Explaining fatigue – the “spoon theory”

The November 2011 newsletter for “Patients Like Me” refers to the “spoon theory” as a way to explain fatigue to those not dealing with chronic illness.  A woman with lupus, Christine Miserandino, developed the “spoon theory” when faced with her best friend who didn’t understand how Christine could be so fatigued when she looked so good (“you don’t look sick”).

Christine posted about this on the website, butyoudontlooksick.com.  Here’s a link to the post:


The Spoon Theory
by Christine Miserandino

The post is also available in Spanish, French, and Hebrew.


Book – “Mindfulness: An Eight-Week Plan For Finding Peace In A Frantic World”

This post is about the book “Mindfulness: An Eight-Week Plan For Finding Peace In A Frantic World.”  The author of this book was interviewed on last Friday’s radio show “Science Friday,” which aired on KQED.  As part of the 22 minute interview, the book’s author, Mark Williams, guides the radio audience through a three-minute meditation.  The goal is to prove that you don’t need to dedicate a lot of time to mindfulness to be successful and see improvements.  Mark Williams talks about using mindfulness to reduce stress and ward off depression.

I’ve copied below a link to the radio program as well as a transcript.



Be Here Now: Meditation For The Body And Brain
Talk of the Nation/Science Friday
January 20, 2012

In his book Mindfulness: An Eight-Week Plan for Finding Peace in a Frantic World, Oxford University clinical psychologist Mark Williams talks about the brain and body benefits of mindfulness meditation, a cognitive behavioral therapy that can be as effective as drugs at staving off recurring bouts of depression.


[Robin’s note:  you can find the meditation at 6:46.  It ends at 10:14.]

Here’s a transcript:



Up next, mindfulness. Ever find yourself going through day stuck in autopilot mode, waking up at 7:15, wolfing down your usual hot cereal, really, without really tasting it, while you read the paper, your emails, your Facebook feed.

Then it’s off to work, sitting in traffic on the bus or train, consumed by thoughts of that electric bill – oh, I forgot to pay that; the birthday call you have to make; that confrontation you want to avoid at work today; or what you’re cooking for dinner tonight. Any of this sound familiar? Would you like, instead, to turn off those stressful thoughts of the day and just concentrate on what’s going on around you right now? Relax, enjoy the moment and worry about that stuff later.

That’s what my next guest advises, what he calls mindfulness based cognitive therapy, or mindfulness meditation, a practice, he says, can sometimes be as effective as drugs and staving off recurring bouts of depression. What’s the science behind meditation therapy and what are the connections between body and brain? Mark Williams is here to explain and he’s actually going to guide us through a mini meditation session. We wouldn’t want you to do this while you’re driving so a little bit later we’re going to do a little meditation and maybe you’ll pull off the road or listen to it later on the podcast.

Mark Williams is the author of “Mindfulness: An Eight-week Plan For Finding Peace in a Frantic World.” He’s also professor of clinical psychology at the University of Oxford in England. He joins us from BBC Radio (unintelligible) in South Hampton, England. Welcome to SCIENCE FRIDAY.

MARK WILLIAMS: Hi. Thank you very much indeed.

FLATOW: Could you explain – is there a nutshell you can explain what mindfulness is?

WILLIAMS: Well, mindfulness is a form of awareness, really, so we’re all aware sometimes that just as you’re wonderful description of getting up in the morning and as you were driving to work with all these things going through your head, we also know that sometimes we can naturally switch that off sometimes if we take the time to take a walk with a youngster, you know, three or four-year-old, and they’re going very slowly along the road and they’re looking at things.

And sometimes you just have this capacity to slow down at their pace to see what they’re seeing as if through their eyes and to see little tiny details of life as if for the first time. So we know rushing around, but we also know how to slow down sometimes. It’s just that slowing down is actually very difficult to do.

FLATOW: Yeah, especially in this age. Our number is 1-800-989-8255. You can call us to talk about mindfulness. Maybe you practice it yourself. You can go to our Twitter, tweet us at scifri. Is this an especially challenging time with all the distractions from our cell phones and tablets and things like that?

WILLIAMS: There’s no doubt that we have always lots of new challenges. Now, whether cell phones and emails and stuff, which, of course, most of us find get us down from time to time, whether that’s something which is a passing phase in terms of perhaps the new generation coming up will learn how to cope with that better than we who’ve been around a while without it and then find it very overwhelming.

But certainly the 24 hour, seven days a week connectivity, as my colleague John (unintelligible) UMass Medical Center has pointed out, that sense of connectivity means that we have to take special measures to know how to slow down and how to take a brain break, if you like.

FLATOW: Yeah. We’re going to talk about those special methods for slowing down and taking a brain break. We’re going to try and take one right here on SCIENCE FRIDAY, after the break when we come back and have Mark Williams give us a little demonstration of how to practice mindfulness. Our number is 1-800 – this is something, Michael – 1-800-989-8255. 1-800-989-8255 is our number.

