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Are You Sure It's Complex Regional Pain Syndrome?

Are You Sure It's Complex Regional Pain Syndrome?
Susan Denham, EdD, MS, OTR/L, CHT
October 25, 2018

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Susan: Thank you.  I am glad to see so many people logged on. What we are going to talk about today is a topic that has provided lots of confusion and frustration for me over the years. Confusion because I get referrals from lots of different physicians that say things like, "possible Complex Regional Pain Syndrome" or "possible Reflex Sympathetic Dystrophy." The second term is still used quite a bit today. The frustrating thing for me is that often it is not Complex Regional Pain Syndrome and I am not sure why they would think it is. There is a lack of education about what this condition is throughout our medical community. There is also a lot of fear associated with it. I worked with several patients recently, and this prompted my decision to put this presentation together. I hope that you find it helpful.

We are going to be primarily learning how to identify when it really is Complex Regional Pain Syndrome, and there are some pretty specific diagnostic requirements. We see this primarily in the upper limb although it can occur in the lower limb as well. We need to be part of the diagnostic process. This is not something that should happen in one visit.

If you Google Complex Regional Pain Syndrome or Reflexive Sympathetic Dystrophy, you will see some of the most horrific pictures and awful videos you have ever seen. There is one particular video on YouTube of a woman who goes into a therapy session and spends about an hour screaming and crying. It is horrifying. So when a patient has this diagnosis thrown at them, there can be a lot of fear and anxiety that comes with it. We are also going to talk about our role as occupational therapists in evaluating and treating patients with Complex Regional Pain Syndrome.

What Exactly is Complex Regional Pain Syndrome (CRPS)?

First of all, it is a chronic disorder. We typically expect that these symptoms have been going on for longer than six months, and as I tell my students, six months is kind of a guideline. I have seen patients at three months that I was 100% sure was CRPS. So it may be diagnosed earlier than that, but when we talk about chronic pain, we have to make sure that this pain is not coming from a legitimate source like from a fracture that has not been diagnosed or from a bullet that is still in the body. It seems to come from nowhere, and it is chronic. We also need to make sure that it is not due to an acute inflammatory response, and sometimes that takes a little time to rule that out.

The condition typically impacts one limb, at least initially. The arm is the most common place that we see CRPS, but it has also been reported in the leg, the foot, and even in the face. It results in delayed recovery. and there are very noticeable trophic changes that occur that we will talk about in detail. It is somewhat of a mystery, and when my patients ask what exactly causes it, the answer is we have no idea. This topic was not well-researched until very recently. If you do a literature review, you will find that there is just not a lot out there about it. We do not know why one patient can have, for example, a carpal tunnel release in one arm and do beautifully, and then have another carpal tunnel release on the other arm and develop CRPS.

CRPS- A Brief History

  • This is not a new diagnosis
  • French King Charles IX of Valois (1550-1574)
    • After an episode of phlebitis
  • 1812- “I always found him with the forearm bent and in a supine position and supported by the firm grasp of the other hand. The pain was of a ‘burning’ nature, and so violent as to cause a continual perspiration from his face”. British Surgeon-Denmark
  • 1864-“Perhaps few persons who are not physicians can realize the influence of which long continued and unendurable pain can have upon both body and mind.” Silas Weir Mitchell Civil, Neurologist treating Civil War Soldiers
  • 1940-American Doctors (James Evans) identified it-Named it RSD
  • Often described as occurring “dramatic, histrionic personalities”
  • 1994-Renamed CRPS
  • 2003-Budapest Criteria adopted

It was formerly referred to as RSD or Reflex Sympathetic Dystrophy. The name was actually changed in 1994. I did not start practicing as a therapist until 1988 so I only had about six years of calling it RSD before the term changed, but until I put this presentation together, I was still calling it RSD a lot of times. It takes a long time to get people to change terminology, but we are now using the term Complex Regional Pain Syndrome (CRPS) because we feel like that better describes what exactly it is. We do think that it has something to do with the sympathetic nervous system, and that is where RSD came from originally. However, it is much more complex than that so that is why the name changed. You will also see it in the literature that it has been called shoulder-hand syndrome, Sudeck's atrophy, causalgia, and reflex neurovascular dystrophy.

