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The Onset, Types & Stages Of CRPS

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Onset of CRPS

Complex Regional Pain Syndrome (CRPS) typically originates from an injury, which determines the type of CRPS the individual experiences. While the type usually does not impact treatment, there are cases where it may have some influence. CRPS is classified into three types:

1. Type-1

This type does not involve direct nerve injury but may result from soft tissue damage, such as blunt force impact, ligament sprains, bruises, or even minor incidents like stubbing a toe. It was previously known as Reflex Sympathetic Dystrophy (RSD) until the name change in 1994 by the International Association of Pain Study (IASP) to encompass a broader range of conditions.

2. Type-2

 In this type, there is direct nerve injury, which can occur due to surgical procedures, incisions, amputations, or lacerations. Previously referred to as Causalgia, it was renamed RSD in 1946.

3. Type-NOS

Type-NOS (Not Otherwise Specified): The IASP, also known as Orlando, convened in 1994 and merged RSD and Causalgia with the Budapest Criteria. However, this left a group of individuals who did not fit the Budapest Criteria and were previously diagnosed. The Type-NOS category was created to include these individuals and maintain their diagnosis. The Sympathetic Response System comes into play when pain receptors detect an issue. Once pain receptors perceive pain, they signal the sympathetic nervous system (SNS). The SNS, integrated with various bodily systems, regulates inflammation and triggers the well-known “fight or flight” response in dangerous situations, injuries, or stress. The SNS serves as a tool to alert our brain and focus on immediate threats, providing two choices: fight or flight. For those living with CRPS, the identification with being warriors becomes evident. They, too, face choices between fighting the condition or succumbing to its effects. The SNS maintains equilibrium by collaborating with systems like the immune, neurological, vascular, and digestive systems. It works in harmony with the hypothalamic-pituitary-adrenal (HPA) axis, the vagal nervous system (VNS), and the sensory nervous system. It also influences vasoconstriction and vasodilation. In most cases, when a healthy individual sustains an injury, the body’s balanced systems facilitate a timed healing process that typically takes 5-7 days to complete (barring any complications). However, individuals with CRPS experience a connection between the SNS and inflammation, causing them to become trapped in a cycle.
  
  • Calor (redness)
  • Swelling
  • Heat
  • Pain
  • Mototrophic (difficulty of movement)

So why do some people recover from their wounds while others do not? The reason remains unknown, but it is clear that there are issues within the nerves and the brain’s interpretation of pain, as indicated by the association with migraines.

The cycle begins with an injury to any body part connected to the nervous system. Pain receptors send signals to the sympathetic nervous system, which notifies the brain via sensory nerves about the injury. The brain, in turn, sends a signal to the sympathetic nervous system, leading to the activation of the Inflammatory Response. This response includes blood vessel spasms, increased pain, limb swelling (even in non-injured areas), and the initiation of the cycle again.


As a result, CRPS develops, introducing new symptoms unique to individuals with the condition. By understanding these processes, individuals affected by CRPS can gain insight into their condition and explore appropriate treatment options.

Types of CRPS

Reversing Complex Regional Pain Syndrome (CRPS), formally called Reflex Sympathetic Dystrophy (RSD), which is considered the most painful condition known to humankind, is possible if the disease has three different types determined by the IASP (International Association for the Study of Pain):
  1.  Type 1
  2.  Type 2
  3.  Type NOS (added 2004)

1. Type 1

CRPS Type 1 is the most commonly reported type, accounting for over 90% of diagnosed and reported CRPS cases. It requires an acute injury or a “noxious event” to the soft tissues or immobilization of a limb for a period of time, such as putting it in a cast or a straight leg brace.

Characteristics of CRPS Type 1 include:

  1.  Initiating a noxious event or cause of immobilization:
  2.  Continuing pain or allodynia, or hyperalgesia that is disproportionate to the inciting event:
  3.  Evidence, at some point, of edema, changes in skin blood flow, and abnormal sudomotor activity in the region of pain.
  4.  The diagnosis excludes the existence of any other condition that would otherwise account for the symptoms.
  5.  It does not present with a cold limb or mechanical allodynia.
  6.  It does present with Thermal Allodynia and Circumferential Pain around the entire foot or arm. Pain or pressure on the ulnar, radial, median, tibial, sural, superficial, and deep peroneal nerves will be no more or less painful than pressure anywhere else on the limb.
  7.  It may or may not present with “classic” symptoms such as pitting edema, mottled/shiny skin, or hair/nail growth.
  8.  Thermography would show temperature differences.
  9.  It responds well to sympathetic blocks but not to nerve blocks.

