Reflex sympathetic dystrophy (RSD) is a form of a complex regional pain syndrome that affects the extremities. It is characterized primarily by pain, swelling and vasomotor changes.
Presentation
Reflex sympathetic dystrophy may occur in either the upper or lower limbs. The clinical course moves in stages:
- Stage 1: The patients develops pain with an identifiable cause (it may not be found or apparent). There is throbbing pain, abnormal sensations like burning and allodynia with localized edema. The distribution of the pain is not compatible with a nerve region. There will be vasomotor disturbances with changes in temperature and skin colour. There may be radiological changes but they are minimal at this stage.
- Stage 2: In this stage there is progression of soft tissue edema and skin thickening with associated muscle wasting. This occurs from 3 to 6 months after onset.
- Stage 3: The third stage is characterized by limitation of movement, with severe contractures and trophic skin. The radiograph shows significant demineralization [8].
Entire Body System
- Pain
Introduction Reflex sympathetic dystrophy (RSD) is a pain syndrome in which the pain is accompanied by loss of function and evidence of autonomic dysfunction (Amadio et al ., 1991). [oandplibrary.org]
Abstract Reflex sympathetic dystrophy is usually referred to as causalgia because of its chief symptom—an agonizing burning pain. [annals.org]
Sayantika Dhar Hand & Orthopedic Rehabilitation services Pain profilePAIN QUALIFIERS JOINT PAIN Aching 0= no pain Burning 1= mild pain with deep palpation Cramping Heaviness/fatigue 2= severe pain with deep palpation Numbness 3= severe pain with [slideshare.net]
Definition of reflex sympathetic dystrophy : a painful disorder that usually follows a localized injury, that is marked by burning pain, swelling, and motor and sensory disturbances especially of an extremity, and that is often considered a type of complex [merriam-webster.com]
While RSD is traditionally associated with pain in the extremities, the possibility is raised that the same process may underlie chronic pain syndromes affecting more central structures, such as testicular or pelvic pain. [ncbi.nlm.nih.gov]
- Swelling
Despite the intense pain, swelling, and dermatologic changes that followed, the diagnosis of RSD was not considered until scintigraphic studies suggested it. [ncbi.nlm.nih.gov]
The former term is preferable, however, because in many patients with reflex sympathetic dystrophy the distinguishing features are not pain but other manifestations of sympathetic stimulation, namely, rubor, pallor, or both, swelling, sweating and often [annals.org]
Definition of reflex sympathetic dystrophy : a painful disorder that usually follows a localized injury, that is marked by burning pain, swelling, and motor and sensory disturbances especially of an extremity, and that is often considered a type of complex [merriam-webster.com]
Title: shoulder-hand syndrome Learn about this topic in these articles: major reference In joint disease: Reflex sympathetic dystrophy Reflex sympathetic dystrophy —also called shoulder-hand syndrome because pain in the shoulder is associated with pain, swelling [britannica.com]
It is characterized primarily by pain, swelling and vasomotor changes. Reflex sympathetic dystrophy may occur in either the upper or lower limbs. [symptoma.com]
- Burning Pain
Abstract Reflex sympathetic dystrophy is usually referred to as causalgia because of its chief symptom—an agonizing burning pain. [annals.org]
Definition of reflex sympathetic dystrophy : a painful disorder that usually follows a localized injury, that is marked by burning pain, swelling, and motor and sensory disturbances especially of an extremity, and that is often considered a type of complex [merriam-webster.com]
Reflex sympathetic dystrophy (RSD) is characterized mostly by: (burning) pain, restricted range of motion, oedema and autonomic disturbances. [ncbi.nlm.nih.gov]
- Disability
Your Reflex Sympathetic Dystrophy Disability Case If you are disabled because of Reflex Sympathetic Dystrophy that is so severe it prevents you from working, you may well be entitled to Social Security Disability benefits. [disability-benefits-help.org]
Click here to begin your application for disability benefits. [premierdisability.com]
The SSA has now defined RSDS/CRPS as a Medically Determinable Impairment which can be used a basis of finding of disabled for SSA disability benefits. [smithdickeydempster.com]
Can I Get Disability for My RSD? Although a diagnosis of RSD alone will not qualify you for disability, you can still win approval if the Social Security Administration (SSA) finds that your RSD prevents you from working. [disabilitysecrets.com]
- Weakness
– Motor changes, including decreased ROM and weakness and movement disorders (tremor, dystonia and neglect) 25. [slideshare.net]
