From Case Reports to Disinhibition: CRPS Odds and Ends

It's been a day of music and a kind of leisure.  It seems appropriate to top the hours off with a bit of light housekeeping:  Here are a few of the research article abstracts that are piling up around here. [I would say that the waste of redundant hard copies gather dust in the corners... but I can't fool you.  You know that Computer Turrets don't have corners!]

There is nothing earth-shattering, just the train-train quotidien of progress.  So long as we have that dull, repetitive noise as a lazy day soundtrack, we're okay.

For today's lazy day, that's fine.  Come tomorrow?  Maybe we can step it up a bit, eh, you scientist types?

Tease a little more information from those short fibers (Destined for Ganglia!)!

Add the Ketamine to the Propofol, whaddaya get?  Ketofol!

[Will my insurance cover Deep Brain Stimulation, do you think?  The At Home version?  I've had about enough of this tw-tw-twitching... Shouldn't be that hard to sink a few carelessly modified TENS leads into the old gray matter, then hook those babies to the ramped-up three-phase step-down transformers that Fred mounted between the two utility poles in back of the barn...  Don't worry, we'll carefully document our results and protocols.  The ABSTRACT ought to be ready to go by the weekend, pending publication in the October DIY CRPS Revue.]

Efficacy of Outpatient Ketamine Infusions in Refractory Chronic Pain Syndromes: A 5-Year Retrospective Analysis

Patil, S. and Anitescu, M. 
(2011), Pain Medicine. doi: 10.1111/j.1526-4637.2011.01241.x


Objective.  We evaluated whether outpatient intravenous ketamine infusions were satisfactory for pain relief in patients suffering from various chronic intractable pain syndromes.
Design.  Retrospective chart review.
Setting and Patients.  Following Institutional Review Board approval, we retrospectively analyzed our database for all ketamine infusions administered over 5 years from 2004 to 2009.
Outcome Measures.  Data reviewed included doses of intravenous ketamine, infusion duration, pain scores on visual analog scale (VAS) pre- and post-procedure, long-term pain relief, previous interventions, and side effects. All patients were pretreated with midazolam and ondansetron.
Results.  We identified 49 patients undergoing 369 outpatient ketamine infusions through retrospective analysis. We excluded 36 infusions because of missing data. Among our patients, 18 (37%) had a diagnosis of complex regional pain syndrome (CRPS). Of the remaining 31 (63%) patients, eight had refractory headaches and seven had severe back pain. All patients reported significant reduction in VAS score of 5.9 (standard error [SE] 0.35). For patients with CRPS, reduction in VAS score was 7.2 (SE 0.51, P < 0.001); for the others, the reduction was 5.1 (SE 0.40, P < 0.001). The difference of 2.1 between groups was statistically significant (SE 0.64, P = 0.002). In 29 patients, we recorded the duration of pain relief. Using the Bernoulli model, we found (90% confidence interval) that the probability of lasting pain relief in patients with refractory pain states was 59–85% (23–51% relief over 3 weeks).

Conclusions.  We conclude that in patients with severe refractory pain of multiple etiologies, subanesthetic ketamine infusions may improve VAS scores. In half of our patients, relief lasted for up to 3 weeks with minimal side effects.

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Complex regional pain syndrome with associated chest wall dystonia: a case report

David J Irwin and Robert J Schwartzman

Journal of Brachial Plexus and Peripheral Nerve Injury 2011, 6:6

doi:10.1186/1749-7221-6-6

Published: 26 September 2011

Patients with complex regional pain syndrome (CRPS) often suffer from an array of associated movement disorders, including dystonia of an affected limb. We present a case of a patient with long standing CRPS after a brachial plexus injury, who after displaying several features of the movement disorder previously, developed painful dystonia of chest wall musculature. Detailed neurologic examination found palpable sustained contractions of the pectoral and intercostal muscles in addition to surface allodynia. Needle electromyography of the intercostal and paraspinal muscles supported the diagnosis of dystonia. In addition, pulmonary function testing showed both restrictive and obstructive features in the absence of a clear cardiopulmonary etiology. Treatment was initiated with intrathecal baclofen and the patient had symptomatic relief and improvement of dystonia. This case illustrates a novel form of the movement disorder associated with CRPS with response to intrathecal baclofen treatment.

