetings are especially appealing to me (because of the guest speakers or topics) BUT remember that these are PD support group meetings:

Santa Rosa, Sat 6/3:  Neurologist speaks about mood, cognitive, and sleep disorders in PD.  This might be applicable to those with DLB and MSA.

Roseville, Tues 6/6:  Learn from a pharmaceutical company rep about a new drug for hallucinations and delusions.  This is applicable to those with DLB.

Sonoma/Vintage House, Thurs 6/8:  Topic is balance and fall prevention.  Speaker unlikely to know about any of the atypical parkinsonism disorders specifically but she probably has some good suggestions.

Stockton, Thurs 6/8:  Medical marijuana is the topic

Gilroy, Mon 6/12:  Listening to and discussing Michael J. Fox Foundation podcasts on sleep disturbances and urinary problems in Parkinson’s.  This is applicable to those with DLB and MSA especially.

Pacific Grove (Monterey County), Tues 6/13:  Speech therapist talks about speech and swallowing changes in PD.  This is applicable to all the disorders in our group.

Palo Alto Young Onset Parkinson’s Group Tues 6/13:  Medical cannabis is the topic

Palo Alto/Avenidas, Wed 6/14:  Manager of Stanford’s Farewell to Falls program will be speaking on the topic of fall prevention.  This is applicable to everyone in our group, whether you can take advantage of Stanford’s program or not.

Sacramento/Arden Arcade, Thurs 6/15:  Movement disorder specialist Lin Zhang, MD, PhD will be addressing the non-motor symptoms of PD.  These symptoms are part of the disorders in our group.

Mill Valley, Fri 6/13:  Registered dietitian speaks about nutrition and PD.  Most of the information should be applicable to those in our group.

Fremont, Mon 6/26:  Movement disorder specialist Han Lee, MD will be the guest speaker.  Unfortunately we don’t know his topic.  But he is very familiar with all the disorders in our group.

Generally, I recommend driving no more than 30 minutes to attend any of these meetings.  If you attend a meeting and learn anything, please share with me so that I can share with others!

Do you need to know the support group meeting location, day/time, contact info, and how to RSVP if required?  Please refer to the Stanford
Parkinson’s website for all Northern and Central California support groups:

parkinsons.stanford.edu/support_groups.html

As always, I’ve deleted the deep brain stimulation-related talks.

Robin

**********************

Half Moon Bay
Thursday, 6/1, 3-4pm
Guest Speaker:  Cherry Tuck, PD fighter
Topic:  Her journey
RSVP?:  No.

San Jose/Willow Glen
Friday, 6/2, 10am-noon (program starts about 10:20am)
Program:  Break into two groups — those with PD and caregivers
RSVP?:  No.

Santa Rosa (Sonoma County)
Saturday, 6/3, 1-3:15pm  (guest speaker 1-2pm)
Guest Speaker:  Allan Bernstein, MD, neurologist, Santa Rosa
Topic:  Mood, cognitive, and sleep disorders in Parkinson’s
RSVP?:  No.

Lodi
Monday, 6/5, 10-11am
Guest Speaker:  Dianna Powell, San Joaquin County coordinator, Legal Services of Northern California
Topic:  HICAP and Medicare updates
RSVP?:  No.

Roseville
Tuesday, 6/6, 1:30-3pm
Guest Speaker:  Saul Avila, Acadia Pharmaceuticals
Topic:  Nuplazid – new drug for psychosis and schizophrenia associated with Parkinson’s
RSVP?:  No.

San Francisco/UCSF Young Onset Parkinson’s Group
Tuesday, 6/6, 6:30-8pm
Guest Speaker:  Cameron Wisdom, Mission Bay Rock Steady Boxing Gym, San Francisco
RSVP?:  Yes, preferred to Monica Volz, [email protected]

Soquel (Santa Cruz County)
Wednesday, 6/7, 1-2:30pm
Guest Speaker:  Jenifer Armstrong, PharmD, pharmacist, Santa Cruz
Topic:  PD – Inside and out of the prescription bottle
RSVP?:  No.

