Lack of sweating — worse in MSA-P than MSA-C but some nearly-normal

Autonomic dysfunction in multiple system atrophy (MSA) includes orthostatic hypotension, urinary dysfunction, constipation, erectile dysfunction, thermoregulation (sweating and temperature intolerance), pupil function, respiration, etc.  This email focuses on sweating in MSA.

Mayo Rochester has published a major paper on anhidrosis (lack of sweating) in 232 cases of multiple system atrophy (MSA).  Quite a few of our local MSA support group members have been seen at Mayo Rochester, and a few were seen during the time period of the study — between 2005 and 2010.

Thirty-four of the 232 patients eventually had autopsy-proven MSA. We aren’t told if any of the 34 brain donation cases had something other than MSA, so I think we can assume not.  Interestingly, some of the 34 with autopsy-proven MSA had close to normal sweat function.

Brain Support Network has helped quite a few MSA families donate a loved ones brain to Mayo Rochester but I’m not sure there were any during that five-year time period of the study.

I’m unclear why it takes Mayo Rochester seven years to publish data from 2005 to 2010.  At that rate, progress is mud-slow.

I’ve copied the abstract of the anhidrosis paper below.  The full paper is available at no charge online at the Movement Disorders Journal website:

onlinelibrary.wiley.com/doi/10.1002/mds.26864/full

(The paper does have some cool images of anhidrosis patterns in MSA.)

The researchers drew these three conclusions:

“(1) sudomotor dysfunction is almost invariably present in MSA and even more common and severe in MSA-parkinsonism than MSA-cerebellar;

(2) a preganglionic pattern of sweat loss is common in MSA; however, pre- and postganglionic abnormalities may coexist; and

(3) the increasing frequency of postganglionic sudomotor dysfunction over time suggests involvement of postganglionic fibers or sweat glands later in the disease course.”

Here are some definitions just to understand these conclusions —

* sudomotor:  describes anything that stimulates the sweat glands

* preganglionic:  something that originates in the brainstem or the spinal cord

* postganglionic:  something that runs from the ganglion elsewhere; exists outside the central nervous system

These MSA patients at Mayo Rochester were given two tests to determine if the problem (lesion) was in the preganglionic or postganglionic part of the autonomic nervous system:

* thermoregulatory sweat testing (TST), a test of autonomic function, and

* quantitative sudomotor axon reflex testing [QSART], a test of postganglionic sudomotor function

The researchers said:  “[An] area of absent sweating on TST in an area with a normal QSART response would indicate a preganglionic lesion. Conversely, a reduced or absent QSART indicates a postganglionic lesion.”

Why was this  study done?  The researchers said:  “Although hypo- or anhidrosis in MSA is well recognized, the degree, pattern, lesion site, and temporal evolution of sudomotor dysfunction in MSA has not been systematically evaluated in a large patient cohort.”

An accompanying editorial in the March 2017 Movement Disorders Journal is titled the “many faces of autonomic failure in multiple system atrophy” (MSA).   According to the editorialists, in the anhidrosis study, researchers “show abnormal thermoregulatory sweat testing in 95% of all assessed MSA patients. Remarkably, only 16% of MSA patients complained about sweating symptoms.”

The short editorial looks briefly at other techniques to assess sweat dysfunction.  See:

onlinelibrary.wiley.com/doi/10.1002/mds.26917/full

OK, I think that’s enough for most of us….

Robin

—————————-

onlinelibrary.wiley.com/doi/10.1002/mds.26864/full

Anhidrosis in multiple system atrophy involves pre- and postganglionic sudomotor dysfunction

Elizabeth A. Coon MD, Robert D. Fealey MD, David M. Sletten, Jay N. Mandrekar PhD, Eduardo E. Benarroch MD, Paola Sandroni MD, PhD, Phillip A. Low MD, Wolfgang Singer MD

Movement Disorders Journal, Vol 32, Issue 3, March 2017, pages 397-404
First published online: 10 November 2016

Abstract

Background
The objective of this study was to characterize the degree, pattern, lesion site, and temporal evolution of sudomotor dysfunction in multiple system atrophy (MSA) and to evaluate differences by parkinsonian (MSA-parkinsonism) and cerebellar (MSA-cerebellar) subtypes.

Methods
All cases of MSA evaluated at Mayo Clinic Rochester between 2005 and 2010 with postganglionic sudomotor testing and thermoregulatory sweat test were reviewed. Pattern and lesion site (preganglionic, postganglionic, or mixed) were determined based on thermoregulatory sweat test and postganglionic sudomotor testing.