In the early stages, Type 1 is a disorder of the Sympathetic Nervous System, hence the name Reflex Sympathetic Dystrophy (RSD), which is a disorder of the sympathetic nerves. As the disease progresses, it becomes a disorder of the spinal cord and the wide dynamic range neurons in lamina II and V of the dorsal horn and NMDA.

Due to neuronal plasticity, meaning their ability to change over time, Type 1 is known to spread to the contralateral (opposite) limb and ipsilateral limb (a limb on the same side of the body).

  1. There are several theories about the Etiology of Type 1 (RSD).
  2.  According to Hendler (Pan Arab Journal of Neurosurgery, 2002), 71% of patients diagnosed with CRPS Type 1 have nerve entrapments, while the other 27% have both.
  3.  According to Dellon (2009), 80% of patients diagnosed with CRPS Type 1 have nerve entrapments that respond well to surgery.
  4.  Knobler suggests an auto-immune disease etiology.
  5.  Ochoa suggests a central etiology involving angry backfiring C fibers.
  6.  Roberts suggests a role for Wide Dynamic Range neurons.
  7.  Dubner suggests neuronal plasticity.
  8.  Bennet suggests hyperpathia.
  9.  Sweet suggests Ephaptic connections.

To date, there has been no proven cause of CRPS.

2. CRPS Type 2

CRPS Type 2 was previously named “Causalgia” by the IASP, and only 10% of patients that are diagnosed with CRPS, are diagnosed with this type. Type 2 requires that the nerve be directly injured which could happen due to surgery, where the nerve was severed, or due to an accident where the nerve was lacerated but not necessarily limited to the distribution of the injured nerve.  Onset can develop immediately, or be delayed by several months.  The most common nerves impacted are the median nerves in the arm or the sciatica nerves in the leg.  Allodynia and Hyperalgesia occur but may not be limited to the peripheral nerve only.  Type 2 does follow a distinct nerve distribution which makes it more responsive to nerve blocks.

  1. Initiating a noxious event or cause of immobilization
  2. Continuing pain or allodynia, or hyperalgesia and the pain is disproportionate to the inciting event but not necessarily limited to the distribution of the injured nerve.
  3. Evidence, at some point, in edema, changes in skin blood flow, and abnormal sudomotor activity in the region of pain.
  4. Type 2 does require a direct injury or damage to a nerve such as surgery and burning pain.
  5. It does follow direct line nerve distribution pain – meaning there is pain when pressing on the area over the direct nerve (i.e., peripheral, median, sciatica).
  6. It is usually present with a cold limb & presents with a difference in temps of +\- 1 degree, but this temperature variation can be much greater in some cases.
  7. It does present with Mechanical Allodynia (pain triggered by movement across the skin), and hyperalgesia.
  8. It may or may not present with “classic” pitting, edema, mottling, shiny skin, or nail/hair growth.
  9. It does respond well to nerve blocks.
  10. It does generally spread ipsilaterally (same side of the body) more frequently.

3. CRPS Type NOS Introduction

When the IASP (aka Orlando) converged for the first time in 1994 and brought together RSD and Causalgia, which were both true in their own right but didn’t tell the whole story, they created “Complex Regional Pain Syndrome” (CRPS) and broke it out by types to fit all areas: Type 1 (RSD) Type 2 (Causalgia).

In 2004 when a “new” criteria were brought into the mix, “The Budapest Criteria” met again but challenged “Orlando” (aka IASP) criteria. 

Budapest vs. Orlando

It is clear by the grayed-out section above and the minor details inside the boxes that Budapest was more stringent than the preceding Orlando criteria. These differences resulted in 15% of previously diagnosed CRPS patients losing their diagnosis up against the IASP/Orlando criteria so they created a third type – CRPS NOS (Not Otherwise Specified) which included those patients who did not fulfill the Budapest criteria but whose signs and symptoms could not be better explained by any other diagnosis.