Also, you can tweet us at scifri, @S-C-I-F-R-I, and we’ll try a little mindfulness during the break. Stay with us. We’ll be right back.


FLATOW: You’re listening to SCIENCE FRIDAY. I’m Ira Flatow. We’re talking with Mark Williams, author of “Mindfulness: An Eight-week Plan For Finding Peace in a Frantic World.” Our number is 1-800-989-8255. You can tweet us or on Facebook, go to our Facebook site at scifri and tell us, do you meditate, why you do it, what do you get out of it. You can tweet us or leave us a little note there in our SCIENCE FRIDAY Facebook page.

Mark, who is this book for? Is it for people who suffer bouts of depression? Is it for people who – is it for everyone? Is it to teach you how to focus on what you want to focus on instead of all those other things?

WILLIAMS: Absolutely. If you start with that last question, most of us find that our attention is often hijacked by our current concerns so our attention just wanders all over the place and it’s very difficult to focus. So one of the first things you learn in mindfulness meditation is how to just settle the mind, how to focus, not to clear the mind. So it’s not the idea that you try to switch off all these thoughts going through, but that you see them passing through the mind like clouds in the sky.

And that already gives you a greater sense of balance and control in your life. And the reason why it’s relevant for everybody and not just people who get depressed is because both getting caught up in the constant spin of rushing around in a frantic world needs some addressing for many, many of us, most of us indeed. But also, we find that exactly the same strategies, the same skills we find in our research actually reduces the risk of depression.

So those how would get depressed in life many times, especially those with three or more previous depressions, it halves the risk of depression coming back.

FLATOW: So this is actually measurable, the effects.

WILLIAMS: Indeed, indeed. So there would be now six trials around the world starting off with the trial at (unintelligible) in Toronto and (unintelligible) in Cambridge and I did now 10 years ago. And that was the first trial to establish that eight weeks of this training could reduce depression. And we measured it both with questionnaires, but also with very careful interviews based on the American Psychiatric Association’s interview to diagnose depression. And the interviewers were blind.

They didn’t know whether people had had the meditation or not, so they couldn’t, as it were, make up the results to try to make the results better. And they found this really striking reduction in the risk of future depression.

FLATOW: Mark, can you give us a little taste of the sort of meditation you teach in the book, a little session here?

WILLIAMS: Yeah. So here’s a two or three minute meditation that people can try out. As you quite rightly said, it’s not wise to do it if you’re in your car and you’re doing lots of things that need your full attention. But if you can, you can become aware of your posture and just if you’re in a sitting position, you might want to just sit up straight so you’ve got a straight spine. But not stiff, not sort of like a sergeant major.

Just with the back straight, the head balanced, the shoulders can be quite relaxed and dropped. And even this sense of changing your posture already signals your intentions to step out of autopilot. And then, there are three steps now that people can try for themselves. The first is just to notice what’s going on in mind and body right now. So in the silence that comes up, just notice any thoughts that are around, any feelings or emotions there may be, any body sensations that are around.

Notice any tendency we have to want to change what we discover and seeing if it’s possible to simply allow it to be just as it is, just as it already is. And then, moving to step two of this short meditation, to gather your attention, to let all that fade into the background, gather the attention and place it lightly on the breath. So just noticing the sensations of the breath moving in and out of the body, and it may be convenient just to focus on the sensations down in the abdomen.

You can put your hand on the abdomen, if you like, and just notice the rising of the in breath and the falling away of the out breath. And just paying attention as best you can to that sensation of breathing in and breathing out. Not trying to control the breath in any way, simply allowing the breath to breathe you. And if the mind wanders at all, just notice where it went, and very gently escort it back to the breath, the sensations of in or out breath.

And now, taking step three of this short meditation and expanding attention to the body as a whole, sitting here. So simply noticing the whole body, all the sensations in the body from the surface of the skin and right deep inside as if the whole body was breathing now and allowing the sensations in the body to be just as you find them. A sense of coming home to the body. And then, when you’re ready, beginning to move fingers and toes, opening your eyes, if they’ve been closed, and taking in wherever you are, all of your surroundings, and allowing thee meditation to pass and coming back to this moment.

So that’s it, Ira.

FLATOW: That’s nice. Is this something that you have distilled from other meditation techniques or something you’ve…

WILLIAMS: Yeah. I mean, right in the beginning of our research in the beginning of the 1990s, we were very, very helped by a tremendous breakthrough that had been made by John Cabbot Zen(ph) and his colleagues at the UMass medical center in Wooster. And he developed mindfulness based stress reduction for chronic pain and people whose illness was caused by stress or who were stressed by their illness. And he developed an eight-week program in which he’d taken some of the essence of these centuries old – I like to call them spiritual exercises.

