Type I, also known as reflex sympathetic dystrophy (RSD), Sudeck's atrophy, Reflex neurovascular dystrophy (RND) or algoneurodystrophy, does not have demonstrable nerve lesions.
Type II, also known as causalgia, has evidence of obvious nerve damage.
The cause of this syndrome is currently unknown. Precipitating factors include illness, injury and surgery, although there are documented cases that have no documentable injury to the original site.
The condition currently known as CRPS was originally described by Silas Weir Mitchell during the American Civil War, who named the condition causalgia.
In the 1940s, the term reflex sympathetic dystrophy came into use to describe this condition, based on the theory that sympathetic hyperactivity was involved in the pathophysiology.[1]
Misuse of the terms, as well as doubts about the underlying pathophysiology, led to calls for better nomenclature.
In 1993, a special consensus workshop held in Orlando, Florida, provided the umbrella term "complex regional pain syndrome," with causalgia and RSD as subtypes.[2]
Reflex sympathetic dystrophy (RSD) is a complex, poorly understood disorder that is characterized by chronic, severe pain and progressive changes in skin, muscle, and bone.
Although the precise causes of RSD are unknown, it often occurs following an injury, often minor in nature.
Some experts believe that RSD represents an exaggerated response of the sympathetic nervous system to some form of injury or insult (eg. surgery) to the area resulting in chronic, severe, sometimes debilitating pain.
Although the signs and symptoms of RSD vary depending upon the clinical stage of the disorder, the one common feature shared by all 3 clinical stages (early, established, or late RSD) is pain.
Because currently there is no cure for RSD, the goals of treatment include: 1) controlling and minimizing pain to the greatest extent possible; 2) restoring function to the RSD-affected limb; 3) preventing progression of the disease process to the late stage; and 4) improving the patient's quality of life and psycholocial functioning.
The MediFocus Guidebook on Reflex Sympathetic Dystrophy will inform you about the signs and symptoms of RSD, the treatment options including drug therapy, sympathetic nerve blocks, chemical and surgical sympathectomy, physical therapy, and other methods used for controlling pain and improving quality of life.
You will also learn about new, experimental treatments that are currently under investigation that may be effective for the management of RSD.
You will also learn about the doctors, hospitals, and medical centers that are at the leading edge in conducting clinical research about RSD.
A list of important questions to ask your doctor, and a valuable directory of resources, organizations, and support groups that can help patients with RSD are included in this Guidebook.
Complex regional pain syndrome (CRPS) is a chronic pain condition.
The key symptom of CRPS is continuous, intense pain out of proportion to the severity of the injury, which gets worse rather than better over time.
CRPS most often affects one of the arms, legs, hands, or feet.
Often the pain spreads to include the entire arm or leg.
Typical features include dramatic changes in the color and temperature of the skin over the affected limb or body part, accompanied by intense burning pain, skin sensitivity, sweating, and swelling.
Doctors aren’t sure what causes CRPS.
In some cases the sympathetic nervous system plays an important role in sustaining the pain.
Another theory is that CRPS is caused by a triggering of the immune response, which leads to the characteristic inflammatory symptoms of redness, warmth, and swelling in the affected area.
Reflex Sympathetic Dystrophy Syndrome (RSD) - also known as Complex Regional Pain Syndrome (CRPS) - is a chronic neurological syndrome characterized by:
*severe burning pain
*pathological changes in bone and skin
*excessive sweating
*tissue swelling
*extreme sensitivity to touch
[For a complete description of CRPS symptoms, please read the Diagnosis section of the Clinical Practice Guidelines section of this website.]
There are Two Types of CRPS - Type I and Type II.
CRPS Type I (also referred to as RSD) - cases in which the nerve injury cannot be immediately identified
CRPS Type II (also referred to as Causalgia) - cases in which a distinct "major" nerve injury has occurred
CRPS is best described in terms of an injury to a nerve or soft tissue (e.g. broken bone) that does not follow the normal healing path
CRPS development does not appear to depend on the magnitude of the injury.
The sympathetic nervous system seems to assume an abnormal function after an injury
Since there is no single laboratory test to diagnose RSD/CRPS, the physician must assess and document both subjective complaints (medical history) and, if present, objective findings (physical examination).