Results
The majority of the 232 patients were MSA-parkinsonism (145, 63%). Initial postganglionic sudomotor testing was abnormal in 59%, whereas thermoregulatory sweat test was abnormal in 95% of all patients. MSA-parkinsonism patients were more likely to have an abnormal thermoregulatory sweat test compared with MSA-cerebellar (98% versus 90%, P = 0.006) and had a higher mean percentage of anhidrosis (57%) compared with MSA-cerebellar (48%; P = 0.033). Common anhidrosis patterns were regional (38%) and global (35%). The site of the lesion was preganglionic in 47% and mixed (preganglionic and postganglionic) in 41%. The increase in anhidrosis per year was 6.2% based on 70 repeat thermoregulatory sweat tests performed on 29 patients. The frequency of postganglionic sudomotor abnormalities increased over time.

Conclusions
Our findings suggest: (1) sudomotor dysfunction is almost invariably present in MSA and even more common and severe in MSA-parkinsonism than MSA-cerebellar; (2) a preganglionic pattern of sweat loss is common in MSA; however, pre- and postganglionic abnormalities may coexist; and (3) the increasing frequency of postganglionic sudomotor dysfunction over time suggests involvement of postganglionic fibers or sweat glands later in the disease course.

© 2016 International Parkinson and Movement Disorder Society

“More Iowans face multiple system atrophy diagnosis”

Here’s a link to a nice letter-to-the-editor of The Des Moines Register about a woman’s father with MSA:

www.desmoinesregister.com/story/opinion/readers/2017/04/12/more-iowans-face-multiple-system-atrophy-diagnosis/100332402/

More Iowans face multiple system atrophy diagnosis
Darcy Maulsby, Lake City
Letter to the Editor
6:00 p.m. CT April 12, 2017

The daughter discusses blood pressure issues, the facial mask, small handwriting, and the medication Northera.

The letter refers to a public TV story two years ago about MSA:

www.desmoinesregister.com/story/news/2015/04/07/dan-miller-rare-disease-multiple-syndrome-atrophy/25439089/

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
—————————————
“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

Mayo Rochester finally reports results from mesenchymal stem cell study in MSA

Finally Mayo Rochester has reported results from its mesenchymal stem cell study of multiple system atrophy.  Local support group member John Yanez-Pastor participated in the study.  This was a phase I/II safety and tolerability study, NOT an efficacy study.  Wolfgang Singer, MD, reported at this week’s annual meeting of the American Academy of Neurology that the 24 probable MSA patients who participated in the very small study reported no serious adverse events with the treatment.

I guess with the phase II aspect of the study, a bit about efficacy could be studied.  Dr. Singer said that the “efficacy of MSCs on slowing multiple system atrophy progression….appeared to be dependent on the dose, and, in the highest dose individuals, had a painful implantation response.”

Slowed disease progression was measured by the Unified MSA Rating Scale.  There was only one point difference between the study group and a “historical placebo group.”  I’m not sure a one point difference really leads to better quality of life.  (Sorry.)  There was no change on on any autonomic scales.

Mayo Rochester is in the late planning stages for a multicenter, double-blind, placebo-controlled phase II/III study.

There’s a four-minute video interview with Dr. Singer here:

link.videoplatform.limelight.com/media/?mediaId=689b6aec47124b42b39095f7863f1cf8&width=630&height=421&playerForm=19ca168687014a7b8cff84fa4c87d03f

And here’s a link to the full article from Clinical Neurology News:

www.mdedge.com/clinicalneurologynews/article/136483/movement-disorders/video-pilot-stem-cell-trial-multiple-system

VIDEO: Pilot stem cell trial for multiple system atrophy shows promising results
Publish date: April 25, 2017
By: Jeff Evans, Clinical Neurology News
At AAN 2017

Robin

Teacher who lost the ability to walk, talk and care for himself gives one last incredible lecture

This sweet article was published last week on the website of ITV, a news station in the UK.  It’s about Paul Norman, a former teacher with multiple system atrophy (MSA), giving “one last incredible lecture” and his son making a film about his dad.  The lecture is at the end of this article. If you go to the webpage, you can view “One Last Lecture” plus some photos of Paul Norman and his family.