Chico
Wednesday, 6/7, 1:30-3pm
Guest Speaker:  Attorney, Corporon Law Offices
Topic:  Long-term care planning (trusts, wills, and other legal documents)
RSVP?:  No.

Sonoma/Vintage House
Thursday, 6/8, 10-11am
Guest Speaker:  Vanessa Kettler, balance instructor
Topic:  Balance and fall prevention
RSVP?:  No.

Stockton
Thursday, 6/8, 1:30-3pm
Guest Speaker:  Christopher Trinchera
Topic:  Medical marijuana
RSVP?:  No.

St. Helena/Rianda House  (new group)
Thursday, 6/8, 3:30-4:30pm
Guest Speaker:  Barbara Brown, PT, physical therapist, St. Helena Hospital
Topic:  Importance of a PT’s expertise in a PD care plan
RSVP?:  No.

Fresno
Saturday, 6/10, 10am-noon
Guest Speaker:  Beate Ritz, MD, PhD, UCLA
Topic:  PEG (Parkinson’s, Environment & Genes) study at UCLA
RSVP?:  No.

Yuba City (Tri-Counties)
Monday, 6/12, 1-2pm
Guest Speaker:  Carly Pacheco, deputy director, FREED Center for Independent Living, Grass Valley
Topic:  FREED Center’s services
RSVP?:  No.

Gilroy
Monday, 6/12, noon-1:30pm (new time)
Program:  Listening to and discussing Michael J. Fox Foundation podcasts on sleep disturbances and urinary problems in Parkinson’s
RSVP?:  No.

Bakersfield
Tuesday, 6/13, 2-4pm
Guest Speaker:  Lin Zhang, MD, PhD, movement disorder specialist, UC Davis, Sacramento
Topic:  PD and the management of off episodes with Apokyn
RSVP?:  Yes to group leaders Linda Feist, 661-304-9227, or Bill Burgemaster, 661-343-2707

Pacific Grove (Monterey County)
Tuesday, 6/13, 3-4:30pm
Guest Speaker:  Katie Pietsch, SLP, speech therapist, CHOMP
Topic:  Think LOUD! – Speech and swallowing changes in PD
RSVP?:  No.

Palo Alto Young Onset Parkinson’s Group
Tuesday, 6/13, 6:30-8pm
Guest Speaker:  Helen Garvy, PD advocate and care partner
Topic:  Medical cannabis for PD
RSVP?:  Preferred, if this is your first time.  RSVP at least 24 hours in advance to Martha Gardner, group leader, email [email protected]

Turlock
Wednesday, 6/14, 1-2pm
Guest Speaker:  Robert McCulla, DDS, dentist
Topic:  Parkinson’s and sleep
RSVP?:  No.

Palo Alto/Avenidas
Wednesday, 6/14, 2-3:30pm
Guest Speaker:  Ellen Corman, manager, Farewell to Falls, Stanford Health Care
Topic:  Fall prevention in Parkinson’s
RSVP?:  No.

Sacramento/Arden Arcade
Thursday, 6/15, 10am-noon
Guest Speaker:  Lin Zhang, MD, PhD, movement disorder specialist, UC
Davis, Sacramento
Topic:  PD – more than motor symptoms
RSVP?:  No.

Walnut Creek (Mt. Diablo)
Saturday, 6/17, 9am-noon  (speaker 10:45am-11:45am)
Guest Speaker:  Nijee Luthra, MD, PhD, movement disorders fellow, UCSF
Topic:  Advances in treatment of Parkinson’s
RSVP?:  No.

Lincoln
Tuesday, 6/20, 10-11am
Guest Speaker:  Millie Nunez, PD cycling instructor, Sun City Lincoln Hills
Topic:  Nutrition and forced exercise
RSVP?:  No.

Auburn
Tuesday, 6/20, 1:30-3pm
Guest Speaker:  Stephanie Fiola, RN, AbbVie Pharmaceuticals
Topic:  Discovering Duopa – carbidopa/levodopa eternal suspension
RSVP?:  No.

