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    Complex Regional Pain Syndrome

    What is complex regional pain syndrome (CPRS)?

    • Complex regional pain syndrome (CRPS) is a poorly understood condition where a person experiences persistent severe and debilitating pain.

    How common is CPRS?

    • It is a very uncommon condition affecting 0.00026% of the population.

    Should I worry?

    • No.
    • At present there is no proven cure for complex regional pain syndrome but, with quick diagnosis and early treatment, significant improvements can be achieved.
    • Early treatment can allow the symptoms of complex regional pain syndrome to become more manageable, subside, or become relapsing and remitting in nature.
    • People with lasting symptoms may need access to other services such as pain management.

    Who is most likely to suffer from CPRS?

    • Complex regional pain syndrome commonly arises after an injury to an affected limb.
    • The wrist is the most frequently affected joint following a fracture.
    • Complex regional pain syndrome is 3 to 4 times more likely in women than men.
    • It can occur at any age, however, it is rare in children and adolescents.

    What are the common symptoms?

    • Unprovoked or spontaneous pain that can be constant or fluctuate with activity (1).
    • Excess or prolonged pain after use or contact.
    • Changes in skin temperature, skin colour or swelling of the affected limb.
    • Changes in skin texture.
    • Abnormal sweating and nail or hair growth.
    • Stiffness in the affected joints.
    • Loss of muscle or excess bone growth.
    • Reduced muscle strength and movement.

    What can I do?

    • Speak to your GP/consultant about pain relief.
    • Desensitisation (stroke the affected limb to make it less sensitive to touch/sensation) (2).
    • General exercises (2).
    • Continue with normal daily activities.
    • Pacing your day-to-day life to make it more manageable.
    • Relaxation techniques.

    How long will it take to recover?

    • There is currently no definitive cure for chronic regional pain syndrome but appropriate treatment can reduce the effect of the condition.
    • Limb signs (such as swelling, sweating and colour changes) usually reduce with time, even where pain persists (2).
    • 15% of people with complex regional pain syndrome are considered to have a long-term condition, although with lesser degrees of ongoing pain and dysfunction.
    • Research has also shown that, depending on the severity of the condition, some may recover with no symptoms or very mild/minimal symptoms (2).
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    1. Introduction

    Complex regional pain syndrome can be a debilitating, painful condition in a limb, associated with sensory, motor, autonomic, skin and bone abnormalities (1). Complex regional pain syndrome commonly arises after an injury to the affected limb, with many people noticing the onset of symptoms within one month. However, there has been no relationship shown between the onset of complex regional pain syndrome and the severity of the initial trauma (2). In some cases, there is no initial trauma at all (9%). Complex regional pain syndrome usually affects one limb, however, in 7% of cases, the condition has been shown to later spread and involve additional limbs (5).

    Complex regional pain syndrome can be divided into two types:

    At present, the diagnosis of either type of complex regional pain syndrome has no bearing on the management strategies (2). This means that regardless of the type of complex regional pain syndrome you have, the treatment will be the same. Complex regional pain syndrome is still a relatively poorly understood condition so research and clinical studies into the causes, management and treatment are ongoing.

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    2. Signs & Symptoms

    Stage 1: acute (early) inflammation. This usually occurs 10 days after the initial trigger, surgery or trauma. Common symptoms include (7):

    Stage 2: this stage usually begins 3-6 months after the onset of pain. During this stage, symptoms may progress/change and include the following (6):

    Stage 3: the final phase usually begins after 6-12 months. During this phase, the symptoms may resolve or resolve and then reappear. The pain may begin to spread proximally (towards the body) or the level of pain felt may plateau (stay the same). Other symptoms in the final phase may include (6):

    3. Causes

    The cause of complex regional pain syndrome is not yet fully understood however, it is thought that it usually begins after sustaining an injury or trauma. One avenue explored by research into the cause of complex regional pain syndrome is thought to be related to either an injury or abnormality to the peripheral nervous system (nervous system outside the brain and spinal cord) and central nervous system (brain and spinal cord) of the body.