CRPS Disease Description

The following video is by Strong Medicine and is a great video that explains the disease. We hope you find it as informative as we did.

CRPS Sub-Types

Understanding the Asymmetry of Skin Temperatures in CRPS

The presence of an asymmetry in skin temperatures is a notable symptom experienced by individuals suffering from Complex Regional Pain Syndrome (CRPS). This neurological condition affects various components of the Central, Autonomic, Sympathetic, and Enteric Nervous Systems. While there are multiple available treatments for CRPS, understanding the underlying causes of symptoms is crucial.

In CRPS, the misfiring of chemicals in the body can lead to peculiar symptoms, such as swelling even after an injury has healed or vascular issues like redness and discoloration. However, one of the most distinct symptoms reported by many CRPS sufferers is an abnormal fluctuation in skin temperature, with some experiencing hot skin, others feeling cold, and some individuals experiencing alternating changes.

Cold CRPS Sub-Type

Approximately 30% of individuals with CRPS experience cold skin associated with their condition. Thermographic imagery reveals that in these cases, the affected skin can exhibit hypothermic characteristics, with temperatures ranging from 1 to 14 degrees Celsius colder than normal skin surface temperatures.

IASP Requirements for Cold Sub-Type

The International Association for the Study of Pain (IASP) and the American Medical Association (AMA) have included skin temperature asymmetry as a diagnostic criterion for CRPS. Researchers believe that this asymmetry could result from excess vasospasm caused by hypersensitivity of sympathetic denervation due to the dropout of sympathetic efferents related to damage to small-caliber nerve fibers. The hyperexcitability of fast potassium voltage gates has also been implicated.

Presentation of Cold Sub-Type

Cold Sub-Types typically present with ice-cold skin that appears white, purple, or blue in color, often in the hyperthermia temperature range (1 to 14 degrees Celsius below normal). Previous trauma is often a precursor to this subtype. A cold sub-type of CRPS is characterized by a cold, blue extremity with less edema. Dystonia, a movement disorder, is observed exclusively in individuals with cold CRPS and may be a result of central changes at the spinal cord or brain level.

Studies have shown that patients with a history of trauma tend to have colder limbs, and the severity of trauma correlates with colder limbs. Additionally, individuals with the cold subtype of CRPS experience pain that lasts longer compared to those with the warm subtype. Patients describe the “cold” symptom as similar to frostbite.

Hot CRPS Sub-Type

For individuals experiencing “hot CRPS,” characterized by a sensation of the skin being “on fire” and actual increases in skin temperature, it is important to note that they are not alone. Approximately 70% of individuals with CRPS have Type-1 CRPS, with Thermographic imagery revealing hyperthermic temperatures ranging from 1 to 14 degrees Celsius higher than normal skin surface temperatures of the unaffected limb.

Symptoms of the hot subtype include red skin, increased sensitivity to pain (mechanical hyperalgesia), elevated temperature (hyperthermia), edema, sweating, and signs of inflammation such as neurogenic edema and erythema. Medical professionals can utilize video-thermography or thermography to test and diagnose patients accurately, as these methods provide precise measurements of skin temperatures. Studies have shown that extremity temperatures can rise by as much as 9.5 to 14 degrees Celsius.

To alleviate a hot flare, it is advisable to engage in activities that promote the activation of the Parasympathetic Response, such as deep breathing, meditation, and calming techniques.

Understanding the distinct characteristics of both the cold and hot subtypes of CRPS allows for better management and targeted treatments tailored to individual needs. Consultation with healthcare professionals experienced in dealing with CRPS is crucial for proper diagnosis and the development of effective strategies to alleviate symptoms and improve quality of life.

Stages Of CRPS

The stages of Complex Regional Pain Syndrome (CRPS), formerly known as Reflex Sympathetic Dystrophy (RSD), are essential to understand for proper management. It is important to note that these stages are symptom-based rather than time-based.