Modesto
Wednesday, 6/21, 1:30-3:30pm
Guest Speaker:  Carlos Becerra, personal trainer, Alpha Fitness
RSVP?:  No.

Auburn (special bonus meeting at same location as regular meeting)
Thursday, 6/22, 6-7:30pm
Guest Speaker:  Robert Ghelfi, MD, Northern California Surgical Group, Redding
Topic:  Stem cell therapy for PD
RSVP?:  No.

Mill Valley (Marin County)
Friday, 6/23, 1-3pm  (guest speaker 1-2pm)
Guest Speaker:  Sue Weiss, RD, dietitian, Kaiser San Rafael
Topic:  Nutrition and Parkinson’s
RSVP?:  No.

Fremont
Monday, 6/26, 7-9:30pm
Guest Speaker:  Han Lee, MD, movement disorder specialist, Kaiser San Leandro
RSVP?:  No.

Letter to newly-diagnosed MSAers in “MSA in South Africa with Sonja”

There was a lady named Sonja with multiple system atrophy in South Africa.  She had a blog aptly called “MSA in South Africa with Sonja.”

Many of her blog posts contain at least one scripture from the Bible. Copied below is her blog post from four years ago, addressed to the “newly diagnosed.”

The other blog posts with the label “My MSA Story (from 1st Symptoms to Diagnosis)” are all worth reading.  They are beautifully-written.

According to the home page, Sonja died in March 2017.

Robin

—————————–

msainsouthafricawithsonja.blogspot.co.za/2013/08/dear-newly-diagnosed-patient.html?m=1

Tuesday, August 6, 2013
DEAR NEWLY DIAGNOSED PATIENT
MSA in South Africa with Sonja

Although it is now almost 3 years since I’ve been diagnosed (6 Sept 2010), memories of that traumatic time flood back easily to make me relive that devastating period in my life.  Not only the patients, but also their families and friends are hit hard when receiving a diagnosis of an incurable, terminal disease.

Like many of you, the day I was diagnosed was the first time we had ever heard of this disease called Multiple System Atrophy (MSA). Back at home we looked it up on Google. I glanced only very briefly at the symptoms listed, and quickly closed the lap top. That was too much information too soon. Some people can take the whole bitter pill in one go, but I was far from ready to face all those symptoms.

My mind was racing with thoughts and questions: Will we be able to cope with this? How fast will all of this happen? Why did this happen to me? Did I do anything wrong? What do I do now? Where do we start? The only prayer I could manage was the desperate cry; “Oh God, please help me!”

The neurologist said we would need all the help we could possibly get and referred us to a psychologist.  Faced with the diagnosis of a dread disease that would require drastic changes in our lifestyle, we went through many emotions and all the different stages of grief.  This can, and did put strain on relationship. My husband’s patience was tested to its limits during my ‘angry stage’ and he often had to bear brunt of my frustrations.  He’s still here supporting me.  Bless him!

My emotions roller coasted from sadness, extreme guilt, and fear and uncertainty of what the future held.   Grief is a natural response to loss, and I was going to have to face many losses in the future.

On the psychologist recommendation I started writing down my feelings and experiences.  With my friend Karin’s help and encouragement I started my first blog post in my mother tongue Afrikaans, ‘Sonja se Griffels’, wondering whoever would want to read my first humble posts.  Writing however did help me to see my problems in perspective.

By the time of the final diagnosis I was fortunately past the first stage of denial.  This however didn’t mean that everybody in my life was past denial.  Some took longer to get past denial and others never did.  They were consequently unable to deal with me and the disease and were left behind.  Perhaps facing my physical frailty reminded them too much that, in the blink on an eye, it could happen to them.

At first I isolated myself from other patients, fearing that those in a more advanced stage would only depress me further.  Grieving has no time limit, it’s a very personal experience, and there is no ‘right’ way to do it.  It took me the best part of the first year to digest the diagnosis, the changes taking place in my body, and what I would have to face in the future.  This is an ongoing process as the disease progresses.