They exist in all religions and they exist in secular context as well. And he’d put them in the heart of a general hospital for chronic pain and he generously allowed us to use that as a format for applying mindfulness to our problem that we had as psychologists, which was the gradual realization that had come to the fore at the last part of the 20th century that depression was getting more and more common and recurrence was very, very in the minds of clinicians, because people were getting depressed earlier in life so they’re having a whole lifetime where they were at risk of a new episode of depression.

So the emphasis changed from treating depression to preventing depression. And so we distilled from John Cabbot(ph) in using many of the meditations he used. And the three minute breathing space, which is what we’ve just been through, was a distillation even further down so that people could have a mediation which was very portable, that they could take around and do it any time of day whenever they felt they needed to gather themselves.

And you notice the very first step of the breathing space is not actually going to the breath at all, but just checking in with what’s the weather pattern like in your mind and body, a sense of – what is this? What’s arising from me right now? And that itself is a huge gesture of openness to yourself, of friendliness towards yourself, and for people who are depressed or frantic all the time, we’re not very much friends with ourselves. You know, we tend to beat ourselves up all the time.

FLATOW: 1-800-989-8255. Let’s go to the phones and see some questions we’ve gotten. Robin in Brumfield, Colorado. Hi, Robin.


FLATOW: Hi, there.

WILLIAMS: Hi, Robin.

ROBIN: Am I on the air?

FLATOW: You certainly are. I know you probably put yourself very much at ease at that mindful session we just had.

ROBIN: Well, I can tell you firsthand that mindfulness works and it absolutely changed my life. I am so excited that you’re running this program. Thank you for running this program to make people more aware of mindfulness. I am in the process through my nonprofit organization to launch a program for children, teaching children mindfulness in the schools. And it’s such an amazing thing for kids. And I’m doing all this research to that, how it’s helping children with impulse control and more focused and assured in their ability to just help them to redirect their thoughts and be more clear.

And when you clear away the stuff, it’s a lot easier for them to do that and take in and retain information.

WILLIAMS: Absolutely.

ROBIN: So it’s really exciting.

WILLIAMS: Yeah. Thanks, Robin. Absolutely. We’ve got some schools program over in the United Kingdom as well and it’s extraordinary how children get it so quickly. Do you find that?

FLATOW: Oh, we lost her. I think she…


FLATOW: …she…

WILLIAMS: She’s gone.

FLATOW: But you have experience with kids and…

WILLIAMS: Yeah. We mostly, in our Oxford mindfulness center deal with adults, 18 to – towards older age adults, but we support various other groups that are looking at children, and we also do, even earlier than that, for mindfulness-based childbirth and parenting to prepare for a new baby based on the Californian work going on by Nancy Bardacke, a nurse midwife in California who’s developed childbirth and parenting programs with Mindfulness.

But the school’s work that Robin has alluded to: Goldie Hawn’s doing a lot of work with her Mind Up program in the States. That’s also come into the U.K. And I mean, the whole idea of brain breaks, for example, is from the Goldie Hawn Mind Up program, where she just – is very much a fact about the way in which kids are able to take these short breaks, and it really helps them focus their attention.

FLATOW: Talking with Mark Williams, author of “Mindfulness: An Eight-Week Plan For Finding Peace In A Frantic World.” Can people get frustrated trying to do this correctly during your instructions?

WILLIAMS: Oh, absolutely. And in fact, the frustration is a real good opportunity during meditation to notice all the adverse sort of little reactions that happened, like I noticed you say to doing it correctly. And there’s a great emphasis in our world – isn’t there – on making sure you do things well.

FLATOW: Exactly.

WILLIAMS: And, you know, we don’t ever heed that wise advice that says if a thing is worth doing, it’s worth doing badly. And I think in one sense, with meditation, the sense of having the intention to be – to give yourself a little break, to be with yourself as you are, that’s already enormous. It’s an enormous act of generosity towards yourself. And then, you can watch all these thoughts coming up like, am I doing it correctly, or maybe I’ve done it wrong. I’m not trying hard enough.

Oh, I went to sleep. Oh, my mind wandered. And that’s exactly the stuff of meditation. Meditation is not sitting blissfully at the top of the mountain with a mind clear. It’s actually noticing all the stuff that we don’t normally notice going through our mind, and then learning to relate differently to all of the stuff. We notice that sense of failure. We notice the sense of frustration, and we notice the sense of I must always get things right or it means I’m a bad person.

We notice that and then gradually, sort of, step back a little, not in an avoidant way, but see it like standing behind the waterfall, seeing its force but not getting dragged down by it.

FLATOW: In your book, “Mindfulness,” one of the things you recommend is being more spontaneous. Tell us about that.