Provisional PDF of the complete article is available HERE.

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Complex regional pain syndrome in adults

1. Andreas Goebel

Rheumatology (2011) 50 (10):1739-1750.

doi: 10.1093/rheumatology/ker202

Complex regional pain syndrome (CRPS) is a highly painful, limb-confined condition, which arises usually after trauma. It is associated with a particularly poor quality of life, and large health-care and societal costs. The causes of CRPS remain unknown. The condition's distinct combination of abnormalities includes limb-confined inflammation and tissue hypoxia, sympathetic dysregulation, small fibre damage, serum autoantibodies, central sensitization and cortical reorganization. These features place CRPS at a crossroads of interests of several disciplines including rheumatology, pain medicine and neurology. Significant scientific and clinical advances over the past 10 years hold promise both for an improved understanding of the causes of CRPS, and for more effective treatments. This review summarizes current concepts of our understanding of CRPS in adults. Based on the results from systematic reviews, treatment approaches are discussed within the context of these concepts. The treatment of CRPS is multidisciplinary and aims to educate about the condition, sustain or restore limb function, reduce pain and provide psychological intervention. Results from recent randomized controlled trials suggest that it is possible that some patients whose condition was considered refractory in the past can now be effectively treated, but confirmatory trials are required. The review concludes with a discussion of the need for additional research.

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The 'disinhibited' brain

New findings on CRPS -- a disease characterized by severe pain

The Complex Regional Pain Syndrome (CRPS), also known as Morbus Sudeck, is characterised by "disinhibition" of various sensory and motor areas in the brain. A multidisciplinary Bochum-based research group, led by Prof. Dr. Martin Tegenthoff (Bergmannsheil Neurology Department) and Prof. Dr. Christoph Maier (Bergmannsheil Department of Pain Therapy), has now demonstrated for the first time that with unilateral CRPS excitability increases not only in the brain area processing the sense of touch of the affected hand. In addition, the brain region representing the healthy hand is simultaneously "disinhibited". The group has been performing research on and treatment of CRPS for a number of years. The researchers are reporting the new findings in the renowned journal Neurology. The study was supported by the Research Funds of the Deutsche Gesetzliche Unfallversicherung (DGUV).

Contact: Dr. Martin Tegenthoff
martin.tegenthoff@rub.de
49-234-302-6810
Ruhr-University Bochum 
The rest of this press release can be read HERE.

This is the ABSTRACT for the much-hyped research itself:

Bilateral somatosensory cortex disinhibition in complex regional pain syndrome, type 1 

Neurology September 13, 2011 vol. 77 no. 11 1096-1101

1. M. Lenz, 
2. O. Höffken, MD, 
3. P. Stude, MD, 
4. S. Lissek, PhD, 
5. P. Schwenkreis, MD, 
6. A. Reinersmann, 
7. J. Frettlöh, PhD, 
8. H. Richter, 
9. M. Tegenthoff, MD and 
10. C. Maier, MD

Objective: In a previous study, we found bilateral disinhibition in the motor cortex of patients with complex regional pain syndrome (CRPS). This finding suggests a complex dysfunction of central motor-sensory circuits. The aim of our present study was to assess possible bilateral excitability changes in the somatosensory system of patients with CRPS.

Methods: We measured paired-pulse suppression of somatosensory evoked potentials in 21 patients with unilateral CRPS I involving the hand. Eleven patients with upper limb pain of non-neuropathic origin and 21 healthy subjects served as controls. Innocuous paired-pulse stimulation of the median nerve was either performed at the affected and the unaffected hand, or at the dominant hand of healthy controls, respectively.

Results: We found a significant reduction of paired-pulse suppression in both sides of patients with CRPS, compared with control patients and healthy control subjects.

Conclusion: These findings resemble our findings in the motor system and strongly support the hypothesis of a bilateral complex impairment of central motor-sensory circuits in CRPS I.

Data Supplement: Three tables; Three Microsoft Excel documents

Bert Jansch (3 November 1943 – 5 October 2011)

Uploaded by schaaapje123 on Jul 16, 2009

From the record L.A. Turnaround by Bert Jansch 1973. With Red Rhodes on Pedal Steel. Producer Mike Nesmith.

