Friday, May 31, 2013

Movement Disorder in CRPS: Not Typical Dystonia... But What Is It?

It's on my mind again, what I've become accustomed to name "CRPS Dystonia."  It's rearing its incredibly ugly head again -- in my case, we theorize, in conjunction to a worsening of the infection in my bones.  It's just a theory, and like many theories, was devised to give us a gracious way out to a difficult conversation.

It's the standard reaction of an entrenched medical establishment to accord psychogenic causalities to that which lacks sufficiently convincing research to support another explanation.

I believe that it says a lot that I could give a royal crap whether anyone thinks my sustained, severe, hours-long sessions spent twitching and spasming, all to the tune of some very fine moaning and intermittent screams, is a psychological problem or the result of some CNS misfiring.  If a psychiatrist can find a way to stop them, all praise psychiatry!  If my neurologist manages to do something beyond shake his pretty, pretty head, and give me a painful hug, all hail that sweet guy, too!  Mostly, though, it is me, my MDVIP go-to-guy medico, and the poor slob assigned my case over at the Pain Management Dood's Place -- It's the three of us against the world of CRPS dystonia, or, as the kids say, "what-ev-er."

The fact that baclofen tends to work, if one is able to take the dose needed, is one huge argument for CNS involvement, but, hey!  Don't let decades of clinical practice hammered and honed into an unofficial "Best Practices" sway you.

Seriously, don't.

While those of us dealing with it must also deal with managing symptoms based on the blank areas of a road map, we also want the pure research to continue.  The problem seems to be the necessity of basing the scientific inquiries on what is known to those who study movement disorder.  And these disorders are weird enough to witness that one of the first things medicos want to weed out are those infamous "fakers." 

It's the Turdish Ochoa effect!

Finally, researchers are getting down to the infamous brass tacks.  Some basic understanding about weird and obsessive guarding, weird and excessive non-use has helped.  That leaves the rest of us with eyes rolling up into our skulls and one leg lost in space, while the other is making pretzels.

Here is the most recent study I've found, from the May 2013 issue of Clinical Neurophysiology.
Clinical Neurophysiology is the official journal of the International Federation of Clinical Neurophysiology, the Brazilian Society of Clinical Neurophysiology, the Czech Society of Clinical Neurophysiology, the Italian Clinical Neurophysiology Society and the International Society of Intraoperative Neurophysiology.The journal is dedicated to fostering research and disseminating information on all aspects of both normal and abnormal functioning of the nervous system. The key aim of the publication is to disseminate scholarly reports on the pathophysiology underlying diseases of the central and peripheral nervous system of human patients. Clinical trials that use neurophysiological measures to document change are encouraged, as are manuscripts reporting data on integrated neuroimaging of central nervous function including, but not limited to, functional MRI, MEG, EEG, PET and other neuroimaging modalities.
The Journal has special emphases on epilepsy and on studies of cognitive function and cognitive disorders. Motor neurone and neuromuscular diseases, vestibular disorders, motor control and somatosensory physiopathology are also covered by the Journal. Studies on animals and technical notes must have clear relevance and applicability to human disease, and studies reporting normative data for specific tests must have clear novelty. Case Reports are not generally accepted as full length submissions but may be considered as peer-reviewed Letters.

Note, please, that the article concentrates on hand postures.  I recall rolling down the hall at the orthopedic clinic where I was having a follow-up visit after reconstruction of my right elbow.  I was holding my arm in the way that caused the least amount of pain (the skin felt on fire, yadda yadda), which amounted to a sharp bend at that very elbow, with the entire arm, though, held tucked and away from potential accidental touch or jostling.  My surgeon was walking down the hall toward me and called out, "Why are you holding your arm like that?" The simple question made me aware that I was doing something that looked strange, and also made clear (to me) why I was doing it -- to avoid pain.  

I made a conscious effort to NOT hold my arm in that manner while in that doctor's presence, and then noted, on the drive home, that I'd reverted to the less painful pose.  Nutty, huh?

This is a photo of one of Dr. Anthony Kirkpatrick's patients, Ana English.  First, I so understand her
apparent mood.  Second, the signs of CRPS just leap off the page.  Third, I believe she is doing wonderfully well now following his ketamine protocol.  But yes, that is exactly the posture I had also adopted, and to which my surgeon responded so negatively.  But when it is you and your painful limb against the world?  You find the "posture" that protects you best. 

From Kirkpatrick's Clinical Practice Guidelines

I happen to be just that simple a thinker -- that "posturing" is something related purely to the attraction of positions that cause the least pain.  A nonscientific statement, which I'd make even more embarrassing by requesting a greater emphasis put on first quantifying the variables that are assessable during spasm/tics/dystonia movement disorder manifestations.  I can be such a bitch.

Electromyography can be pretty painful for CRPS patients but I hope they keep recruiting willing subjects, because that gives impressive information about what muscles/nerves are up to, and how.