Robin

www.itv.com/news/london/2017-03-15/teacher-who-lost-the-ability-to-walk-talk-and-care-for-himself-gives-one-last-incredible-lecture/

Teacher who lost the ability to walk, talk and care for himself gives one last incredible lecture
ITV REPORT
15 March 2017 at 3:31pm

Multiple system atrophy results in parts of the brain and spinal cord gradually becoming more damaged over time. For more information click here.  (http://www.nhs.uk/conditions/multiple-system-atrophy/Pages/Introduction.aspx)

A teacher who lost the ability to walk, talk and care for himself gave one last incredible lecture. Paul Norman, 58, was diagnosed with Multiple System Atrophy in 2012 forcing him to retire from his job as an English teacher.

His son Will, 22, began filming his every day life, eventually taking him back to Billericay School, Essex, where he taught English for 15 years.

Paul delivered an inspiring final assembly to a packed hall of his former students, written on his iPad and played out on a speaker.

He told his audience of his devastating struggle with his condition, even making them laugh with jokes about his “Stephen Hawking” voice.

He passed away ten months later, in April last year, before film and English student Will had finished the documentary ‘One Last Lecture’, featuring the scene.

Much to Will’s regret, his father never got to see the completed film, which

has been watched thousands of times since it was published online last month.

During the emotional lecture, he tells the students:

“I’m doing this partly to educate you about disability. People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either. But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.”

“It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me. The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.”

“And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.”

– PAUL NORMAN

And leaving them with some important life lessons, he adds:

“Teaching is really all about learning, and I have not stopped learning due to my disability. In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.”

“When I see the atrocities that people commit I wonder how far human beings can have yet to evolve. War is pointless. There are many good things about this world and most people are good.”

“They should look at the positive side of people rather than the faults. Health is more important than money and the world is still a beautiful place.”

“Love is important and making this film has certainly brought me closer to my son William. Life is a challenge and one should strive to be a better person.”

“Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.”

– PAUL NORMAN

Will started making the film in July 2014 after he visited his father from Billericay, Essex, and found he was wheelchair bound.

He choked on a piece of rice, forcing Will to perform the Heimlich manoeuvre to save his life, and he went back to his university that night and “broke down”.

University of Sussex student Will explained:

“That’s when I decided to start filming, just to capture memories and record how he is now, to hold on to the past.”

– WILL NORMAN

The pair grew close and for the first time Will saw the reality of his dad’s every day struggles. They gathered an assembly of former students and staff, and wrote the speech together.

Final year student Will added:

“It gave him and myself something that we could work on together.”

“He always liked to help me with my homework, and now we had a project to work on together.”

– WILL NORMAN

He completed filming in the summer of 2015, before Paul passed away in April last year, and he completed the movie in mid-January this year.

“One of the most depressing elements of the whole process really is dad never got to see the full finished film. That’s my fault really.”

“He was always asking me when it was going to be ready, but I was always striving for perfection, trying not to let him down with it. There is a feeling of emptiness that lots of people have seen it but he never got to see it.”

“He never got to be the movie star that he wanted to be.”

– WILL NORMAN

Here is the full text of Paul Norman’s lecture.

“Hello everyone. It’s good to see you all.

When William first suggested this to me I was a bit apprehensive because of the changes in me and looking at me is scary.

If I see people slipping away I will understand, however if I see anybody on their phones they will be confiscated.

The last time most of you saw me I was a different person. I now have Multiple System Atrophy, or MSA for short.

It affects every part of me and I mean every part, except my brain.

The worst things are not being able to talk or walk anymore and having to rely completely on other people.

So seeing all of you is very emotional. I cried enough last Christmas when I saw the wonderful video which some of you were in.

I’m doing this partly to educate you about disability.

People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either.

But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.

It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me.

The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.

And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.

I have friends and family who visit me regularly and have stood by me.

I also have a wonderful team of carers who go beyond the call of duty to help me and I can’t praise them enough.

I certainly couldn’t do that job if I was able to. They get paid little by society and they do 12 hour shifts.

Imagine 12 hours with me. It’s enough to drive anyone balmy.

I’m not saying they are balmy by the way.

Honestly, I feel like Stephen Hawking – except I have a better voice.

As you can see my sense of humour has not gone, in fact it helps me see the funny side even the moments when it is most difficult.

In fact inappropriate laughter is one feature of MSA, so I’ll laugh when it is most serious. That’s my excuse anyway.

For example I was in a church not long ago and I suddenly started laughing for no reason.

This would have been even more embarrassing had the congregation not known.

It was my uncle Bob’s funeral.