Criteria for Diagnosing:
Complex Regional Pain Syndrome Type I (RSD)
The presence of an initiating noxious event, or a cause of immobilization Continuing pain, allodynia, or hyperalgesia with which the pain is disproportionate to any inciting event
Evidence at some time of edema, changes in skin blood flow (skin color changes, skin temperature changes more than 1.1°C difference from the homologous body part), or abnormal sudomotor activity in the region of the pain
This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction
Complex Regional Pain Syndrome Type II (Causalgia)
The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve
Evidence at some time of edema, changes in skin blood flow (skin color changes, skin temperature changes more than 1.1°C difference from the homologous body part), or abnormal sudomotor activity in the region of pain
This diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction.
Reflex Sympathetic Dystrophy, Complex Regional Pain Syndrome, are conditions still not fully understood and sometimes difficult to diagnose.
Although the symptoms were detailed over 100 years ago, the mechanism of the condition still remain unclear.
Reflex Sympathetic Dystrophy, has over the years, been referred to by many different names, these include Sudeck's Astrophy, Algodystrophy and most recently, Complex Regional Pain Syndrome (CRPS) Complex Regional Pain Syndrome type 1 is when the the condition does not reveal any detectable nerve damage.
CRPS type 2 is diagnosed as such, when there are clearly nerve lesions.
Reflex Sympathetic Dystrophy is a multi-symptom condition, some of the main attributes being, pain, abnormal regulation of the blood flow, sweating, oedema of the skin and active passive movement disorder, including physiological tremor.
Sufferers may also experience several other abnormalities, these include, rapid and abnormal growth to the nails on the affected limb and abnormal hair growth to the limb.
To be diagnosed with RSD, a person does not have to exhibit all the symptoms mentioned, the only one common factor being pain.
Whilst the condition is difficult to diagnose, the medical profession are now becoming more aware of the symptoms and are discovering more advanced ways in diagnosing the condition.
The use of MRI and Raioisotope scans can help in the diagnosis of the condition, but the use of such scans alone doesn't provide all of the answers.
Although the condition is not fully understood, it is generally thought that a group of nerve fibres called the 'sympathetic nerve system' is in some way involved in the development of the condition.
This booklet has been produced for anyone interested in finding out more about reflex sympathetic dystrophy (RSD).
Many people now prefer the term 'complex regional pain syndrome (CRPS)' to 'RSD'.
There are also a number of other names which have been used to describe the condition in different situations, including 'Sudek's atrophy' and 'algodystrophy'.
However, the term RSD is still in widespread use, probably because it is much easier to remember and say.
So RSD is the term used in this booklet.
We want to explain as much as possible about the condition; how it is diagnosed and how it can be treated.
However we cannot hope to answer all your questions.
Everyone is different and this booklet is not intended to replace an individual consultation with a doctor.
Reflex sympathetic dystrophy (RSD) is a condition characterized by localized or diffuse pain, usually with associated swelling, trophic changes and vasomotor disturbance [1].
Allodynia, hyperhydrosis, and nail or hair growth changes may also occur.
Motor abnormalities have been reported [2], in particular, tremor, involuntary movement and muscle spasm.
Contractures may occur in the later stages.
Involvement may be either unilateral or bilateral. Most commonly recognized is peripheral disease [3], although RSD may affect any region of the trunk or limbs.
There is often a history of trauma, occasionally of such low significance that it may be overlooked by the patient.
Symptoms may occur up to 6 months after injury [4]. Other triggering factors have been reported.
Several drugs have been implicated, for example, phenobarbitone, phenytoin, isoniazid [5], and the immunosuppressive agents cyclosporin [6] and tacrolimus [7], as has surgery or a neurological event, particularly with peripheral manifestations.
Rapamycin is currently under investigation as an immunosuppressive agent administered after solid organ transplantation.
A recent report associated this drug with bone pain, osteolysis on plain radiographs and high uptake of tracer on isotope bone scanning.
Resolution of symptoms occurred on withdrawal or reduction of rapamycin, or following administration of the bisphosphonate pamidronate [8].
Concurrent medical conditions may predispose to RSD, and diabetes mellitus, hyperthyroidism, hyperparathyroidism and type IV hyperlipidaemia have all been associated.
RSD may occur at any age, and is well recognized in children, where vasomotor changes may be particularly marked [9–11].
Chronic pain in a poorly understood condition may cause depression and isolation, and although a higher rate of psychological abnormalities has been reported, this appears to be little different from other patient groups who suffer chronic pain [12, 13].
COMPLEX regional pain syndrome (CRPS) type I, formerly known as reflex sympathetic dystrophy (RSD), and CRPS type II, formerly known as causalgia, are debilitating pain syndromes that have been recognized for more than a century.