Interestingly enough, I've had two divergent doctor-types make the same very self-assured statement:  "It's a sign of insufficient pain control." I couldn't get them to ruminate further, as they'd only reassert the belief and did not spout supporting research.  Again, they were going with their decades of "best clinical practice." 

 2013 May 18. pii: S1388-2457(13)00310-6. doi: 10.1016/j.clinph.2013.03.029. [Epub ahead of print]

Deficient muscle activation in patients with Complex Regional Pain Syndrome and abnormal hand postures: An electromyographic evaluation.


Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands; Research Institute MOVE, Faculty of Human Movement Sciences, VU University Amsterdam, The Netherlands.



Motor abnormalities in Complex Regional Pain Syndrome (CRPS) are common and often characterized by a restricted active range of motion (AROM) and an increased resistance to passive movements, whereby the affected body part preferably adopts an abnormal posture. The objective of the present study was to obtain a better understanding of the factors that are associated with these abnormal postures and limitations of the AROM, and to investigate whether these motor impairments reflect dystonia.


We evaluated characteristics of surface EMG of the flexor carpi radialis and extensor carpi radialis muscles during active maintenance of various flexion-extension postures of the wrist of the affected and unaffected side in 15 chronic CRPS patients, and in 15 healthy controls.


Deviant joint postures in chronic CRPS - at least in those patients with some range of active movement - were not characterized by sustained muscle contractions, and limitations of the AROM were not attributable to excessive co-contraction. Rather, the agonistic muscle and its antagonist were activated in normal proportions, albeit over a limited range.


The AROM limitations and abnormal postures that are often observed in chronic CRPS patients are not associated with excessive muscle activity and hence do not exhibit the characteristics typical of dystonia.


We hypothesize that structural alterations in skeletal muscle tissue and pain-induced adaptations of motor function may contribute to the observed motor impairments. Our findings may have important clinical implications, since commonly prescribed treatments are aimed at reducing excessive muscle contraction.
Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Thursday, May 30, 2013

Corey Dutina Of Pura Vida Bracelets Is A Good Egg

I'm a visual person, which makes my declining eyesight a bitch of a situation.  But mostly it's for finer stuff, like the details of a television show -- and when was the last time the details of a television show or a movie really made that much of a difference?  It's this online stuff that is getting difficult.

Well, this is ONE post that is not a woe-is-me.  Do you remember that old post, written in the middle of a long ago ManorFest, when Tête de Hergé suffered from a heat wave from Hell?  And our fridge and freezers died?  With all the beastial flesh for ManorFest at risk?  Oy, what a crisis!

We turned to the Queen of the Universe at Sears Customer Service, and then to her supervisor, for help.  Help, alas, was not easy to obtain due to... well, a host of dirty word concepts, the typical ones having to do with Customer Assistance. It was like a Carl Reiner and Mel Brooks' comedy routine, scripted back in the days of the "2000 Year Old Man."  At least on our end, there was a lot of laughter, as we were beyond tired and facing a night of roasting, and pit watching, and wild abandonment of mandatory kitchen safety measures, with half the population of the territory at risk.  Praise the Lord for On Site Universal Health Care Coverage & Catering.

Hey, take a moment and enjoy Reiner and Brooks again.  This is the stuff that makes me laugh.  I just hooted my way through three paragraphs, and hooting is where it's at... Then I will regale you with the tale of a true Customer Service Employee, my hero, Corey Dutina.

 Mel Brooks: The 2000 Year Old Man -- uploaded to YouTube byRogerBsDelaine

Let's see. My opening, irrefutable assertion was my essential visual orientation.  That extends beyond entertainment, believe it or not.

Okay, I'm sentimental.  Looking, simply looking at a meaningful something can save a moment teetering between disaster and absolutely fine.  Touchstones for the eye.

Add to that a tactile element, if possible, and it's a reminder to center, a centering focus, that goes with you everywhere.  That's right -- hand and arm wear.  Rings.  Bracelets.  It is difficult, with CRPS, to wear any ring, any bracelet, but I consider them Physical Therapy, like doing the dishes and the laundry, and giving Dobby the beloved belly rub.

Of course, I've fallen in love with Pura Vida Bracelets.  I've owned my own versions of their pricey textiles for years, and making them is probably pretty easy... but well, when I'm depressed, I spend money.  Just last night I ordered a new shower chair to replace the one with the screw that has been ripping a hole into my right haunch for months.  I'm snappy.  Taking care of business.  And so on.  This time, I got a stool that... swivels!  My back shall have equal time.

Right.  Bracelets!

First I purchased the lovely Deep Impact Pack,
shown on the right.  The colors.  The imagined sheen.  The sale price plus 20% off.

But what I had long wanted was the NEGU Charity Pack.
And it has been perpetually out of stock.  So I
put it on my Wish List -- who actually uses Wish Lists?  Then, suddenly, it wasn't a question of wanting it, it was a question of needing it.  Near the nadir (one can never be sure) of my depression, I was trying to keep good thoughts for Brayden and Ashley, and their families, as well as even more potent meditations for my darling Fred, for TW, for Grader Boob, for my mother and her blessèd relatives (myself, TW, and Grader Boob, excepted).  And I knew I was losing my own fight, weird as a fight to stop fighting can be, as a fight!

