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What is Complex Regional Pain Syndrome, Central Pain Syndrome

Reflex Sympathetic Dystrophy/ RSD AKA: CRPS Complex Regional Pain Syndrome/ NID/ Causalgia/RND /Reflex Neurovascular Dystrophy/ CPS Central Pain Syndrome

This neuro-autoimmune disease has changed names over 20 times throughout history and is projected to again change names as more bio-mechanical information is discovered. Other names include: Post Sympathectomy Pain, Hyperpathic Pain, and Mimocausalgia, among many others. This condition is a progressive neurological condition that can affect one extremity or all 4, and can go full body. It also can affect the immune system, skin, muscles, joints, and bones. The pain can start in one area but spread to other areas of the body. The syndrome usually develops after a minor insult to the body, such as whiplash, sprain, broken bone, or following surgery and in a few cases, no precipitating insult can be identified. RSD/CRPS is characterized by constant burning pain at various levels but always present. Patients may also see excessive sweating, swelling, and sensitivity to touch. RSD can go into remission for a period of time and then reappear with a new injury. This most commonly occurs when it is treated within the first 9 months with appropriate care.

Things to remember:

  • Minor injuries, such as a sprain or a fall are frequent causes of RSD/ CRPS. One characteristic of RSD/ CRPS is that the pain is more severe than expected for the type of injury that occurred.
  • Early and accurate diagnosis and appropriate treatment are key to recovery, yet many health care professionals and consumers are unaware of its signs and symptoms. Typically, people with RSD/ CRPS report seeing an average of five physicians before being accurately diagnosed.
  • RSD/ CRPS is three times more frequent in females than males.
  • The common age at diagnosis is 42 years. However, more injuries among young girls, and children as young as 3 years old can get CRPS.
  • This is not a psychological syndrome, but people may develop psychological problems when physicians, family, friends, and co-workers do not believe their complaints of pain. RSD/ CRPS has been categorized with two types, although both share the same signs and symptoms.

Millions of people in the United States suffer from RSD. According to Dr. Robert Schwartzman, a leading doctor in the field of RSD treatment, RSD appears to involve a complex interaction among the sensory, motor, and autonomic nervous systems, and the immune system. The brain and spinal cord (central nervous system) which as control over these various processes is somehow changed as a result of an injury. Early treatment is the key, Leading experts now believe that any invasive procedure or injury can cause the RSD to worsen or spread. Treatments such as spinal cord simulators, pain pumps and sympathectomies are now considered contraindicated. As well application of ice to the affected areas is also not recommended. Ketamine Infusions are believed to be the future standard treatment for RSD by top doctors in the field and have been very successful to date. There are not many doctors performing this procedure and wait lists are extremely long for the doctors who are successful with it. The protocol makes a difference, be sure to check with your doctor(s) prior to any treatments or medical intervention. Other treatment modalities include nerve blocks, massage, traction for upper extremity, proper posture, medications At this time, there is no cure, but the bio-mechanical mechanisms are now known and progress for a cure is underway. At this time the closest thing to a cure is Ketamine Infusions and hope.

Sympathetically Maintained Pain

One of the factors common to RSD and Causalgia is that almost all patients will respond to sympathetic blockade (blockage of the sympathetic nerves supplying the area with local anesthetics), which will take away their pain for a variable length of time. However, many patients do not present with the full-blown syndrome that includes all the signs listed above, but do respond to a sympathetic blockade. For example, a patient may come to a doctor with pain only. There may not be Allodynia, there may not be swelling, there may not be muscle spasms or any of the other factors relevant to the diagnosis of RSD. However, a sympathetic blockade takes away their pain. In fact, patients can present with any of the symptoms of RSD on their own. They can present with just swelling, just Allodynia, just burning pain, muscle spasm, etc., and if these people respond to sympathetic blocks, they are then defined as having sympathetically maintained pain or sympathetically maintained pain syndrome sympathetically maintained pain.