WILLIAMS: Well, one of the things we ask people to do, week by week, is not just to meditate, but do things in their daily life which just, sort of, shake up the habits a bit. So, for example, we suggest just sitting in a different sort of different chair at meetings, occasionally and – or at home, just to get that different perspective. Or maybe doing, sort of, going to a movie theater without planning – with a friend – perhaps without planning beforehand what you’re going to see. So you just turn up at seven in the evening or eight in the evening, and you just watch what’s there, just choose when you get there.

Now, most movie theaters often have a big choice, so it’s not a disaster to do that. But there is a, sort of, sense of spontaneity, a sense of reclaiming the life that you’ve probably, you know, lost when you moved out – teenager or early 20s. Many of us are very cautious. We want to plan our times to the last second, and that means not going to see anything that we didn’t plan beforehand and know what it was. So that sense of just shaking up and being a little more spontaneous can help reclaim your life a bit more.

FLATOW: But if I – the idea of living for the moment, I mean being – actually being in the moment that you’re living in, a very interesting and worthwhile pursuit. I’m Ira Flatow. This is SCIENCE FRIDAY from NPR. Talking with Mark Williams, author of “Mindfulness: An Eight-Week Plan For Finding Peace In A Frantic World.” To follow up on that thought, just to be able to sit there and say this is the moment, and I’m going to enjoy this moment because I can’t control what’s going to happen in the future…

WILLIAMS: Exactly.

FLATOW: …but I can control what’s happening right now.

WILLIAMS: That’s right. That the only time that that we really make our choices is in the present moment. And it doesn’t mean that you have to suspend all your planning, sometimes you have to plan for the future. But most of us are pre-living the future. We’re not really planning the future now. We’re just pre-living it and all the worries and things that might go wrong. And we’re reliving the past. So, you know, sometimes, we have to remember what happened in the past.

And – but can we remember knowing that we’re remembering? Can we plan knowing that we’re planning? And that brings the remembering and the planning into the present moment. And the science, the neuroscience is really interesting. The brain changes when you do that in really interesting ways.

FLATOW: In what way – can you describe that for us?

WILLIAMS: Well, there are a number of things. One of the things that my colleague David Creswell, in UCLA, found. When he put people in a brain scanner, and he took people who are either high or low on a mindfulness scale. So if you’re low on that scale, it means that you’re rushing around all the time. You don’t taste your food. You know, you’re always listening only with one ear to what people are saying because your other ear is off doing something else – that sort of sense of rushing all the time.

So he had people that varied on that dimension, that mindfulness dimension, and he put them in a scanner and looked to see what their brains were doing. And what he found was a pretty characteristic feature of people who are always rushing around, is the part of the brain that is usually in fight-and-flight mode – is called the amygdala – was actually in a sort of chronic state of over activity. So when we rush around, we may believe that we’re rushing around to get things done or that we’re being very creative. But that is – it’s an illusion of productivity. And as far as the brain is concerned, it’s like as if we’re running away from a tiger.

FLATOW: Wow. So…

WILLIAMS: And that’s really interesting. Now, when he puts them people through an eight-week course, you’ll find that the amygdala actually settles down. It normalizes. It switches off. It – instead of running around as it were away from a tiger all the time, it addresses the reality of the situation rather than the constants or looking for threats. So that’s one very important part of neuroscience. Another is the work by Sara Lazar at Mass. General. She’s found that people that meditate for over a long period, actually have structural changes in their brain.

In very interesting parts of the brain, that are about attention, attention control and also part of the brain called the insula, which others have found even short-term changes. And we know the insula is active in empathy. And it also switches on for a lot of other things as well. But one of the critical factors here is it seems to be active in when we have an emphatic response, like feeling the feelings of other people, the insula switches on. That is changed by mindfulness meditation.

And what also other people have found – is this Toronto group, Norman Farb and his colleagues in Toronto, found that this is sort of a moving – an uncoupling of our ability to appreciate the body with thoughts about things…

FLATOW: All right…

WILLIAMS: …and we switch off the stories.

FLATOW: If you want to read the rest of what’s going on, read Mark Williams’ book “Mindfulness: An Eight-Week Plan For Finding Peace In A Frantic World.” Thank you for joining us.

WILLIAMS: Thank you, Ira.

FLATOW: We’re going to have this up on our Facebook page as a SciFri snack, the whole meditation that we went through will be up there at the end of the show. So if you missed it, you can check it out then. I’m Ira Flatow. This is SCIENCE FRIDAY from NPR.