Despite the long history of these disorders, the natural course and pathophysiology of CRPS types I and II are elusive, and hence, their therapies remain controversial.
The following overview highlights the clinically relevant aspects of CRPS type I in adults to guide practitioners in treating these patients.
The reader is referred to recent reviews for discussions about the proposed pathophysiologic mechanisms of this syndrome.
Complex Regional Pain Syndrome Type I (CRPSI; also known as Reflex Sympathetic Dystrophy) is a poorly understood and not well defined symptom complex comprising a combination of sensory, trophic, autonomic and motor impairments 1, 2.
The syndrome usually follows surgery or trauma, and is generally expressed in the extremities.
In addition to the impairments, CRPSI can lead to serious disabilities performing activities of daily life and handicap 3, 4.
In the acute phase of CRPSI, pain in particular may constitute a major cause of disability and/or handicap, whereas during the later stages CRPSI associated motor impairments, together with pain, are thought to bring about disabilities and/or handicaps 1, 5, 6.
The complex entity of CRPSI has often been investigated, leading, however, to confusing and conflicting results and theories about the aetiology and pathophysiology 7.
As the disease is not yet understood, plus the fact that each speciality has its own discipline-specific approach, a wide variety of treatments (more than 50) are found in literature 8.
As a consequence, numerous measures to determine treatment outcome have been described.
In the present paper, the numerous measures that are used to determine treatment outcome in CRPSI research and clinical practice will be classified.
So far, one of the difficulties in interpreting reports on treatment efficacy in CRPSI, has been the (objective) quantification of patient findings and the lack of uniform measurement of treatment outcome 9, 10.
Classification of outcome measures may not only be a useful tool to indicate the extent of the (obvious) inconsistency in defining treatment outcome in CRPSI research.
The main aim of classifying outcome measures in the present paper is to determine whether relevant and objective outcome measures for rehabilitation medicine are available.
It is clear that objective outcome measures are preferable to subjective outcome measures; the latter are more likely to endanger reliability and validity of measurements.
As for the relevance of outcome measures:
outcome measures are considered most relevant for rehabilitation medicine when they concern the goal of rehabilitation, that is regaining and/or maintaining of functionality by decreasing the consequences of a disease 11, 12.
Outcome measures concerning impairments are considered less relevant for rehabilitation medicine, especially since the relation between the consequences of a disease is often found to be rather ambiguous 13-15.
The international classification of impairments, disabilities and handicaps (ICIDH) 16 is an often-used classification, in which three hierarchical levels of the consequences of a disease on everyday life of patients are distinguished.
Outcome measures on the level of impairments, disabilities and handicaps concern the consequences of diseases at the level of the body, the person and the person as a social being, respectively.
As for CRPSI, the consequences at the ICIDH level of impairments can be categorised into sensory impairments (e.g. neuropathic pain, allodynia, hyperalgesia, hypesthesia, anaesthesia, dysesthesia, hyperpathia), autonomic impairments (e.g. oedema, hyperhydrosis, skin colour change, change of temperature), trophic impairments (e.g. atrophy of skin, nails, muscles and bone), and motor impairments (e.g. dystonia, weakness, spasms, tremor, difficulty initiating movement, increased tone and reflexes, and increase of complaints after exercise) 8.
Disabilities associated with CRPSI are those directly related to the involved extremity (e.g. problems with getting dressed with upper extremity CRPSI or climbing stairs with lower extremity CRPSI) and general disabilities in daily functioning (e.g. slow performance of activities of daily living).
Handicaps associated with CRPSI concern limitations in social functioning (e.g. alienation) and problems with role fulfilment (e.g. a grandmother with CRPSI cannot play with her grandchild), as a consequence of pain, other impairments or disabilities 17.
From this list of consequences it becomes clear that CRPSI encompasses all three levels of the consequences of a disease as described in the ICIDH.
Although some discussion continues about the sometimes unclear distinction between the theoretical levels of the ICIDH 18, 19, we consider the ICIDH framework useful to classify outcome measures in order to make a statement on availability of relevant and objective outcome measures for rehabilitation medicine.
Reflex Sympathetic Dystrophy (RSD), also known as Complex Regional Pain Syndrome (CRPS) Type I (here called RSD/CRPS), is a chronic condition characterized by burning pain and abnormalities in the sensory, motor and autonomic nervous systems.