This is not a new diagnosis. I had a physician that said, "I'm not up on some of these newer diagnoses." He was an older physician. My response was, "Well it's actually been around since about the 1500s as far we know so it's not really new." If you go back and look at history, King Charles reported what we think was probably CRPS in the 1500s. We have found documentation from British surgeons in Denmark where they described a burning pain and continual and constant perspiration. There were violent reactions noted in the 1800s. In the 1860s, neurologists reported this type of symptom in Civil War soldiers. Then in 1940, we got the diagnosis of RSD. Dr. James Evans first identified it, and he described it as something that occurred in individuals with dramatic and histrionic personalities. When I first finished therapy school and started practicing, this was what was written up in a lot of the journals. That women especially, who had histrionic or dramatic personalities, were at a greater risk for this diagnosis. To be quite honest, with the first couple of patients that I saw with this diagnosis, that was the first thing that popped into my mind. "You must be a little crazy because that's why you got this disorder." We now know that that is not true, and there have been multiple studies that have looked at personality disorders in patients who have CRPS. There is no correlation between being crazy at the time that you get it and this disorder. Now I will say this, having your arm hurt 24 hours a day, seven days a week, for six months is enough to make any of us a little bit crazy. While there are some dramatic and some behavioral things that often occur with this disorder, it does not mean that they are histrionic or that they are dramatizing the symptoms that they are having. In 1994, we renamed it Complex Regional Pain Syndrome to better describe it. In 2003, the Budapest Criteria was adopted and that helps us to better diagnose CRPS. We are going to go through that criteria in just a little bit.

Common Theories of Causation

There are many theories about what causes CRPS.

  • Pain cycle out of whack because of the SNS
    • More to it than that
  • Peripheral Nerve Damage
    • Creating vascular issues
      • Over and under dilation
      • Leaking of interstitial fluids
    • Some sort of myelination problem resulting in PNS dysfunction
      • Possibly due to damage to microvessels
  • Autoimmune System dysfunction
    • More common in people with other immune issues: asthma, lupus, etc.
  • Disagreement between the PNS and CNS
    • Some sort of communication gap, inflamed by psychological factors
  • Genetics
  • Brain Change-Neuroplasticity: changes have been seen in somatosensory portions of the brain on BOTH SIDES!
  • Mental Illness
  • Eating a hot dog on a Tuesday with a full moon before the fracture?
  • In reality, NO ONE REALLY KNOWS!

Some of the more popular theories are that the pain cycle is out of whack because of something to do with the sympathetic nervous system. We know that it has to be more than just that. We do know that in some cases of peripheral nerve damage that there could be vascular issues with some leaking of interstitial fluid. It could also be a myelination issue or microvessels within the nerves that could be damaged that we do not even have the technology to see well at this point. There are other theories that it is an autoimmune issue as it occurs more commonly in individuals who have autoimmune dysfunction like asthma, lupus, or things like that. Another theory is there is a disagreement between the peripheral nervous system and central nervous system, and there is some sort of communication gap. It could be inflamed by psychological factors, and that could be where some of the theories of histrionic behavior came from. There could also be a genetic component. We believe in neuroplasticity from the standpoint of changing a patient from injury to function, and so there is also a theory that there is a brain change. That having an injury that is painful and causes lots of functional problems begins to create a negative neuroplastic change. In fact, changes have been seen in the somatosensory portions of the brain. When this happens, the theory is that it happens on both sides. This is an important theory to remember as we go through this talk because we are going to talk about some of the crazy symptoms that patients report. There are still theories about a mental illness component. Or, maybe it is because you decided to eat a hotdog on a Tuesday. The reality is that we really do not know what causes CRPS. And for every patient that we can identify and say, 'Yes that is a factor," we can identify three patients that do not have that same factor. At the end of the day, we really do not know what causes it, but we still have to deal with it. 

Types of CRPS

There are two primary types of Complex Regional Pain Syndrome. Type 1 and Type 2, and these are simply based on how the injury occurred. 