    The two types of complex regional pain syndrome, although similar in terms of signs, symptoms and treatment, are thought to have different causes. It is believed that type 1 complex regional pain syndrome occurs after an illness or injury that did not directly damage the nerves in the affected limb. Type 2 is thought to only occur after a distinct nerve injury. Overall, 5% of people who develop symptoms do so following a traumatic injury and 91% of all complex regional pain syndrome cases occur following surgery (2).

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    4. Risk Factors

    Research into complex regional pain syndrome is still in its infancy and is currently ongoing so determining the risk factors for developing the condition is slightly more challenging. It is thought that as type 2 complex regional pain syndrome occurs as a direct injury to the nerve, there are no risk factors associated with developing it. On the other hand, research into type 1 complex regional pain syndrome has found some potential risk factors as follows (3):

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    5. Prevalence

    This is a very rare condition that normally begins after another condition or injury, overall it only affects 0.00026% of the population. It is more common in people that have had paralysis to one side of the body, nerve injury or in conditions such as multiple sclerosis.

    6. Assessment & Diagnosis

    A diagnosis of complex regional pain syndrome is usually made by a GP or consultant. Confirmation of the diagnosis is based on clinical examination and presenting signs and symptoms in accordance with the Budapest diagnostic criteria.

    An answer of yes to all questions A-D on the Budapest diagnostic criteria will generate a diagnosis of complex regional pain syndrome. However, for those who fulfil the criteria but do not have any limb signs (i.e. swelling, colour or temperature changes) a diagnosis of ‘complex regional pain syndrome – not otherwise specified’ is given (2). This means patients who did not fulfil the Budapest criteria, but whose signs and symptoms could not be better explained by any other diagnosis.

    As well as this, other diagnostic tools can be used:

    • Sweat testing: this involves taking a sweat sample from both sides of the body at the same time and using a Q-sweat instrument to measure the amount of sweat production. In complex regional pain syndrome, the affected limb may sweat excessively or not at all.

    7. Self-Management

    Your physiotherapist should be able to help you understand the condition and what needs to be implemented to effectively reduce the impact on your day-to-day life. This will include activity modification strategies as well as other useful treatments aimed at reducing discomfort. Regular adherence to a condition-specific rehabilitation programme is important in the management of this condition. It should be noted that rehabilitation exercises are not always a quick fix, but if adhered to on a consistent basis (weeks to months), over time they have been shown to yield positive outcomes.

    The approaches that have been shown to have a positive effect are:

    • Gentle limb movements.
    • Frequently giving attention to the affected limb.
    • Desensitisation (touching and stroking the limb).
    • Remaining as active as possible.
    • Pacing, prioritising and planning activities (2).
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    8. Rehabilitation

    As pain is typically the presiding symptom of complex regional pain syndrome and is often associated with limb dysfunction and psychological distress, an integrated interdisciplinary (combination of people from several academic areas) treatment approach is recommended. The primary aim of rehabilitation is to reduce pain, preserve or reserve function and enable (4).

    The four pillars of care show how education, pain relief, physical rehabilitation and psychological intervention all have equal importance in the long-term management of complex regional pain syndrome. Due to the complex nature of the condition, all rehabilitation will focus on patient-centred care, helping both physically and psychologically with movements and goal setting (2). Also, where possible, including the patient’s partners/families.

    9. Complex Regional Pain Syndrome Rehabilitation Plans

    Early Plan

    During the early phase of complex regional pain syndrome, the main aim is gentle movement and therapy focussing on the opposite side of the body to the affected limb – this is called contralateral therapy. Contralateral therapy is usually undertaken with your physiotherapist who will devise a programme for the unaffected limb. It is believed that working on the unaffected limb, will stimulate muscles and pain signalling in the affected limb thereby improving the movement and reducing pain. This should not exceed any more than 3/10 on your perceived pain scale.