Like a high stepping pony strutting and prancing

Ah she's so full of life

Sparkling with tiny red roses

Let there be music to please her

Let it be sunbright to light up her day

Let the moon light her night

And fill her with deep silent sleepiness

If I were a small bird so tiny

I'd hide in her hair just to be near her

To hear her sweet voice

And feel her sweet body beside me

And if I were a high lord with riches

I'd clothe her in satin from India's far highlands

I would shoe her in gold

Then invite her to sit at my table

Step out young lady a-dancing

To the sound of sweet music so gaily come singing

For your beauty so rare

Is as fresh as a sweet Sunday morning

Uploaded by ItinerateWolfMop on Mar 29, 2009

"An instrumental guitar duet between Bert Jansch & John Renbourn. It was originally on Bert Jansch's "It Don't Bother Me" and their duo album "Bert & John" which were both released in 1965. It ended up on many other albums, including a compilation of Jansch material which featured this as the title track, released in 1969. A little ironic considering that all the credits I've seen for the composition point to John Renbourn."

Uploaded by VerhipBe on May 25, 2007

Uploaded by juutalaiswhynot on May 12, 2009

"Here's another clip from Dreamweaver, the Bert Jansch documentary (2000). Reynardine is a traditional English ballad that was first released by Bert on his album Rosemary Lane (1971)."

According to Wikipedia "in versions most commonly sung and recorded today, Reynardine is a werefox who attracts beautiful women to him so that he can take them away to his castle. What fate meets them there is usually left ambiguous."

One evening as I rambled
Among the leaves so green
I overheard a young woman
Converse with Reynardine


Her hair was black, her eyes were blue
Her lips as red as wine
And he smiled to gaze upon her
Did that sly old Reynardine


She said, "Kind sir, be civil
My company forsake
For in my own opinion
I fear you are some rake"


"Oh no," he said, "no rake am I
Brought up in Venus' train
But I'm seeking for concealment
All along the lonesome plane"


"Your beauty so enticed me
I could not pass it by
So it's with my gun I'll guard you
All on the mountains high"


"And if by chance you should look for me
Perhaps you'll not me find
For I'll be in my castle
Inquire for Reynardine"


Sun and dark, she followed him
His teeth did brightly shine
And he led her up a-the mountains
Did that sly old Reynardine

A Better Life

The purported goal of this post is to recommend a blog by Australian blogger jeisea.  She has several and while they're all wonderful, crps/rsd a better life is great. 

I've known of it for several years, used to include it in my Google Reader, and added it back into the mix today.  

Somewhere along the line, I also added jeisea to the list of people who had pissed me off by getting better, and by adopting and maintaining a good attitude.  Harrumph.

In November 2006, in her second post, jeisea states her tantalizingly simple thesis for the blog:  This blog is an attempt to remind myself about what works for me to help in relieving pain and symptoms.  She might even have put it down in a bolded font, and in blood red. 

Remarkably, she did not stray.  

Remember Dr. Schwartzman of Drexel University fame?  I was so excited at the thought of being Philly bound, and getting to see one of the world's best in the field of CRPS.  It did not work out, mostly because the rarefied air around experts makes them incapable of understanding the limitations of their own impossible schedules!  That's why they have experienced gate-keepers, usually older women with cigarette-ravaged voices and an attitude.  The gate-keepers get to tell all the patients to whom the expert has offered the moon that the moon is made of cheese. 

No matter, because I was fortunate enough to find an expert of my own who had the distinct advantage of being local, and together, we ripped off the Schwartzman Protocol for subanesthetic ketamine infusions, though we had no success.  It was one of the most meaningful times of this life, finding someone willing to assist me in taking a risk.  I'll be forever grateful. 

Anyway, I sometimes forget that Dr. Schwartzman had already given me a great gift, way back in the day.  Back in the constant confusion of I-have-what?  

In a short email exchange, he ended by giving this advice, which, at the time, I just did not get, although I thought I did:  Above all else, stay in life.  "Well, that's easy enough," said my TweedleDee self to my TweedleDum one.

jeisea would have understood!