  • Type I
    • No confirmed nerve injury (could be a fracture, pin stick or bug bite).
    • Pain may not follow the Peripheral Nerve Path
  • Type 2
    • There is a confirmed injury to a specific peripheral nerve
    • Crush injury, gunshot or knife injury, lacerating fracture
    • With either type, if it follows the nerve path it is probably NOT CRPS

This is probably a good place for me to point out that with CRPS, there will always be some type of injury. There has to be. If the patient wakes up on a Monday morning, and their left arm is swollen and painful and they cannot tell you anything that happened to it, by definition it cannot be CRPS. Could they have had an injury that we might not see? Yes. They could have a bug bite, a pinprick, or something like that. That is possible, but in order to be defined as CRPS, there must be some sort of initiating injury.

Type 1

If there is an initiating injury, but there is no confirmed nerve injury, we refer to that as a Type 1 CRPS. In Type 1, the pain does not typically follow the peripheral nerve path. This makes sense because the injury is not to a specific nerve. The injury could be to a tendon, a muscle belly, connective tissue, or to the skin. We would not necessarily expect it to follow the median or ulnar-radial nerve pathway. It is usually circumferentially reported pain or pain all over. Many times, the patient has a hard time defining specifically where the pain is.

Type 2

With Type 2 Complex Regional Pain Syndrome, there is a confirmed injury and there is also a confirmed injury to very specific peripheral nerve injury like a gunshot or a stab wound. It could also be a crush injury or carpal tunnel, although not typically.

With either type of these, whether you are talking about Type 1 or Type 2, it is important to understand that the nerve path is probably not going to be followed. Unlike a carpal tunnel patient or radial nerve patient that comes in and has a definitive sensory loss to that nerve pathway, with Type 1 and Type 2, there is not going to be a specific nerve path that is followed. 

How Is It Diagnosed?

How do we diagnose CRPS?

  • Despite its long history, there is no one test to diagnosis CRPS
  • A correct diagnosis should be based on:
    • In-depth medical chart review
      • Including issues associated with 2ndary gain
      • Pt’s who fake it
    • Rule out other diseases or system issues:
      • Lyme Disease
      • Arthritis
      • There is always an incident of injury, so we must be clear that there is not something else going on.
  • MRI, X-RAY, Bone Scans- as the patient enters the 2nd stage- bone reabsorption occurs
  • Nerve and Vascular tests
  • OT  or PT evaluation (needs to be a therapist with CRPS experience)
  • Psychological Evaluation
  • Meet the requirements of the Budapest Criteria

I kind of put CRPS and autism in the same classification in that there is no one test to diagnose it, You cannot look at an x-ray or an MRI. Instead, we look at functional outcomes, and we look at how the patient progresses in their daily life. We are used to looking at multiple factors and diagnosing something. Anytime I get a diagnosis from an urgent care that says, "possible CRPS," my head explodes a little bit because this is not something that should be diagnosed on a Saturday night at midnight. A correct diagnosis should involve an in-depth medical chart review, and this includes looking at secondary gain issues. Does the patient have a reason to be faking it or not telling us the truth? Are they getting a large lawsuit out of this? I have several stories of patients who have tried to fake CRPS pretty successfully, and if we have time at the end of this, I will give you a couple of case studies on them. You also want to rule out any other diseases, particularly systemic diseases. Sometimes when a bone is broken, other things can flare up like rheumatoid arthritis flares or Lyme disease. It is important to rule out any other disease and to recognize that there is always an incident of injury. We have to be very clear that something else is not going on via MRIs, X-rays, or bone scans particularly as the patient begins to enter the second stage of suspected CRPS. At the second stage, we will be able to see bone reabsorption that occurs, and that is a definitive sign that we are dealing with CRPS. There should be nerve and vascular tests to make sure that there are not any lacerations or blockages. And, we need an OT or PT evaluation. This needs to be somebody that has experience with CRPS. In some cases, the recommendation is that there also be a psychological evaluation to rule out any of those other psychological issues that we might see. Finally, we want to make sure that the conditions meet the Budapest criteria before we assign the diagnosis.

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susan denham

Susan Denham, EdD, MS, OTR/L, CHT

Susan Denham, EdD, OTR/L, CHT, is a graduate of the University of Alabama at Birmingham and a Certified Hand Therapist. She has over 29 years of experience in hand therapy and industrial rehabilitation. She is employed by Alabama State University as the Department Chair and Professor in the Occupational Therapy program. In addition to teaching, Dr. Denham maintains a clinical practice in outpatient hand therapy and industrial rehabilitation.

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