Common symptoms: Pain that can be moderate to severe Neuropsychological deficits Constitutional symptoms (lethargy, tiredness, weakness) Respiratory complications (dystonia in chest wall muscles) Endocrine system dysfunction (low baseline cortisol levels [drvictorpedro.com]
Specifically, you may experience RSD in your: hands fingers arms shoulders legs hips knees Symptoms include: stiffness discomfort pain or burning sensation swelling sensitivity to heat or cold weakness feeling warm to the touch skin redness skin paleness [healthline.com]
A person with RSD often has overgrown and grooved nails, muscle weakness and atrophy, and swollen, stiff joints. A neurological exam is usually called for to look for severe pain as a response to a mild stimulus. [disability-benefits-help.org]
A good sympathetic block should increase the temperature of the extremity without producing increased numbness or weakness. [amputee-coalition.org]
Cardiovascular
- Hypertension
[…] thought to relate to strands of amniotic membrane enwrapping the developing limb Cushing's syndrome raised blood cortisol (e.g. due to pituitary tumour; long-term steroid therapy); characterized by central obesity, moon-like facies, acne, skin striae, hypertension [medical-dictionary.thefreedictionary.com]
- Tachycardia
[…] encephalopathy brainstem ischaemia causing nystagmus and other ocular effects, tremors and ataxia, mental confusion, hypothermia and hypotension; more common in chronic alcoholics Wolff–Parkinson–White syndrome congenital atrioventricular interconnection causing tachycardia [medical-dictionary.thefreedictionary.com]
Musculoskeletal
- Osteopenia
Radiographs of her left knee showed severe periprosthetic osteopenia and a triple phase bone scan was negative. Clinically, reflex sympathetic dystrophy was considered likely and a lumbar sympathetic block was performed. [ncbi.nlm.nih.gov]
An X-ray of the right foot showed peri-articular osteopenia. A diagnosis of reflex sympathetic dystrophy syndrome was made. The patient markedly improved on conservative treatment. [med.harvard.edu]
- Swelling of Hand
(Photo provided by Jenny Loder) One symptom of RSD is swelling of hands and feet. Shown is Jenny Loder's left hand during an RSD flair up. When Jenny Loder had surgery five years ago, she didn't realize how much it would change her life. [nj.com]
- Arthralgia
[…] for >3/12 in four or more joints Minor criteria Current/historic Brighton score of 1, 2 or 3/9 (0, 1, 2, 3/9 if >50 years old) Arthralgia for minimum of 3 months in 1–3 joints, or back pain for minimum of 3 months, or spondylosis/spondylolysis/spondylolisthesis [medical-dictionary.thefreedictionary.com]
- Flexion Contracture
Persists for several months Atrophic phase Irreversible Pain diminishes or becomes intractable Skin and subcutaneous tissues become atrophic Flexion contractures in foot become fixed Osteoporosis becomes advanced; bone has a ‘ground-glass’ appearance [medical-dictionary.thefreedictionary.com]
Skin
- Abnormal Sweating
Weir Mitchell first described "causalgia" following wartime nerve injury, with its persistent distal limb burning pain, swelling, and abnormal skin color, temperature, and sweating. [ncbi.nlm.nih.gov]
The symptoms of Type II are similar to Type I and include changes in temperature near the site of the injury, abnormal sweating, and changes in blood flow, which affects skin coloration. [ddjlawtampa.com]
There is pain and abnormal sensation in a certain area with abnormal sweating and swelling. Cause: The cause is unknown, but it is thought that inciting events such as trauma and surgery may be the cause. In many people no cause is found. [symptoma.com]
Reflex Sympathetic Dystrophy (RSD) is a chronic pain disorder associated with sensitivity to light touch swelling, skin color and temperature changes, weakness, abnormal sweating, muscle atrophy, and subsequent depression. [bethesdahbot.com]
He will then note if the continuing pain you are suffering is disproportionate to the injury suffered (if there was one) and will determine whether there is swelling or excessive accumulation of fluid in your tissues (edema) or abnormal sweat gland activity [disability-benefits-help.org]
- Flushing
Dystrophic (three to six months): early skin changes of shiny, thickened skin and contracture with persistent pain, but diminished swelling and flushing. [medicinenet.com]
In the second stage, a person may have early skin changes of shiny, thickened skin with persistent pain, but diminished swelling and flushing. [disability-benefits-help.org]
- Hypertrichosis
Clinical findings Dysaesthesia, pain, swelling of an extremity with trophic skin changes, hypertrichosis, osteoporosis. Aetiology Acute MI, cervical osteoporosis, CVAs, nerve injury, neurologic events, surgery, trauma. [medical-dictionary.thefreedictionary.com]
Face, Head & Neck
- Facial Pain
The pain relief from these injections can dramatic. Procedures that focus on facial pain and upper extremity RSD include the Stellate Ganglion Block. Lumbar Sympathetic Blocks are typically performed for lower extremity RSD. [ainsworthinstitute.com]
