Monday, September 29, 2008

Complex Regional Pain Syndrome

Updated 3/2017-- photos and all links removed as many are no longer active and it was easier than checking each one.

Complex Regional Pain Syndrome (CRPS) is a multi-symptom, multi-system syndrome that remain poorly understood. It was called reflex sympathetic dystrophy (RSD) when I first learned about it. I admit I still tend to call it RSD.
Historical Review
In 1864 Silas Weir Mitchell published his findings on gunshot wounds of nerves in a now classic article (2nd ref below). Then in 1867, Mitchell called this condition causalgia from the Greek word meaning "burning pain". Mitchell described the condition well, but was unaware of the etiologic connection to the sympathetic nervous system. Over the years other authors who did make the connection of the vascular and nervous systems in this condition have suggested names such as neurovascular dystrophy, post-traumatic vasomotor disorders, sympathetic neurovascular dystrophy, post-traumatic vasospasm, postinfarctional sclero-dactyly, traumatic angiospasm, causalgic state, minor causalgia, mimo-causalgia, and Sudeck's atrophy. It was in 1967 after Richards article (3rd reference) that the term reflex sympathetic dystrophy began to be used.
In 1995, the International Association for the Study of Pain (IASP) decided that the terms complex regional pain syndrome (CRPS) type I and type II were better than the respective names reflex sympathetic dystrophy and causalgia. The term "Complex" was added to convey the reality that this condition expresses varied signs and symptoms.
What is RSD / CRPS?
It is a condition that does not follow the normal healing path after an injury to a nerve or soft tissue. Development of RSD / CRPS does not correlate to the magnitude of the initial injury. The reasons that the sympathetic nervous systems assumes an abnormal function after an injury is still not understood.
The International Association for the Study of Pain (IASP) lists the diagnostic criteria for complex regional pain syndrome I (CRPS I) (RSDS) as follows:
  1. The presence of an initiating noxious event or a cause of immobilization
  2. Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia disproportionate to the inciting event
  3. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain
  4. The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
According to the IASP, CRPS II (also known as causalgia) is diagnosed as follows:
  1. The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve
  2. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain
  3. The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
The primary difference between type I and type II is the identification of a definable nerve injury. For more information check this eMedicine article and this website (RSD Foundation). You will also find a nice video animation on the RSD Foundation site that shows how an injury might trigger RSD / CRPS.
The cornerstone in the treatment of RSD / CRPS is normal use of the affected part as much as possible. This is done through education, pain control, and physical therapy.

The RSD Foundation is a site full of information. There you will find not only the Clinical Practice Guidelines for Reflex Sympathetic Dystrophy (Third Edition): The Clinical Practice Guidelines have become the standard for diagnosis and management of RSD / CRPS, but also videos of Sympathetic Nerve Blocks. If you have an interest in RSD / CRPS, I would strongly suggest their site.
Reflex Sympathetic Dystrophy; Green's Operative Hand Surgery, 2nd Edition; Chapter 15, L. Lee Lankford, MD
Gunshot Wounds and Other Injuries; JB Lippincott, Philadelphia, 1864; Mitchell SW, Morehouse GR, Keen WW (I have not read, but this is the classic article references in Green's Text and elsewhere)
Causalgia: A Centennial Review; Arch Neurol 16:339-350, 1967; Richards, RL (have not read, but another classic article referenced in Green's text and elsewhere)
Complex Regional Pain Syndrome; eMedicine Article, April 2008; Steven Parrillo DO
Complex Regional Pain Syndrome: Comparing Adults and Adolescents; Medscape Article, 2002; Lorraine M Taylor MSN, CFNP
NINDS Complex Regional Pain Syndrome Information Page; National Institute of Neurological Disorders and Stroke Website; last updated July 31, 2008
Complex Regional Pain Syndrome (CRPS); (Dept of Pain Medicine & Palliative Care)
Medical Treatment Guidelines, Complex Regional Pain Syndrome (CRPS), formerly know as reflex sympathetic dystrophy -- Washington State Department of Labor and Industries (pdf file)
International Research Foundation for RSD / CRPS
Reflex Sympathetic Dystrophy Syndrome: Consensus Report of an Ad Hoc Committee of the American Association for Hand Surgery on the Definition of Reflex Sympathetic Dystrophy Syndrome; Plastic & Reconstructive Surgery. 87(2):371-375, February 1991; Amadio, Peter C. M.D.; Mackinnon, Susan E. M.D.; Merritt, Wyndell H. M.D.; Brody, Garry S. M.D.; Terzis, Julia K. M.D., F.R.C.S. (C), Ph.D. Modison, Wise.
Severe Reflex Sympathetic Dystrophy [Correspondence and Brief Communications]; Plastic & Reconstructive Surgery:Volume 101(1)January 1998, p 243; Giraldo, Francisco M.D., Ph.D.; Gaspar, Diego M.D.
Complex Regional Pain Syndrome or CRPS Treatment; Health & Recovery Blog; August 4, 2008


DrB said...

Very nice. Such a poorly understood syndrome, and so difficult to describe to patients.

jesdenm said...