Sympathetically Independent Pain

Some patients will present with the classic symptoms of RSD; however, sympathetic blockade does not take away their pain. This may be due in part to a disease process that we don’t understand, or it may be that these patients have progressed so far along in their disease that the disease has become centrally maintained only (there are now changes in the nerve cells in the spinal cord), and sympathetic blocks have little or no effect on it whatsoever. Often SIP is seen later in the development or progression of RSD patients.

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Symptoms

  • Pain– Aching, Burning, Crushing, Dull, Electric, Feeling as if you’re on fire, Sharp, Stabbing, Throbbing, Tingling are some ways to describe our pain
    • The pain can be anywhere around the affected area , not always right where the trauma was
    • The affected area is usually hot or cold to the touch
    • The pain will be more severe than expected for the type of injury sustained
    • Lowered threshold to pain from external stimuli
  • Extreme sensitivity to touch-Something as simple as a slight touch, clothing, sheets, even a breeze across the skin on the affected area can cause an extreme amount of pain to the patient. The softest touch can now cause pain instead of pleasure
  • Bone Changes– Softening of the bones, Osteoarthritis, Osteoporosis, joint stiffness/ tenderness
    • Thinning and weakness of your bones become more evident
    • At risk for more fractures
  • Cognitive Issues –
    • Short-term memory problems- Many patients think they are losing their mind as their ability to remember things, greatly decreases
    • Things like whether you took your pills this morning and what you were just talking about etc.
    • You are NOT losing your mind
    • Problems with short-term memory is part and parcel of RSD and other Neuropathies
    • Concentration is also lessened while their level of irritability is increased
  • Chronic Fatigue / Body fatigue – Increased body fatigue, fever, rashes, sores, swelling also possible
  • Emotional Disturbances– Depression, Pain CAUSES Depression, NOT the other way around, Agitation, Irritability
  • Hearing/Vision– Pain can be exacerbated by sounds and vibrations, especially sharp sudden sounds and deep vibrations
    • Horner’s syndrome
    • Dry eyes and others
    • Visual disturbances such as blurriness
    • Proprioception function lowered
      • Tinnitus (ringing in the ears)
      • Dizziness, Vertigo, Balance
  • Increased Tone with skin and muscle atrophy – Muscle and skin tightness
  • Immune System Dysfunction – More susceptible to illness/viruses (colds/flu/infection)
  • Low-grade fever
  • Limbic system Dysfunction – This causes many problems that might not initially be linked to a disease like RSD. This system supports a variety of functions, including emotion, behavior, motivation, long-term memory, and olfaction
  • Movement Disorders / Dystonia – Difficulty in beginning movement of the injured part or moving it in general, Joint stiffness resulting in limited range of motion, Permanent damage to muscles and joints, Increased reflexes, Tremors of the extremity and muscle spasms
  • Nails/Hair changes– On affected extremity, they may grow at an increased or slower rate become grooved and brittle. Hair may become coarse and may be followed by hair loss
  • Swelling- It takes various forms, the skin may appear mottled, become easily bruised; have a shiny, dry, red, and tight look to it. Swelling is not always present. Swelling can spread to involve a larger area and becomes brawny (hard), Edema – Swelling that is usually localized to the affected limb and may have a well demarcated edge
  • Sweating– An increase usually occurs
  • Spasms / Tremors – The spasms can be confined to one area or be rolling in nature; moving up and down the leg, arm, or back
  • Skin rashes / Sores / Ulcers
  • Sleep Disorder/Insomnia– Insomnia is often seen, also disrupted sleep pattern. Some Medications help this. Not allowing the body to drift into REM, or rapid eye movement, sleep. REM sleep allows the body to use its own healing abilities. Without it, the patient’s pain cycle continues and becomes more entrenched. As the body cannot heal itself, it becomes harder to achieve that sleep which makes the pain worse and so the cycle continues
  • Vascular – Constriction of blood vessels, arteries, veins. Thoracic Outlet Syndrome symptoms, Ischemia (intestines, heart..), Headaches, Migraines, Optical Migraines, Coldness in the affected extremity

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Treatments

The general strategy in RSD treatment is often multi-disciplinary, with the use of different types of medications combined with distinct physical therapies.