Teaching is really all about learning, and I have not stopped learning due to my disability.

In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.

When I see the atrocities that people commit I wonder how far human beings can have yet to evolve.

War is pointless.

There are many good things about this world and most people are good.

They should look at the positive side of people rather than the faults.

Health is more important than money and the world is still a beautiful place.

Love is important and making this film has certainly brought me closer to my son William.

Life is a challenge and one should strive to be a better person.

Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.

Now enough of this before I turn into John Lennon.

 

Although I can’t talk any more, I can answer you as long as the answer is yes or no.

If for example you ask if it’s nice to see you I can go like that [thumbs up].

Conversely, if you ask me if sitting in a chair all day is much fun, I will go like this [thumbs down].

So if I chat to you later I’m afraid we”ll have to play by those rules, and whatever you do, please don’t ask me two questions at once.

Finally, thank you all for coming. It means a lot to me. I’m sorry it’s a bit short, but at least it’s sweet.”

– PAUL NORMAN

“We Will Go On” Blog by Dan Brooks

My longtime friend Dan Brooks in Riverside reactivated his blog “We Will Go On” in 2016 and moved it to wewillgoon.com.  Dan’s blog has the tagline:

Parkinsonism: Hard to Diagnose.  Harder to live with. 
A blog by a patient with Parkinsonism Plus Syndrome.

In 2006, Dan was diagnosed with multiple system atrophy (MSA), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP).  As time went on, MSA became the most likely.  It seems that CBS has come back into the picture, however.

Dan recently posted about the confusion over whether he has Parkinson’s Disease, CBS, and MSA.  I’ve copied his post below.

Robin

————————-

www.wewillgoon.com/2017/03/confusion-over-parkinsons-contrasted.html

Confusion Over Parkinson’s Contrasted with CBS and MSA
by Dan Brooks
Saturday, March 4, 2017

I have quite often discussed with some of you in the family, and various friends, about the difference between Parkinson’s Disease and the Atypical Parkinsonian disorders.  I am going to give you a few points to chew on, knowing you are the best ambassadors we have for spreading the word about these rarer forms of Parkinsonism.

Parkinsonism is a condition in which signs and symptoms of Parkinson’s appear in the patient’s disease.  Even though the person may not have Parkinson’s Disease, they have a brain disorder that causes similarly appearing symptoms, including tremors, balance problems, stiffness, walking difficulty and cognitive changes.

Parkinsonism appears with Parkinson’s Plus syndromes such as Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy.  Since I was first diagnosed in 2006, the neurologist I saw for ten years thought that my condition was one of these three.  As time went on, Multiple System Atrophy became the most likely.  All three of these conditions are determined to be “probable” in life, and are confirmed after death through a brain tissue study.

Even though I have  Parkinsonism it is not Parkinson’s Disease in the simplest form because the disease process in my brain is more involved than in Parkinson’s.  I have Corticobasal Syndrome (CBS), and it is uncertain if it would be alternatively considered Multiple System Atrophy (MSA).  These have overlapping symptoms and are best described as rarer forms of Parkinson’s.

Has much changed?  Not really, except I have more clarity and certainty of the degenerative brain disease that has taken so much from our lives in the Brooks family.  We are fortunate to have this increased clarity because the neurologist I have been seeing of late is a Movement Disorder specialist, which is a doctor of Neurology who specializes in all things Parkinson’s and Parkinson’s-like.   She saw a clear indication in the results of my DaT Scan which demonstrated that I have Parkinson’s Plus, not simply Parkinson’s Disease.

Parkinson’s Plus has long been the understanding of my condition.  I wrote about this in my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER.  The confusion arises because the word “Parkinson’s” appears in both descriptions of the diseases.  Technically, they are different in that Parkinson’s Plus is a faster progressing disease and causes more disability sooner.

That is why I was unable to continue driving and had to retire at 51.  I have difficulty walking with coordination and I struggle with choking on food and liquids.  I also have digestive, urinary, heart rate, blood pressure regulation and body temperature issues.  These are not visible to friends and family so what appears to be a better day, could be a day I am having trouble with my blood pressure or having great difficulty coughing after drinking liquids.

I also have very abnormal horizontal eye movements which are caused by a loss of neurons in the area of the brain that controls eye movement.  At times I see double as a result.