Copyright © 2012 National Public Radio

Social Worker’s Reading List for Dementia Caregiving

Marguerite Manteau-Rao, LCSW, is a local social worker with expertise in working with dementia caregivers.  As part of her private practice work with dementia caregivers, she recommends these books:

• Dr. Allen Power, Dementia Beyond Drugs
• Richard Taylor, Alzheimer’s From the Inside Out
• Olivia Ames Hoblitzelle, Ten Thousand Joys and Ten Thousand Sorrows
• Nancy Pearce, Inside Alzheimer’s
• Christine Bryden, Dancing With Dementia
• Jon Kabat-Zinn, Full Catastrophe Living
• Rick Hanson, Buddha’s Brain

I’ve read a few of these books as well.  Richard Taylor’s book is wonderful.  He was diagnosed with early onset Alzheimer’s.

Recently, Marguerite mentioned the book “Ten Thousand Joys and Ten Thousand Sorrows” in the context of being able to find joy in disruption.  It sounds like that book in particular would be of interest to all caregivers, not just caregivers to those with dementia.


5-Minute Film about MSA

This is a very touching 5-minute film about a woman (Patricia Drapkin) with multiple system atrophy (MSA).  The film maker is the woman’s daughter.  The focus is on the symptoms of MSA.  Some of the daughter’s feelings about her mother are shared.  The film has been entered into the 2012 Neuro Film Festival:


You can reach the film maker, Paola Vermeer, at [email protected]  She is a cancer researcher at the University of South Dakota.



Suggestions for sharing the neuropathology report

Many families we help with brain donation arrangements share their loved one’s neuropathology report with us.  Thank you for doing this as it helps us learn too!  Plus we keep track of the clinical diagnosis and neuropathological diagnosis.  Often they are different.

We’d like to offer some suggestions of whom else you can share the report with besides Brain Support Network.

Most importantly, share the report with the diagnosing physician, who is probably a neurologist or psychiatrist.  Ask if that MD can talk with you and all family members by phone, drawing correlations between your family member’s clinical records and this neuropathology report.  Request that you be allowed to record the conversation.  Plan in advance for that conference call; prepare your list of questions.  Assign a family member or close friend to take notes.  Assist Brain Support Network by suggesting that physician send other families our way to make brain donation arrangements.

Share the report with any other physicians involved in your family member’s care — even primary care physicians.  This is how physicians can learn.  “Oh, that’s what someone with Lewy body dementia [or whatever the disorder is] behaves and appears!”  Again, suggest to those physicians that they send other families to Brain Support Network to make brain donation arrangements.

By the way, we think it’s important to share the report even with physicians who incorrectly diagnosed your family member.  Perhaps that’s most important as it’s a way the physician can learn.

I also requested that my father’s neuropathology report be placed in my personal medical record (with my primary care physician) as it is evidence of my family medical history.  Not everyone wants to do that.

Best wishes to your family and thank you again for the brain donation (as that helps us all),


Severe Autonomic Failure “Highly Predictive” of Autopsy-Confirmed MSA

This is an important article as it evaluates the clinical records of 29 patients with autopsy-confirmed multiple system atrophy (MSA) at Mayo Rochester.  All 29 of these patients had received “full autonomic function tests.”


The authors conclude that several factors are “highly predictive” of autopsy-confirmed MSA:

1- “presence of severe generalised autonomic failure”

2- severe adrenergic failure.  I believe this is found by testing the blood pressure and heart rate response to the Valsalva manoeuvre and headup tilt.

3- severe sudomotor failure and widespread anhidrosis in particular.  Sudomotor failure is determined by the QSART (Quantitative Sudomotor Axon Reflex Test) and the TST (Thermoregulatory Sweat Test).

4- “rapid progression of autonomic failure”


Among these 29 patients, there were four phenotypes or clinical presentations for MSA:
* MSA-P:  18 of 29
* MSA-C:  8 of 29
* PAF (Pure Autonomic Failure):  2 of 29
* Parkinson’s Disease-like:  1 of 29

Disease duration was shortest for those with the MSA-P phenotype (5 years ± 1.8 years), and longest for those with the PAF phenotype (13 years ± 4.2 years).  In between were those with the MSA-C phenotype (8 years ± 3.6 years).

We are told that one of the MSA-P cases was an “atypical” case in which a diagnosis of corticobasal degeneration (CBD) — one of the other atypical parkinsonism disorders — was considered.  Due to the “severe generalised autonomic failure,” a diagnosis of MSA-P was made.

In another “atypical” case, PSP was considered due to the patient’s “flexed posture, axial as well as appendicular rigidity, limitation of vertical gaze and hypokinetic dysarthria.”  But “given the clinical features of nocturnal stridor and severe generalised autonomic failure, the most likely diagnosis was considered to be MSA.”  (This patient was put in the MSA-P group.)

Along with lots of other excerpts, I’ve copied the stories of all of the atypical cases.  The few of you who fall into that MSA-vs-PSP-vs-CBD category will want to read those stories.  You can really appreciate the challenge for the neurologist in making the clinical diagnosis.