NEGU used to make me snicker in derision.


If you had decided to give up, wouldn't the concept of NEGU, a creation to honor the mantra of a 12 year old girl dead of a brain tumor, of course, reduce you to snickers of derision?

Probably not.

I decided I needed the damn bracelet.  Mostly as a reminder of dignity, and humor, and all of the other saving graces.  Beauty.  Silence.  Mother Earth.  My responsibilities.  That stuff.

But they were never in stock, these loops of blue and white and black, a reminder to rub against my burning skin, something to focus on when I can no longer make out the details of my miniature Rothkos -- and don't even START with me about Rothko, details, and the sizing of the stuff.

So when I was applying my discount for the very available Deep Impact bracelet, I asked about how the heck to get a NEGU charity pack.  And got an unexpected reply:

Hello Retired Educator!
I could make a NEGU pack for you if you want to order it!
Let me know :) 
Corey Dutina, The Problem Solver

And from that point on, poor Corey discovered that I attract bad luck as readily as cat hair.  We went through me not trusting him, we went through my credit card being declined, which encouraged me to not trust Corey, and then, I dunno, NEGU won out... and I woke up this morning to another missive from The Problem Solver:

I will make one available on the site. Let me know when you are ready to purchase because I am only going to put one online for you!

Well, that killed my distrust, and as soon as I signed on, up popped old Corey Dutina in my lower right hand corner, asking if he might be of assistance.  Sure as tootin', the side reported no NEGU bracelets were available, but within seconds, up popped a grand total of ONE available.  I snatched it up, as much as one can when computer shopping, and thanked Corey profusely.

It's in the mail.  

So, pay attention Pura Vida Bracelets -- your employee Corey Dutina, The Problem Solver, went above and beyond the dull patina of normal customer service and onto a higher plane of shining bright platinum.  He did well.  He's a good egg.  Give him a raise, or something else that he might want.  I mean, a strange woman, who doesn't explain herself, just wrote to say she needed, more than wanted, one of your bracelets, and he made it happen, overnight.  

I am going to give up, I really already have.  But I don't have to do it painfully, for myself or for anyone else. In fact, it is required of me to strive for the opposite -- for joy, laughter, silliness.  

I'm just struggling because I've never learned to be honest, emotionally.  With all I've "learned" about centering, about sitting with what I feel, I still am driven to inflict it -- upon myself, upon others, especially those most trying to help.  Upon you.  A cruel writer to her readers, that's not helpful.  If I ever have an idea worth noting, why should you trust me?

Today was better.  Very high pain, but I tried to react differently, as in not reacting.  I took my meds, and curled up with it.  Watched fuzzy television.  Adored Dobby and Buddy, who are, it looks like, either extremely close animal buddies, or fervent feline gay boys.  They comfort each other, which is great, but Buddy, in his exuberance, keeps injuring the older, wiser Dobby -- a very small animal.  We had to goop his eye last night, and in typical Dobby fashion, he forgave us, purring and delivering head rubs with glee within the next five minutes.  His eye is still red, but the other scars he's obtained from love's blossoming are healing.

I got a bit of work done, not much, but enough to feel less like a slimy slug.  The area formerly known as the left shoulder is hot and throbbing, clearly all a-bubble with that detestable character, the gross Frank Pus. The right hip is in competition.  I'm gonna win a medal, or a ribbon, or something.

Every moment is a new start.  I may not get anywhere, but I'll NEGU my way along.

Ah... good news.  If we can swing it, I'm going to get to meet Maranda, Brayden's Mom, and hopefully, Brayden and Mason, too. Maybe next Wednesday, as fate would have us seeking medical succor at the same time in the same area.  Ah, fate!  The latest news on Brayden is that as his broken leg heals, they'll start him on some new, stronger chemo meds.  They are still scouting out possibilities for him, but he's in uncharted territory.  If you think about it, in a NEGU frame of mind, uncharted territory is a place of limitless potential.

 Jessie Rees Foundation.-- NEGU and her JoyJars

On March 3, 2011 eleven year old Jessica Joy Rees was diagnosed with an inoperable and incurable brain tumor called DIPG.  That same month Jessie started her treatment consisting of 30 rounds of radiation and daily chemotherapy at Children’s Hospital Orange County. It was also during this time she asked her parents, “How can we help them?”.She was referring to all of the other kids fighting cancer.Jessie had a burden for her peers and decided to do something about it.  She also wanted to encourage them to with her little motto “NEGU“, which stands for “Never Ever Give Up.” This simple little thought has become “Jessie’s Wish“.Jessie dreamed of the day that her little motto would become an inspiring message of hope to every child and family.Ten months and two days later, Jessie lost her fight on January 5, 2012. On that day she also left behind a global movement of compassion that her foundation continues with the support of amazing corporations, athletes, charities, volunteers and people like you!

If you skipped the Reiner/Brooks videos up above?  That was stupid.  Go back and watch 'em.