I am so glad that you are interested and are trying to grow in your knowledge.  MSA is a disease I have been identified with for 10 years or more.  I have CBS, but if it were to turn out to be MSA at some point, that would be a very similar prognosis. My greatest concerns are pneumonia, breathing constriction, and urinary infections.  I do not have idiopathic Parkinson’s, but I do have a form of Parkinsonism, and much of the research being done for P.D. will have a benefit and weight heavily on the potential for discoveries that relate to PSP, MSA and CBS syndromes.  I will always identify with my fellow patients who have Parkinson’s Disease, and the support groups for Parkinson’s are virtually the only in person, brick and mortar groups we can attend anywhere near the Riverside, CA area. As always, thanks for reading! — Patient-Online

Supranuclear gaze palsy occurs in more than just PSP

“Supranuclear gaze palsy” (SGP) refers to impairment of horizontal gaze and/or vertical gaze.  This symptom denotes “dysfunction in the connections responsible for conducting voluntary gaze commands to the brainstem gaze centers.”

As many of you know, SGP is a classic clinical feature of progressive supranuclear palsy (PSP).  In fact, it is part of the diagnostic criteria for PSP.  However, this symptom is not specific to PSP and can occur in many other neurological disorders, including parkinsonian conditions.

In this Washington University (St. Louis) study, researchers examined the clinical records of 221 parkinsonian patients who had visited the movement disorders clinic and who had donated their brains for research.  [By the way, Brain Support Network has been responsible for over 650 brain donations — quite a bit more than the WashU brain bank.]

Of the 221 parkinsonian brains in their brain bank, 27 had supranuclear gaze palsy noted in the clinical records.  The confirmed diagnoses of these 27 were:
* progressive supranuclear palsy (9),
* Parkinson’s Disease (10),
* multiple system atrophy (2),
* corticobasal degeneration (2),
* Creutzfeld-Jakob Disease (1), and
* Huntington Disease (1).

The researchers also looked at the 14 brains donated of those with PSP in their brain bank.  Nine of the 14 had clinical evidence of SGP but five did not.

Curiously, their brain bank doesn’t have many dementia with Lewy bodies (DLB) cases because their brain bank has a bias towards movement disorders rather than dementia.

This paragraph about MSA is interesting:

“In a study of oculomotor function in MSA, Anderson and colleagues suggest that the presence of clinically slow saccades, or moderate-to-severe gaze restriction, implies a diagnosis other than MSA. In contrast, our data indicate that SGP can be seen in patients who have subsequent autopsy-confirmation of MSA at a frequency similar to that seen in PD. Cognitive impairment is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. However, some patients with pathologically confirmed MSA have been reported to have dementia. Cykowski and colleagues have reported that the presence of Lewy body-like inclusions in neocortex in MSA, but not hippocampal alpha-synuclein pathology, was associated with cognitive impairment. We suggest that the association of SGP with MSA in some individuals provides further evidence for cortical pathology.”

The authors point out that other studies show that 90% of those with CBD develop SGP.

SGP is also reported in other disorders such as spinocerebellar degeneration, amyotrophic lateral sclerosis, Whipple disease, and Niemann-Pick disease type C.

I’ve copied the abstract below.

Robin

———————–

Parkinsonism Relat Disord. 2017 Feb 24. [Epub ahead of print]

Pathologic correlates of supranuclear gaze palsy with parkinsonism.
Martin WR, Hartlein J, Racette BA, Cairns N, Perlmutter JS.

Abstract
INTRODUCTION:
Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic.

METHODS:
We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St. Louis between 1996 and 2015. All patients with parkinsonism from this population who had subsequent autopsy confirmation of diagnosis underwent further analysis.

RESULTS:
221 unique parkinsonian patients had autopsy-proven diagnoses, 27 of whom had SGP documented at some point during their illness. Major diagnoses associated with SGP were: PSP (9 patients), Parkinson disease (PD) (10 patients), multiple system atrophy (2 patients), corticobasal degeneration (2 patients), Creutzfeld-Jakob disease (1 patient) and Huntington disease (1 patient). In none of the diagnostic groups was the age of onset or disease duration significantly different between cases with SGP and those without SGP. In the PD patients, the UPDRS motor score differed significantly between groups (p = 0.01) with the PD/SGP patients having greater motor deficit than those without SGP.

CONCLUSION:
Although a common feature of PSP, SGP is not diagnostic for this condition and can be associated with other neurodegenerative causes of parkinsonism including PD.

Copyright © 2017 Elsevier Ltd. All rights reserved.

PMID: 28256434  (see pubmed.gov for this abstract only)