Some patients had brain MRIs completed.  “Cerebellar atrophy was the most common finding (10/16 patients) and the hot cross bun sign was present in two patients.”

The authors provided these two “clinical red flags” — rapid progression and early postural instability:

* “Rapid progression was characteristic, with gait impairment being present at motor onset in 75% of cases; 93% of patients progressed from motor onset to wheelchair dependency within 5 years.”

* “Another characteristic was early postural instability with mean time to first fall of 21±17 months. Retropulsion on the Pull Test was positive in 92% of patients when it was done at 21±18 months from onset of symptoms.”

Here’s what the authors said about parkinsonian symptoms:

* “A rest tremor was uncommon, being present in 10% of patients.”

* “Levodopa responsiveness was poor or poorly sustained in 80%.”

* “Symmetric motor involvement was the rule; symmetric bradykinesia in 78% and rigidity in 80%.”

I hope this is the first of many papers we see out of Mayo on clinical-pathological correlations in MSA.

The abstract is copied below.



Journal of Neurology, Neurosurgery, and Psychiatry. 2012 Jan 6. [Epub ahead of print]

Autopsy confirmed multiple system atrophy cases: Mayo experience and role of autonomic function tests.

Iodice V, Lipp A, Ahlskog JE, Sandroni P, Fealey RD, Parisi JE, Matsumoto JY, Benarroch EE, Kimpinski K, Singer W, Gehrking TL, Gehrking JA, Sletten DM, Schmeichel AM, Bower JH, Gilman S, Figueroa J, Low PA.
Neurovascular and Autonomic Medicine Unit, Imperial College London, UK.

Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder characterised by autonomic failure, manifested as orthostatic hypotension or urogenital dysfunction, with combinations of parkinsonism that is poorly responsive to levodopa, cerebellar ataxia and corticospinal dysfunction.

Published autopsy confirmed cases have provided reasonable neurological characterisation but have lacked adequate autonomic function testing.

To retrospectively evaluate if the autonomic characterisation of MSA is accurate in autopsy confirmed MSA and if consensus criteria are validated by autopsy confirmation.

29 autopsy confirmed cases of MSA evaluated at the Mayo Clinic who had undergone formalised autonomic testing, including adrenergic, sudomotor and cardiovagal functions and Thermoregulatory Sweat Test (TST), from which the Composite Autonomic Severity Score (CASS) was derived, were included in the study.

Patient characteristics: 17 men, 12 women; age of onset 57±8.1 years; disease duration to death 6.5±3.3 years; first symptom autonomic in 18, parkinsonism in seven and cerebellar in two.

Clinical phenotype at first visit was MSA-P (predominant parkinsonism) in 18, MSA-C (predominant cerebellar involvement) in eight, pure autonomic failure in two and Parkinson’s disease in one.

Clinical diagnosis at last visit was MSA for 28 cases.

Autonomic failure was severe: CASS was 7.2±2.3 (maximum 10).

TST% was 65.6±33.9% and exceeded 30% in 82% of patients. The most common pattern was global anhidrosis.

Norepinephrine was normal supine (203.6±112.7) but orthostatic increment of 33.5±23.2% was reduced.

Four clinical features (rapid progression, early postural instability, poor levodopa responsiveness and symmetric involvement) were common.

The pattern of severe and progressive generalised autonomic failure with severe adrenergic and sudomotor failure combined with the clinical phenotype is highly predictive of MSA.

PubMed ID#:  22228725  (see pubmed.gov for this abstract only)

DLB Patients Don’t Turn Their Heads to the Caregiver?

In this Japanese study, researchers gave a short cognitive test to patients with several types of dementia — 125 with Alzheimer’s Disease (AD), 4 with AD plus vascular dementia, 8 with amnestic MCI (mild cognitive impairment), 34 with dementia with Lewy bodies (DLB), 8 with progressive supranuclear palsy (PSP), and 6 with vascular dementia.

While they were giving the test, they noted the “incidence and severity” of the “head-turning sign” (HTS) — whether the patient turned his/her head to look at the caregiver for help.  They were trying to determine if this “sign” is unique to (specific to) Alzheimer’s Disease.

They concluded:

“HTS [head-turning sign] can be a clinical marker of AD and aMCI, and may represent a type of excuse behavior as well as a sign of dependency on and trust in the caregivers.”

These sorts of studies seem so hokey to me, particularly given the diagnostic accuracy of all of these dementing disorders.  (Actually, the dementing form of PSP, which is the disease my father had — autopsy-confirmed — has the highest accuracy of these dementias listed.)

You can read the full article online at no charge, if you are interested.  I’ve copied the abstract below.



www.ncbi.nlm.nih.gov/pmc/articles/PMC3246279/  –> the full article is available at no charge here

Dementia and Geriatric Cognitive Disorders Extra. 2011 Jan;1(1):310-7. Epub 2011 Oct 11.