I am afflicted with RSD/CRPS. The way I explain it is that my nerves turned on after an accident and never turned back off. It's quick and people tend to understand that more than trying to explain in more detail. Then I tell them it's called RSD/CRPS, in complete terms of course.

Great article. Nicely informative.


Anonymous said...

GREAT post, thank you!
I'll be linking to it from my blog, as I've found that a lot of people are curious about it.
One of the newer treatments that can be helpful for CRPS is mirrorbox and/or motor imagery - the main proponent of this is Lorimer Moseley - our experience is that this approach can be helpful for some people in the earlier stages, paired with graded reactivation. Johan Vlaeyen and colleagues have also used exposure therapy similar to that used for phobia.

Bianca Castafiore? said...

I am grateful for every intelligent post on the internet about CRPS. (I play the "acronym" game, although, as you noted, most people are more comfortable with the more inaccurate "RSD.") I have both Type 1 and Type 2 -- primarily 2 -- but have found that there are few who understand the distinction. Initially in my right leg and left arm, due to nerve and orthopedic injuries, when it "spread," most of the newly afflicted areas were Type 1. Now, any orthopedic insult usually results in a spread -- and since I have avascular necrosis in almost all joints, my odds for injury and spread are high. Yadda yadda! To all the doctors out there: *please* keep an open mind whenever you see severe sensitivity, pain "out of proportion," color and temperature variation, etcetera. Early diagnosis is key, is crucial. My life is quite ruined -- I wasn't diagnosed until 21 months out from my injuries (the doctors had tunnel vision due to all the orthopedic schtuff...), and now, CRPS has a firm hold in all four extremities, and even in a small area around my chin (that *freaks* me out!). Pain management is crucial, and one of the greatest gifts I was given by the medicos was recognition that the intense physical therapy (really the only hope, at this juncture) would be impossible unless my pain levels were addressed. Anyway, a huge THANK YOU for posting about CRPS. Cough... okay, and for posting about *RSD*! If you or others wish to contact those docs on the cutting edge, I would recommend Dr. Anthony Kirkpatrick, founder of the RSD / CRPS Treatment Center and Research Institute in Tampa, and Dr. Robert Schwartzman, chair of neurology at Drexel University, in Philadelphia. Both are dedicated to finding real treatment for this sucky disease and are involved in the current investigation into the ketamine "coma" protocol being researched in Germany and Mexico. (Oh, if I had the money!) I will also link your blog to my tangential list of blog favorites, unless you object. Feel free to object -- I won't be offended!

jeisea said...

Great post. You mentioned "The cornerstone in the treatment of RSD / CRPS is normal use of the affected part as much as possible." I wholly agree with this. Normal use, touch etc reinfrced the "it's ok" message. Normal movements train the brain just as mirror therapy does. Like Bianca I was two years before diagnosis and ended up with whole body problems. I use mirror therapy with success (I don't use a box. David Butler, who wrote "Explain Pain" with Dr Lorrimer Moseley notes on one of his blogs that an ordinary mirror will do.} Mirror therapy is one very effective way of retraining the brain. There are many other effective ways. If you don't mind my putting a link here Matthais Weinberger has a brilliant series of posts about mirror therapy.
The Neurotopian: Mirror Box Therapy - Part I

Gary M. Levin said...

I'm impressed with your knowledge and review about CPRS.
About five years ago my spouse fell down some steps, resulting in a wrist fracture, which healed poorly requiring several more surgeries. Shortly after the first surgery her hand and arm turned purple and black (I thought she had gangrene)...
We are now five years later. CPRS has changed her life, her psyche, and effecte our entire family. When I hear RSD and CPRS I almost go into a state of fugue. In additon to the signs and symptoms you describe there is also a spatial agnosia. It is exacerbated by the smallest touch, and emotional stress. Minimal trauma (and I do mean minimal) creates purpura,blood blisters, and at times an incredible sheen which is glossy to the point of appearing moist. I also believe there is a central nervous system component and it definitely has humoral components, which alters mood. Lidoderm patches, oral neurontin and/or Lyrica are a great help. Very challenging for the patient.

Anonymous said...

I'm an RN who has had fibromyalgia with depression and insomnia for ten years. I am waiting for an appt with my MD but think it likely that I've developed CRPS. On 12-27, I developed a severe burning pain in the area round my right medial malleolus (inner ankle bone) with mild edema and warmth to touch. I cannot tolerate it touching my other foot or the mattress at night. The more I am up on it, the more it hurts although there is still pain at rest. I have had another symptom for a number of years that no one could explain. When I raise my left arm and my right arm is at my side, my right hand becomes dusky and tingly with a minute or two. I wonder if this could be related. And in the past few months, I've had intermittent intense pain in both upper arms, unlike typical fibro pain. (My fibro has improved remarkably with guaifenesin and tramalol.) I also have Type 2 diabetes (5 years) and flat feet with a history of heel spurs and plantar fasciitis. Any thoughts on this? Thank you.

Susan, age 57

rlbates said...

Susan, I'm sorry to hear about your health issues. I'm glad you have an appointment with your own MD. Take care.

crps specialists said...

There is no cure for CRPS at the present time but early diagnosis and treatment is certainly crucial to limit the disability from the disease. Early treatment, ideally within three months of the first symptoms, often results in remission.