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Good Monday, Everyone --

I often feel guilty when my writing here mixes my normal nuttiness with the abnormal sort of insanity that goes with living with CRPS/RSD.  The first few years of dealing with CRPS/RSD, I was unable to think clearly.  Period.  Never mind doing any critical thinking (perhaps while relaxing in a bubble bath, sipping on a peaty single malt after an evening at the ballet?).

No, life was Hell.  I went undiagnosed... and that is very common.  It's hard to delve into treatment possibilities when you are constantly sparring with a hospital and a medical culture that has little investment in your health because of its intense need to Cover Its Ass.

I had no clue what CRPS was doing to my brain in those early, intense days.  My pain management was uninspired and ineffective.  I never slept and did not know how to compensate.  Fred was a taut wire;  Emotional explosions were the norm.

No doubt, when I turned to the Internet, I rarely took away the intent or essence that all those authors thought were clearly expressed.  Life was just too nuts, pain too severe, the whole thing too weird.

When I imagine one of My Dear Readers landing here at elle est belle la seine la seine elle est belle, trying to decipher what the heck this blog is all about, anxious only to find that kernel of information about CRPS/RSD that will help, that will explain, that will clarify, and now, right now -- I feel awful.

But since this blog is my primary source of treatment for CRPS pain and now, also for the ongoing yawn of an osteomyelitis crisis, instead of changing my tone, extending a hand, I tend to blurt out the infamous, snarling "whose blog is it, anyway?" -- all the while hoping that I don't really drive anyone away, that a few of the CRPSers out there will stay to play.

Weirdest of all, but easy enough to figure, is my reaction to the CRPSer who is one of those {shiver} extraordinary sick and suffering people.  The kind who start a research foundation in Nantucket with their left hand, write inspirational limericks with their claw of a right hand, all whilst (never while, mind, always whilst) dancing in an off-Broadway Dysmorphic Body Diversity Troupe.

I HATE them.  To be fair, I HATED them before I developed CRPS.  Back when I aspired to live forever in the snootiest of Ivory Towers, I HATED women with glossy, blunt-cut hair who exuded confidence, had read everything, who were born familiar with the right wine pairings and were genetically pre-wired to prepare, cook, and eat an artichoke.

When my circumstances changed, my HATE barely missed a beat.  But the noxious fumes and the skin-ravaging side effects of maliciousness made my aversions and antipathies evident beyond the usual confines of the blood-brain barrier.  Evil is kind of oily and has a faint yellow tint.  It poured from my leaking torn epidermis, clogged up the joint, encouraged inflammation, made thick awful pus.

HATE made me sick in a way that no illness ever could.

The "why" of it was, and is, a bore.  And, really, even though I can lay out the details of how I was wronged and victimized, I can no longer afford to stomp my feet.

I mean, come on!  Have you seen my freaking feet?  The time for the stomping of the feet is over.

At most every point along the way -- the crazy early days before diagnosis, the crazy days after diagnosis, and the crazy, interminable days after realizing no treatment was effective -- I continued to meet the extraordinary CRPSers, usually when they had taken a wrong turn off the Lone Alp Expressway after having had "two warm sakes" with their balance of cold, passive, sweet yin foods  and hot, aggressive, salty yang nutrients.  I attract the Macrobiotic.

They never seem to be impeded by lack of funds, surplus of pain, or a medical system that says (and means) "no."  They never seem embarrassed when they are asking for help.  I used to interpret their mannerisms and behaviors as products of narcissism.  It has been hard, then, to realize that my pathetic, frenetic version of Perfection is the real Ass in the Room.


The tedium of having to apologize to anyone I've mouthed off to, or about whom I've snickered, is too much to contemplate.  I don't "do" tedium, anymore, just as I have never "done" windows.  However, when life puts a member of the extraordinary CRPS crowd in my path now, I try to do better.

I've learned that I am a decent enough sort.  That seems a good place to start, and even if I never ever budge from that baseline, well... that'll be fine.

Dobby, Marmy Fluffy Butt, and Buddy the Freakishly Large Kitten

I think we have more fans of The Manor Cats than we do of any human inhabitants, which is as it should be.