Stage 1: Acute Phase (Warm Phase)
  1.  Symptoms: Pain, allodynia, hyperalgesia, edema, abnormal skin blood flow, and sudomotor abnormalities.
  2.  Duration: Typically lasts around 3 months from the onset of the first symptom.
Stage 2: Dystrophic Stage (Cold Phase)
  1. Symptoms: Edema, skin dystrophy, ongoing pain, throbbing, burning pain, tight and discolored skin, muscle tightness and cramping, “brain fog,” and increased sensitivity to stress.
  2.  Duration Lasts 3 to 6 months but can vary for each individual.
Stage 3: Atrophic Stage
  1. Symptoms: Skin atrophy, functional impairment, decreased joint movement, loss of mobility, possible limb contraction, and increased risk of spreading.
  2. Duration: Considered as either the final stage or the transition stage, leading to Stage 4.
 

Understanding the stages of CRPS is crucial because early intervention during the acute phase offers the best chance for reversal. Aggressive treatment during this phase can help alleviate symptoms and improve outcomes. It is recommended to consult with a healthcare professional experienced in treating CRPS and undergo diagnostic tests such as X-rays, MRIs, bone scans, thermograms, and EMG to aid in the diagnosis and monitoring of the disease.

Documenting and photographing flare-ups, discoloration, and changes in symptoms can provide valuable information to healthcare providers. It is also important to manage stress levels, engage in physical therapy, and maintain open communication with your doctor regarding the risk of spreading and central sensitization.

Please note that while the stages of CRPS are described here, it is essential to consult with a medical professional for an accurate diagnosis and personalized treatment plan.

Who Is at Risk for CRPS? Understanding the Factors and Epidemiology

Complex Regional Pain Syndrome (CRPS) is a chronic, incurable condition characterized by relentless pain that often develops following an injury. This article aims to shed light on the individuals most vulnerable to this condition. By exploring the epidemiology and demographic patterns of CRPS, we can gain valuable insights into the affected population.

Epidemiology Insights
To comprehend the risk factors associated with CRPS, it is essential to understand its epidemiology—the study of how the disease affects people and how prevalent it is in a given population.

According to a study published by the US National Library of Medicine National Institutes of Health in 2017, the incidence rates for CRPS are as follows:

  1. Type I: Approximately 5.46 cases per 100,000 people per year.
  2. Type II: Roughly 0.82 cases per 100,000 people per year.

Combining both types, the overall incidence rate for CRPS is estimated to be 6.28 cases per 100,000 people per year. However, a subsequent population-based study by de Mos et al. challenged these numbers, suggesting that the true incidence rate might be four times higher, at 26.2 cases per 100,000 people per year. Furthermore, the study revealed a higher risk for CRPS in the upper limbs compared to the lower limbs, with a ratio of 3:2.

Understanding CRPS Demographics

Once we grasp the epidemiology, it becomes crucial to identify the demographic groups at risk. According to the National Institute of Neurological Disorders and Stroke, CRPS can affect anyone regardless of age, although it is more commonly observed in women, with a peak incidence at around age 40. The condition is rare among the elderly, and very few cases occur in children under the age of 10, particularly those under 5 years old.

Moreover, the US National Library of Medicine National Institutes of Health reports that CRPS is most prevalent among individuals aged 61 to 70, with a higher incidence among women, three times that of men. Risk factors associated with CRPS include menopause, a history of migraines, osteoporosis, asthma, angiotensin-converting enzyme (ACE) inhibitor therapy, and elevated intracast pressure due to tight cases or extreme positions. Additionally, smokers tend to have a poorer prognosis compared to non-smokers.

These statistics emphasize a notable gender disparity, with three times as many women being diagnosed with CRPS compared to men. Therefore, women should be particularly attentive to this condition, as it may significantly impact their lives or the lives of those they know.

Factors Influencing CRPS Risk

Several factors contribute to the risk of developing CRPS, including motor, sensory, immune system, and central nervous system components. Considering the complex interplay between these systems, determining the exact cause and risk factors for the disease becomes challenging. Unfortunately, CRPS can potentially affect anyone at any time, making it difficult to pinpoint specific individuals at high risk.

Conclusion

Complex Regional Pain Syndrome poses a significant challenge in identifying individuals at risk due to its multifaceted nature and wide-ranging impact on various bodily systems. Nonetheless, by comprehending the epidemiology and demographics surrounding CRPS, we can raise awareness of the condition and its prevalence among specific groups. Taking proactive measures to mitigate risk factors and promote early detection can be crucial in managing CRPS and minimizing its detrimental effects on individuals’ lives.