Can the ‘head-turning sign’ be a clinical marker of Alzheimer’s disease?

Fukui T, Yamazaki T, Kinno R.
Division of Neurology, Department of Internal Medicine, Showa University Northern Yokohama Hospital, Yokohama, Japan.

To investigate the incidence and severity of the ‘head-turning sign’ (HTS), i.e. turning the head back to the caregiver(s) for help, in patients with various dementias and discuss its clinical specificity in Alzheimer’s disease (AD).

WE INVESTIGATED THE INCIDENCE AND SEVERITY OF HTS WHILE ADMINISTERING A SHORT COGNITIVE TEST (THE REVISED HASEGAWA DEMENTIA RATING SCALE: HDSR) in outpatients with AD [125 patients, including 4 with AD + vascular dementia (VaD)], 8 with amnestic mild cognitive impairment (aMCI), 34 with dementia with Lewy bodies (DLB), 8 with progressive supranuclear palsy (PSP) and 6 with VaD.

Significant differences were found among the 5 disease groups in the incidence and severity of HTS, and HDSR scores. Given the significant differences between AD and DLB in post hoc analyses, patients were dichotomized into AD-related (AD and aMCI) and AD-nonrelated (PSP, DLB and VaD) groups. Both incidence (41 vs. 17%, p = 0.002) and severity of HTS (0.80 ± 1.13 vs. 0.21 ± 0.60, p = 0.001) were significantly higher in the AD-related group, while average age and HDSR scores were comparable between both groups. AD-related disease, female gender and low HDSR score contributed significantly to the occurrence and severity of HTS.

HTS can be a clinical marker of AD and aMCI, and may represent a type of excuse behavior as well as a sign of dependency on and trust in the caregivers.

PMID: 22203823  (see pubmed.gov for this abstract only)


Pregnancy is associated with faster disease progression (MSA)

This is a case report by Washington University (St. Louis) clinicians of a 31-year-old Irish woman who lived in the midwest US with parkinsonism, initially diagnosed with Parkinson’s Disease.  She had a baby at age 35.  “Her pregnancy was complicated by severe orthostatic hypotension and motor fluctuations.”  At age 37, she had DBS (deep brain stimulation).  She died eight years after disease onset.  Upon brain autopsy, it was found she had multiple system atrophy (MSA).  The authors conclude that “pregnancy may be associated with marked disease progression.”

I don’t know if the authors considered that the DBS may’ve been associated with “marked disease progression.”  If anyone looks, let me know!

I’ve copied the abstract below.  The full article is available at no charge online.

Also, this must be one of the youngest documentated cases of MSA.



www.jmedicalcasereports.com/content/pdf/1752-1947-5-599.pdf  –> full article is available here at no charge

Journal of Medical Case Reports. 2011 Dec 30;5(1):599. [Epub ahead of print]

Pregnancy in multiple system atrophy: a case report.

Zhu L, Cairns NJ, Tabbal SD, Racette BA.

Multiple system atrophy is a late, adult-onset alpha-synucleinopathy with no data on the effect of pregnancy on the disease course. Early stage multiple system atrophy can be difficult to distinguish from Parkinson’s disease.

We describe the case of an Irish woman with parkinsonism starting at age 31, initially diagnosed as having dopa-responsive, idiopathic Parkinson’s disease, who successfully delivered a full-term child at age 35. Her pregnancy was complicated by severe orthostatic hypotension and motor fluctuations. Two years post-partum, she underwent bilateral subthalamic nuclei deep brain stimulation for intractable motor fluctuations and disabling dyskinesia. After this treatment course she experienced deterioration of motor symptoms and death eight years after disease onset. Post-mortem neuropathological examination revealed striatonigral degeneration and alpha-synuclein-positive glial cytoplasmic inclusions in brain stem nuclei, basal ganglia and white matter tracts, consistent with a neuropathological diagnosis of multiple system atrophy.

Multiple system atrophy can affect women of child-bearing age and pregnancy may be associated with marked disease progression.

PubMed ID#: 22208291  (see pubmed.gov for the abstract only)

Four Markers of Future Neurological Problems

This Italian paper notes that many people develop RBD (REM sleep behavior disorder) and, on average, 25 years later many of them develop a neurological disorder, such as Lewy body dementia (LBD), Parkinson’s Disease (PD), or multiple system atrophy (MSA).  Authors say:

“The estimated 10-year risk of neurodegenerative disease for…RBD is about 40%.” 

Obviously, it would be good to identify who is in that 40% bucket.  The authors indicate that potential markers of neurodegeneration include:

(1) marked EEG slowing on spectral analysis;
(2) decreased striatal 123I-FP-CIT binding and substantia nigra hyperechogenicity;
(3) impaired olfactory function;
(4) impaired color vision.