  • Blocks: Injection of a local anesthetic such as lidocaine is often the first step in treatment. Injections are repeated as needed. The results of local anesthetic injections are short lasting and the procedure is risky. However, early intervention with non-invasive management may be preferred to repeated nerve blockade. The use of topical lidocaine patches has not been shown to be of use in the treatment of RSD-1 and -2

 

  • Cortical Integrative Therapy (CIT®) – a scientific, research-based treatment designed to address brain and neurological dysfunction in both children and adults.  By adopting a multidisciplinary approach, we are changing the way in which brain-related disorders and traumatic brain injury (TBI) are treated.  Instead of managing symptoms, we identify the root cause of the problem and work to significantly reduce and/or resolve the symptoms of a disorder. Cortical Integrative Therapy (CIT®) has been successful in treating a wide range of painful and debilitating conditions including: ADD/ADHD; apraxia; ataxia; chronic pain; dystonia; Dysautonomia; hypotonia; memory difficulties; movement disorders; Parkinson’s disease; Reflex Sympathetic Dystrophy (RSD)/Chronic Regional Pain Syndrome (RSD); RLS; sciatica; traumatic brain injury; tremors; and vertigo, balance, and gait problems. In simplistic terms, CIT® stimulates the cerebral cortex through a combination of non-invasive therapies tailored to meet the specific treatment needs of each patient.  This treatment methodology stimulates brain cells to increase their size and efficiency, thereby promoting the formation of pathways which facilitate the transfer of information throughout the brain.  In the end, the affected area of the brain and overall brain function are improved without medication or surgery. The duration of treatment will vary based on the age, physical condition, and motivation of each patient; the type and stage of disorder; the kinds of medications that have been prescribed; and a host of other individual factors.
  • Creams/Lotions: RSD can also be treated with DMSO 50% cream a novel approach to treat RSD is with the multimodal stepped care approach. Step by step a topical analgesic will be tried to examine its effectiveness in reducing pain. When a topical analgesic has some pain reducing effects, though not completely, another topical analgesic from a different class can be added to enhance the pain reducing effects. Usually one to four topical agents can be used simultaneously to get an optimal pain reducing effect.

 

  • EEG Biofeedback – various forms of psychotherapy, relaxation techniques and hypnosis are adjunctive treatments which assist coping.

 

  • Graded Motor Imagery and Mirror Therapy are particularly helpful. Graded motor imagery is a sequential process that consists of (a) laterality reconstruction, (b) motor imagery, and (c) mirror therapy. Mirror box therapy uses a mirror box, or a stand-alone mirror, to create a reflection of the normal limb such that the patient thinks they are looking at the affected limb. Movement of this reflected normal limb is then performed so that it looks to the patient as though they are performing movement with the affected limb (although it will be pain free due to the fact it is a normal limb being reflected). Mirror box therapy appears to be beneficial in early RSD (McCabe et al., 2003b);. However, Lorimer Moseley (University of South Australia) has cautioned that the beneficial effects of mirror therapy for RSD are still unproven. Importantly, the precise neural mechanisms of action are unknown, and need to be studied using a combination of behavioral and neuroimaging approaches. Because studies have shown that problems in the primary motor cortex are found in patients who suffer from RSD, treatments have been developed that focus on normalizing motor representations in that part of the brain.

 

  • Infusion Therapy: Ketamine, Lidocaine, IVIg, Stem-cell
    • Ketamine, a dissociative anesthetic, is being used in the treatment of RSD more and more. During the infusion the patient is monitored constantly, and it should be administered only by a qualified physician such as an anesthesiologist. The theory of ketamine use in RSD/RSD is primarily advanced by neurologist Dr Robert J. Schwartzman of Drexel University College of Medicine in Philadelphia, and researchers at the University of Tübingen in Germany, but was first introduced in the United States by Doctor Ronald Harbut of Little Rock, Arkansas. Ketamine blocks NMDA receptors which might reboot aberrant brain activity and binds to the same glia receptors that opiods do, except they calm instead of excite the glia receptors.
      • There are two treatment modalities; the first consist of a low-dose subanesethesia Ketamine infusion of between 10–90 mg per hour over several treatment days, this can be delivered on an outpatient basis. This is called the awake or subanesethesia technique. One study demonstrated that 83% of the patients that participated had complete relief and many others had some relief of the symptoms. Another evaluation of a 10-day infusion of intravenous ketamine (awake technique) in the RSD patient concluded that “A four-hour ketamine infusion escalated from 40–80 mg over a 10-day period can result in a significant reduction of pain with increased mobility and a tendency to decreased autonomic dysregulation”.