Dobby remains the perennial favorite.  He was born here, after all, as the very confused runt of Marmy Fluffy Butt's first and only litter. He is imbued with a generous spirit and serves on the Marlinspike Hall Hostage Negotiation and Intervention Team.  He has few vices -- the main one being a propensity to demand over-brushing;  He lives to be groomed.  Dobby is our secret weapon in the training of the more difficult felines:  a model of decorum for his Mother, the original Wild Thing, and a living "how-to" guide for Buddy the Freakishly Large Kitten, who has been with us since March.

We were pretty much insane the day we got nine-week-old Buddy from the shelter.  Uncle Kitty Big Balls, Marmy's brother and Dobby's uncle, had suddenly taken ill and died the previous weekend.  Fred and I had invested a lot of time, money, and loving energy, first rescuing UKBB from the street, then having his hard-used body patched up.  We were glad, ultimately, to have been able to provide him with a year of comfort.  Still, it was hard to believe he had fought back from such a difficult life only to die just when he was discovering fun.  I was beginning the subanesthetic ketamine treatments for CRPS, and Fred was exhausted by all of it.

We're well versed in when not to go kitten/cat-hunting but we did it anyway -- things were just too freaking hard and diversion was needed.  Like taking care of Marlinspike Hall, the moat, the outbuildings, the errant Cistercians, the livestock (miniature, normal, and oversized), the orchards, the labyrinth -- Like all of that wasn't enough to distract us!  No, we needed a kitten.

Distract us, he did.  I managed four months of treatment, without success, and went broke in the process.  We'd each break down, tour à tour, but after, at most, a minute of weeping, giggles of "oh, you numbnut, you" would take over, as Buddy decided to attack a toe or a tassel, jump in a bag or a box, leap on Marmy Fluffy Butt's fluffy butt or sit on Dobby's head.

Buddy the Kitten has turned into a Maine Coon, that ultimate in Americana.  We grew suspicious when his growth came less in spurts and more in onslaughts, and when his tail assumed a separate identity that even his head could not understand.  He is, it seems, having a war between his front end and his rear, with that tail flitting about to truly confuse things.  One end has been known to attack the other, while Buddy the Kitten rolls his eyes wildly about.

He thinks himself tiny which is ridiculously cute but frequently irritating.  I am thinking of my bedside table, mostly.  Not entirely in control of his body, when Buddy decides to take a leisurely stroll along the invisible roundabout on my bedside table, most everything ends up on the floor or between his jaws.

He deconstructed the blinds that were custom-cut for our windows so that he can slip behind the headboard and ogle the birds, and when he outgrew the opening he'd made, he renovated.  I made lots of noise about making repairs but have not a clue how to prevent him from tunneling back in...

Anyway, the Maine Coon, for you non-cat people, doesn't follow the rules of kittenhood.  Instead of maturing in the first year of life, they continue to grow well into their third year and can keep going until age five.  He will end up being between 15 and 25 pounds.

His racoon tail has already encountered my wheelchair wheels on three occasions, and they were not happy moments.  It's not going to get better as he seems comfortable challenging the chair.  He doesn't yet make the connection that I am driving the thing, else he would be afraid, very afraid.

This week, he has been contemplating his poop production.  He does not play with it, eat it, sniff it, or do anything disgusting at all.  He just sits outside one of the three litter boxes, with his head poked through the front opening, and... contemplates.  This activity appears to be very soulful and satisfying.  He will maintain his meditative state for up to twenty minutes at a time.  I only hope his next project is as quiet and non-destructive.

Marmy remains all about Marmy.  She wants what she wants, when she wants it.  We hang together, me and Marmy.

In case you've forgotten what Buddy looked like a few months ago, here is a photo of that engaging and innocent young thing.  The tail, even then, should have been a clue.  We were clearly in denial.

PICTURE OF THE DAY

I'm trying to salvage some stuff off the little Flip video camera and realized that one of the seemingly endless cat videos from recent days contained a lovely updated snap of my legs.

So if you've not seen CRPS/RSD (Complex Regional Pain Syndrome/Reflex Sympathetic Dystrophy), this is your big chance to ogle my gams.  It's fitting that "gams" refers as much to a school of whales, porpoises, or dolphins as it does to the shapeliness of my extremities.

I'll update with a visual of my hands soon.  Can't have too much excitement going on at one time.