The syndrome typically appears after an acute injury to a joint or limb, though it may occur with no obvious precipitating event.
In most cases, regardless of the site of injury the symptoms begin and remain most intense in the distal most extremity.
In the initial stages of RSD/CRPS, pain and swelling from the injury do not subside but actually intensify, spreading from the site of the injury to other parts of the limb, to the contralateral limb or to remote regions of the body.
The skin in affected areas and particularly deep somatic tissues are painfully sensitive to touch, often red and abnormally warm due to alterations in regional blood flow.
Changes in sweating patterns, hair growth, subcutaneous tissues, muscles, joints or bones and difficulty moving the joint or limb are other hallmarks of the disorder.
In addition to the evidence of inflammation and abnormal autonomic nervous system function, there are changes in motor systems including tremor, weakness and dystonia, which strongly suggest a central nervous system component to the disease in a subgroup of patients.
The syndrome may evolve through three stages (acute, dystrophic, atrophic), although this is very much debated, each marked by progressive pain and physical changes in the skin, muscles, joints and bones.
RSD/CRPS can affect both genders and all ages (including children), although it is thought to be more common between the ages of 40 and 60 and may be more frequent in women.
The cause of RSD/CRPS is unknown, and current treatments are not effective for many patients.
Complex regional pain syndrome (CRPS) is a condition of chronic pain and sensory changes that can occur after trauma to an arm or a leg.
The initial injury may be minor or severe.
Pain is the main feature of CRPS, but changes in blood flow to the skin, increased sweating, and swelling are common symptoms.
Movement disorders (MDs) such as tremor or dystonia develop in up to half the patients with CRPS. Dystonia is an abnormal twisting posture of the hand or foot.
In this study from the Netherlands, patients with CRPS and dystonia are compared to patients with CRPS who do not have MDs.
The authors tried to find out what causes the MDs to develop.
They looked at age, duration of symptoms, type of injury, and severity of symptoms as possible factors that cause MDs to develop.
They were able to come to several conclusions after comparing the two groups.
First, the patients who developed dystonia were younger than patients in the group without dystonia.
The length of time between the start of CRPS and the start of dystonia varies greatly.
For some patients this interval of time was within one week.
For others, the MD started up to five years later.
They found that once one extremity was affected, the chances of a second (or more) limb(s) developing dystonia increased greatly.
It's still not clear what causes dystonia to develop.
It's not linked to age, age, gender, or type of trauma.
Disease duration or which limb is affected first doesn't seem to make a difference either.
It looks like a central processing problem in the nervous system.
There may be many neural circuits at multiple levels affected causing the problem.
A change in function of nerve fibers capable of starting an inflammatory response may be the basis of the problem.
The authors refer to this as a central circuit disorder.
There is a poor prognosis for CRPS patients who have dystonia.
Finding out the underlying cause of the problem may help scientists find a way to prevent this from happening.
Complex regional pain syndrome (CRPS) I, formerly known as reflex sympathetic dystrophy (RSD), is a painful neuropathic condition that most commonly affects a traumatized extremity.
It is characterized by pain that is out of proportion to the original injury, has a distal predominance, and is not attributable to a specific peripheral nerve injury.
The name RSD has been changed to CRPS I reflecting the fact that although sympathetic dysfunction can maintain the painful state, it is not the essential pathophysiologic lesion.
Successful treatment hinges on early recognition of suspected cases, prompt referral to pain specialists, and ultimately pain control and return of limb function.
Treatments range from noninvasive medications and therapies to sympathetic ganglion blockade and sympathectomy.
The sports medicine physician is in an ideal position to recognize CRPS I in its earliest stages postinjury, and is advised to make prompt referral to a pain specialist when suspected.
Complex regional pain syndrome (CRPS) is a chronic pain condition that is believed to be the result of dysfunction in the central or peripheral nervous systems.
Typical features include dramatic changes in the color and temperature of the skin over the affected limb or body part, accompanied by intense burning pain, skin sensitivity, sweating, and swelling.
CRPS I is frequently triggered by tissue injury; the term describes all patients with the above symptoms but with no underlying nerve injury.
Patients with CRPS II experience the same symptoms but their cases are clearly associated with a nerve injury.
Older terms used to describe CRPS are "reflex sympathetic dystrophy syndrome" and "causalgia," a term first used during the Civil War to describe the intense, hot pain felt by some veterans long after their wounds had healed.
CRPS can strike at any age and affects both men and women, although most experts agree that it is more common in young women.