The Role of Genetics in CRPS: Can CRPS be Inherited?

A pressing concern for many parents suffering from Complex Regional Pain Syndrome (CRPS) is whether the condition can be passed down to their children. Researchers worldwide are conducting studies to investigate the potential link between CRPS and familial relationships, seeking to determine if the disease is indeed carried in our genes.

Inheriting CRPS: What Parents Need to Know
Once diagnosed with CRPS, parents often find themselves asking a crucial question: Does our DNA play a role in the development of CRPS, and can the condition be passed on to our children through our genetic makeup? This question looms large, fueling the fear that they may inadvertently burden their children with this unwanted affliction that has haunted them.

 

 

Studies have revealed that CRPS can, in fact, be inherited through genetics and encoded in our DNA. This revelation brings daily contemplation for thousands of parents who have survived the challenges of CRPS. Mothers, observing their beautiful daughters dancing around the room, cannot help but wonder if a simple misstep or ankle twist could sentence them to life mirroring their own. Fathers, witnessing their sons grow into strong young men while participating in physically demanding activities like football, experience a pang of worry with each tackle, fearing that one hit could confine them to a wheelchair, just like themselves.

As parents, it is a difficult balance to strike. We cannot shield our children within a protective bubble and force them to live in constant fear. However, we yearn to understand the risks involved, to determine if this monstrous condition can also haunt their lives and rob them of joy, as it has for us. Our hearts ache for our children, desiring that they lead happy lives. We long for them to grow up successful, healthy, and able to experience the life we couldn’t. Yet, lurking at the back of our minds is the unsettling presence of genetics.

CRPS is an incurable nervous system disorder with no known remedy, and studies have indicated an increased risk when a parent, sibling, or close relative has the disease. While we cannot alter our genetic legacy, being aware of the potential genetic predisposition can help us navigate the complexities of CRPS and take necessary precautions to ensure our children’s well-being.

Conclusion

Understanding the role of genetics in CRPS is a paramount concern for parents facing this debilitating condition. While studies suggest a potential link between familial relationships and the development of CRPS, it is crucial to approach the topic with informed caution. By staying informed and proactive, parents can empower themselves to navigate the challenges of CRPS and support their children in leading fulfilling lives, irrespective of inherited risk factors.

 

Risk Factors for CRPS: Exploring Neurological Associations and Unknown Causes​

The risk factors for developing Complex Regional Pain Syndrome (CRPS) appear to be linked to other neurological disorders characterized by the presence of pain. It’s important to note that the exact cause of CRPS remains unknown. However, several factors have been identified that may increase the likelihood of developing this condition.

 

  1.  Surgery
    1.  Post-surgery complications involving swelling, atrophy, osteoporosis, pseudo-arthrosis, joint stiffness, and tendon adhesions.
    2.  Operative procedures on the shoulder, distal radius, carpal tunnel, and Dupuytren’s contracture.
    3.  Operative procedures on the lower limbs, such as tibial fractures, knee surgeries, intramedullary

     

  2. Menopause
  3. History of Migraine or Chronic Migraine Headache
  4. Osteoporosis
  5. Asthma
  6. Use of angiotensin-converting enzyme (ACE) inhibitors at the time of trauma.
  7. Being the sibling of a young-onset case of CRPS.
  8. Individuals with elevated intracast pressure due to tight casing or extreme positions.
  9. Current or Previous Smoking
  10. Cerebral Lesions
  11. Heart disease, including a history of heart attacks
  12. Infection
  13. Paralysis on one side of the body (hemiplegia)
  14. Radiation therapy
  15. Repetitive motion disorders (e.g., carpal tunnel syndrome)
  16. Spinal cord disorders
  17. Trauma, such as bone fractures, gunshot wounds, and shrapnel injuries
  18. Having a parent or close family member with CRPS

It’s important to recognize that while these risk factors may be associated with the development of CRPS, they do not guarantee its occurrence. The interplay of various factors and the complex nature of the condition contribute to the challenges in identifying a definitive cause.

By understanding these risk factors, healthcare professionals can better evaluate individuals who may be at higher risk of developing CRPS. Early identification and appropriate management can help mitigate the impact of this debilitating condition and improve patients’ quality of life.

Los Angeles, California

victory@warriorsofcrps.org

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