     

  • Medications: Physicians use a variety of drugs to treat RSD, including antidepressants, anti-inflammatories such as corticosteroids and COX-inhibitors such as piroxicam, bisphosphonates, vasodilators, GABA analogs such gabapentin and pregabalin, and alpha- or beta-adrenergic-blocking compounds, and the entire pharmacy of opioids.

 

  • Psychotherapy aims to increase the individual’s sense of his/her own well-being. Psychotherapists employ a range of techniques based on experiential relationship building, dialogue, communication and behavior change that are designed to improve the mental health of a client or patient, or to improve group relationships (such as in a family).

 

  • RSD specific physical and occupational therapy are important components of the management of RSD primarily by desensitizing the affected body part, restoring motion, and improving function. Physical therapy interventions for RSD can include specific modalities such as TENS, non-weight bearing exercises, tactile desensitization, massage, traction, stretching. These interventions tailored specifically to each individual person can be used to improve pain and function to help people perform more activities of daily living. Some people with RSD are incapable of participating in physical therapy due to touch intolerance. People with RSD often develop guarding behaviors where they avoid using or touching the affected limb. This inactivity exacerbates the disease and perpetuates the pain cycle. Therefore optimizing the multimodal treatment is paramount to allow for use of the involved body part. Physical therapy works best for most patients, especially goal-directed therapy, where the patient begins from an initial point, regardless of how minimal, and then endeavors to increase activity each week. Therapy is directed at facilitating the patient to engage in physical therapy, movement and stimulation of the affected areas. One difficulty with the idea of Physical Therapy, however, is that it means different things to different people. There is one systematic review of the use of physical and occupational therapy for the treatment of RSD. That review concluded: “Narrative synthesis of the results, based on effect size, found there was good to very good quality level II evidence that graded motor imagery is effective in reducing pain in adults with RSD-1, irrespective of the outcome measure used. No evidence was found to support treatments frequently recommended in clinical guidelines, such as stress loading. CONCLUSIONS: Graded motor imagery should be used to reduce pain in adult RSD-1 patients.”  Physical therapy has been used under light general anesthesia in an attempt to remobilize the extremity. Such remobilization is used cautiously to avoid damage to atrophied tissue and bones which have become osteodystrophic.

 

  • Scrambler therapy/ Calamare use direct nerve electrocutaneous stimulation to produce analgesia via the MC-5A computerized medical device. The MC-5A recently gained FDA clearance for use in the United States. Invented in Italy by Professor Giuseppe Marineo, the computerized medical device mixes non-pain with pain messages carried through surface electrodes to c-fibers. This mechanism helps resolve pain signals to the affected area reducing the perception of neuropathic pain. Several studies have suggested this technology is effective at reducing chronic neuropathic pain with a durable effect. Usually several 30 minute treatments sessions on consecutive days are needed to allow the brain to change its perception of the pain message leading to a lasting and sometimes permanent no/low pain state. There are no known side-effects and the technique is drug free and noninvasive.

 

  • Surgical, chemical, or radiofrequency sympathectomy — interruption of the affected portion of the sympathetic nervous system — can be used as a last resort in patients with impending tissue loss, edema, recurrent infection, or ischemic necrosis. However, there is little evidence that these permanent interventions alter the pain symptoms of the affected patients and in addition to the normal risks of surgery, such as bleeding and infection, sympathectomy has several specific risks, such as adverse changes in how nerves function. However, there is some research suggesting good prognosis for patients who have responded favorably to a series of sympathetic blocks (3-6).

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