Before all this happened, I was fit and healthy.  When the first symptoms presented, I tried to fight it by becoming fitter and living healthier.  I bought organic foods from farmer’s markets, distilled our water, and juiced vegetables and fruits.  I still believe in, and reap the benefits of a healthy lifestyle, but going to extremes didn’t stop the disease from progressing.  I therefore concluded that life is best lived balanced.

Many well meaning friends have over the years suggested many therapies and ‘cures’.  If I had to try them all out, we’d be bankrupt.  I have come up with the following question for such suggestions; has the product/therapy been subjected to double blind clinical tests specifically for MSA?  I have yet to receive a positive answer.  There are currently dedicated researchers trying to solve the mysteries of MSA, but this is a process that takes time.  You’ll have to decide if there is anything out there that seems worthwhile to try.

I found the love and support of my family and friends, and avoiding stress as far as possible, to be the best medicines.  The support of my kind, and always available neurologist, who regularly attends conventions on movement diseases, has gone a long way in keeping me satisfied that I’m receiving the best care currently available.

Sometime before the diagnosis the doctor prescribed an anti-depressant, which I still take.  Some may not agree with this – I however don’t believe in unnecessary suffering.  I also found humour and laughter helpful the ward off the blues.

Attitude can make a huge difference in how we cope with difficult situations.  I have adopted Viktor Frankl’s philosophy from his book, ‘Man’s search for Meaning’, as my own;

“Everything can be taken from a man but one thing: the last of human freedoms – to choose one’s attitude in any given set of circumstances, to choose one’s own way.”

This is my life and I had a choice how I could respond to this.  I have an incurable disease and it will only get worse, but I wasn’t going to spend to rest of my life being miserable as well.  I had plenty of people to love and to live for, and I am blessed with their love in return.

It took me the best part of the first year before I tentatively started reaching out to others with the disease.  My first step was to register on ‘Patients like Me’, where I searched for others who were more or less at the same stage as me.  I found plenty in a far worse state than me.  Their plight triggered deep feelings of compassion, and I stopped feeling sorry for my self.

Concerned, I wondered how I could help them. When I first heard projects to create awareness for MSA, I was ready and eager to put my name and face to this disease and join these campaigns.  With plenty of help from my supporting friend Karin, we organised our first local awareness event.  This added new purpose to my life.

I have since been privileged to make contact and get to know many patients and/or their families from all over the world, as well as a few locally.  From them I have not only learned a lot about the disease, but also how to cope with it.  We support each other, and they have enriched my life. Now I can not imagine my life without my compassionate MSA buddies.

Being a fiercely independent person before, accepting the help from others was very difficult initially.  I have since learnt the valuable lesson that in accepting help you give a gift to the giver.  Don’t underestimate the value this can add to the lives of others, and the new depth in friendships/relationships this can lead to.  In turn, reaching out to fellow patients in their darkest hours has added value to my life.

Living with an incurable disease has caused my life to take on a new intensity.  I love more deeply, have more compassion, value friendships deeply, laugh more joyously, take more joy in music, and appreciate the beauty of nature more.  I live in the moment, savouring every beautiful moment.  The awareness that my earthy life, like all life, is finite has made it more precious.

One can never be fully prepared for the expected diminished capacities, but I hope my mental  and spiritual preparation will soften the impact.  Concentrating on what I have left has made what I have lost easier to endure.  I now see my physical body as only a part of the real me.  This allows me to look at the disease objectively, to accept it, but not succumb to it.  Coming to acceptance is an ongoing journey, a destination to aspire to.  I now often experience the inner peace that comes with acceptance.  At times I cry a little, but it is no longer tears of desperation for I am not without hope.

Dear fellow patient, you are at a cross road in your life now. Your journey will not be the same as mine.  It will be unique to you and how you respond to this extreme challenge.  The road ahead will be rough.  Open your heart to see the beautiful flowers along this road.  They will be there.

Blessings for your journey,
Sonja

Psalm 10:17 – You, Lord, hear the desire of the afflicted; you encourage them, and you listen to their cry

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

———————————————–

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

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

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Robin