I was in a meeting once with a neurologist and a local support group member.  The neurologist was reviewing the late husband’s neuropathology report.  I remember the neurologist being very interested in finding the EEGs done on the husband early on as he said that a certain finding on an EEG would’ve been an indicator the husband was going to develop a neurological disorder.”

This is the first time I’ve seen “impaired color vision” as a potential marker.

I’ve copied the abstract of the medical journal article below.



Sleep Medicine. 2011 Dec;12 Suppl 2:S43-9.

Does idiopathic REM sleep behavior disorder (iRBD) really exist? What are the potential markers of neurodegeneration in iRBD?

Ferini-Strambi L.
Sleep Disorders Center, Vita-Salute San Raffaele University, Milan, Italy.

REM sleep behavior disorder (RBD) may be idiopathic or associated with other neurologic disorders. A strong association between RBD and a-synucleinopathies has recently been observed, with the parasomnia often heralding the clinical onset of the neurodegenerative disease. The idiopathic form accounts for up to 60% of the cases reported in the three largest series of patients with RBD. Some clinical follow-up studies revealed that a large proportion of these patients will eventually develop a parkinsonian syndrome or a dementia of the Lewy bodies type in the years following the RBD diagnosis. The estimated 10-year risk of neurodegenerative disease for idiopathic RBD is about 40%. Moreover, it has been reported that the median interval between RBD and subsequent neurologic syndrome is 25years. Several studies have looked at neurophysiologic and neuropsychological functions in idiopathic RBD and have found evidence of CNS dysfunction during both wakefulness and sleep in a variable proportion of these patients, challenging the concept of idiopathic RBD. Identifying subjects with a high risk of developing a neurodegenerative process may be crucial to develop early intervention strategies. Prospective studies in idiopathic RBD showed that potential markers of neurodegeneration include: (1) marked EEG slowing on spectral analysis; (2) decreased striatal 123I-FP-CIT binding and substantia nigra hyperechogenicity; (3) impaired olfactory function; (4) impaired color vision.

PubMed ID#: 22136899  (see pubmed.gov for this abstract only)


Test cannot differentiate PD and MSA early-on

This is an interesting Swedish study.  As you may know, a test that examines how well the anal sphincter works is a good test to differentiate Parkinson’s Disease from multiple system atrophy (MSA).  According to the abstract of this article, that test only differentiates PD and MSA “many years into the disease.”

In the Swedish study, they examined 148 newly-diagnosed patients — 100 definite PD, 21 probable PD, 16 MSA, 11 progressive supranuclear palsy, and 40 controls.  They gave them all an external anal sphincter electromyography (EAS-EMG) within 3 months of their first visit.  They found:  “No EAS-EMG differences were found between the patient groups, especially not between PD and MSA.”  So they concluded that this test cannot differentiate PD and MSA early in the disease course.

The abstract to the medical journal article is copied below.



Acta Neurologica Scandinavica. 2012 Jan 3.  [Epub ahead of print]

Anal sphincter electromyography in patients with newly diagnosed idiopathic parkinsonism.

Linder J, Libelius R, Nordh E, Holmberg B, Stenlund H, Forsgren L.
Department of Pharmacology and Clinical Neuroscience, Epidemiology and Public Health Sciences, Umeå University, Umeå, Sweden.

The differential diagnosis of patients with idiopathic parkinsonism is difficult, especially early in the course of the disease. External anal sphincter electromyography (EAS-EMG) has been reported to be of value in the differential diagnosis between Parkinson’s disease (PD) and multiple system atrophy (MSA). Patients with MSA are reported to have pathological EAS-EMG and patients with PD are reported to have significantly less pathological EAS-EMG results. Comparisons between patients with parkinsonian disorders have usually been made many years into the disease, and thus it is largely unknown if the results of EAS-EMG can be used to distinguish the different diagnoses in the early phase of the disease.

We investigated 148 newly diagnosed patients with idiopathic parkinsonism from a population-based incidence cohort (100 definite PD, 21 probable PD, 16 MSA, 11 progressive supranuclear palsy, and 40 controls) with EAS-EMG within 3 months of their first visit and, in the majority of patients, before start of treatment with dopaminergic drugs. The clinical diagnoses were made using established clinical diagnostic criteria after a median follow-up of 3 years.

All patient groups had more pathological EAS-EMG results than controls. No EAS-EMG differences were found between the patient groups, especially not between PD and MSA.

External anal sphincter electromyography examination cannot separate the different parkinsonian subgroups from each other in early course of the diseases.

© 2012 John Wiley & Sons A/S.

PubMed ID#: 22211900  (see pubmed.gov for this abstract only)