    Early Plan - Rating

    Advanced Plan

    In this phase focus will move to more functional exercises aimed to increase overall tolerance for movement. This should not exceed any more than 2/10 on your perceived pain scale.

    Advanced Plan - Rating
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    10. Return to Sport/Normal Life

    For patients wanting to achieve a high level of function or return to sport, we would encourage a consultation with a physiotherapist as you will likely require further progression beyond the advanced rehabilitation stage.

    As part of a comprehensive treatment approach, your musculoskeletal physiotherapist may also use a variety of other pain-relieving treatments to support symptom relief and recovery. Whilst recovering you might benefit from a further assessment to ensure you are making progress and establish the appropriate progression of treatment.  Ongoing support and advice will allow you to self-manage and prevent future re-occurrence.

    11. Other Treatment Options

    • Research has also shown that techniques such as relaxation, cognitive behaviour therapy, deep breathing exercises and biofeedback (a type of therapy that uses sensors attached to your body to measure key body functions, e.g. heart rate monitor) can also aid recovery/management.
    • Occupational therapy to help with adaptations and aids for functional tasks, educating around pacing and helping with engagement with community groups.
    • Dermatology to help with skin changes including dryness, glossy texture, swelling/oedema.
    • Psychological support, with studies showing 1:1 input, can be more beneficial when compared to group settings, using cognitive behavioural therapy, behavioural treatment, and relaxation methods such as tai chi, yoga. (4)
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    Book an Appointment

    Please book an appointment with one of our physiotherapists if you think you are suffering from this condition and would like to find out more.

    We have Pure Physiotherapy clinics across the country including Norwich, Ipswich, Manchester, Bolton, Wakefield and Sheffield. Please view our clinics to find the closest physiotherapy clinic for you.

     

    References

    1. Ninds.nih.gov. (2021). Complex Regional Pain Syndrome Fact Sheet | National Institute of Neurological Disorders and Stroke. Available at: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Complex-Regional-Pain-Syndrome-Fact-Sheet
    2. Royal College of Physicians (2012). Complex regional pain syndrome in adults UK guidelines for diagnosis, referral, and management in primary and secondary care. PDF. file:///C:/Users/Pure%20Physio/Downloads/Complex%20regional%20pain%20syndrome%20guidelines.pdf
    3. Tracey Pons, Edward A. Shipton, Jonathan Williman, Roger T. (2015) Mulder, ‘Potential Risk Factors for the Onset of Complex Regional Pain Syndrome Type 1: A Systematic Literature Review’, Anaesthesiology Research and Practice,bhttps://www.hindawi.com/journals/arp/2015/956539/. 15.
    4. Royal College of Physicians (2018). Complex regional pain syndrome in adults UK guidelines for diagnosis, referral and management in primary and secondary care. PDF file:///C:/Users/Pure%20Physio/Downloads/Complex%20regional%20pain%20syndrome%20in%20adults%20-%20second%20edition_0.pdf
    5. G Wasner, J Schattschneider, K Heckmann, C Maier, R Baron. ‘Vascular abnormalities in reflex sympathetic dystrophy (CRPS 1): mechanisms and diagnostic value’ 2001, Part 3, 587 – 99. https://pubmed.ncbi.nlm.nih.gov/11222458/
    6. Schattschneider, Jörn MD; Binder, Andreas MD; Siebrecht, Dieter MD†; Wasner, Gunnar MD; Baron, Ralf MD. (2006.) ‘Complex Regional Pain Syndromes: The Influence of Cutaneous and Deep Somatic Sympathetic Innervation on Pain’. 22, 240-244. https://journals.lww.com/clinicalpain/Abstract/2006/03000/Complex_Regional_Pain_Syndromes__The_Influence_of.3.aspx
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