Neurologic
- Stroke
DESIGN: Blind clinical assessment of 2 groups of stroke patients defined on the basis of absent or preserved motor evoked potentials (MEPs) on the affected side. SETTING: Stroke rehabilitation center. [ncbi.nlm.nih.gov]
Prevention: Quick physical therapy after an injury or stroke is advised to reduce the occurrence of the disease. [symptoma.com]
People who’ve had a stroke should be mobilized soon afterward to avoid developing RSD. If you’re taking care of a loved one with a stroke, help them get up and walking around. This movement may also be useful to people who’ve had heart attacks. [healthline.com]
- Tremor
Movement disorders Patients may feel a stiffness and pain when moving, and may have muscle tremors. Severe muscle spasms can leave hands or feet disabled in cramped up positions. [stanfordhealthcare.org]
[…] intolerable to many RSD patients. null Play null Now the other thing that happens is that there can be swelling, temperature change, and in addition to that, one can develop a disorder of the system that moves the body so that we can get atrophy or we can get tremor [abcnews.go.com]
Tremors and involuntary jerking of extremities may also be present. Contraction of the hands and feet can occur over time in some patients. Over time, pain and symptoms may become more diffuse. [docneuro.com]
[…] sweaty and cold Changes in skin color, ranging from white and mottled to red or blue Changes in skin texture, which may become tender, thin or shiny in the affected area Changes in hair and nail growth Joint stiffness, swelling and damage Muscle spasms, tremors [mayoclinic.org]
Other symptoms include muscle spasms or tremors, increased sensitivity to even light touch, atrophy, increased sweating, color and/or temperature changes, altered hair and nail growth. [drgetson.com]
- Dystonia
Discussion.- Tonic dystonia is an underrecognized complication of RSDS that often develops after a minor injury yet causes prolonged pain and disability. Spread of the dystonia to other sites is not infrequent. [ncbi.nlm.nih.gov]
- Hyperalgesia
The serum concentration of noradrenaline was lower on the painful side than on the healthy side, and neurotropin, which has an antinociceptive effect to hyperalgesia, was clearly effective. [ncbi.nlm.nih.gov]
Interaction of Hyperalgesia and Sensory Loss in Complex Regional Pain Syndrome Type I. July 2008, Volume 3, Issue 7, e2742. www.plosone.org. [slideshare.net]
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 [thebridgerecoverycenter.com]
- Neuralgia
We propose that persistent RSD/CRPS-I is a post-traumatic neuralgia associated with distal degeneration of small-diameter peripheral axons. [ncbi.nlm.nih.gov]
[…] and other Cranial Neuralgias (3 Viewing) For discussion of Occipital Neuralgia, Glossopharyngeal Neuralgia, Nervus Intermedius (or Geniculate Neuralgia), and Vegal and Superior Laryngeal Neuralgia. [neurotalk.psychcentral.com]
Noradrenaline-evoked pain in neuralgia. Pain. 1995; 63:11-20. [10] Maihofner CM, Handwerker HOM, Neundorfer BM, et al.: Patterns of cortical reorganization in complex regional pain syndrome. [ainsworthinstitute.com]
In addition to causalgia, RSD is also known as Sudeck’s atrophy, post-traumatic neuralgia, and shoulder-hand syndrome. All of these names are used interchangeably, adding to the confusion in diagnosing and treating RSD. [amputee-coalition.org]
Workup
There is no useful laboratory testing in this syndrome. Majority of the diagnosis will come from the history and physical exam. Plain radiographs will show pronounced demineralization of the skeleton affected by the pain and edema. This worsens as the disease progresses.
Bone radionuclide test may be useful in early disease, but findings are not specific for this condition. Magnetic resonance imaging (MRI) may be useful in identifying patients, as it gives useful information on the soft tissue and skin changes. CT scans may show multiple focal areas of osteoporosis.
Autonomic testing like resting skin temperature and resting sweat output may be done to document the vasomotor symptoms. An increased resting skin temperature is highly suggestive of this disease [9].
Treatment
There are two major therapies being used currently. There is insufficient data on other effective therapies and little randomized control studies.
- Sympathetic blocks: A local anesthetic such as lidocaine or bupivacaine is injected into the stellate and upper dorsal sympathetic ganglia to block the efferent nerves. There should be a Horner's syndrome induced after this, which will disappear soon after (30 mins). The patient then undergoes intensive physiotherapy and occupational therapy. The patient may require 1 to 2 blocks per week until the condition is treated. For lower limbs a lumbar block is used [11].
- Sympatholytic drugs in early disease may be effective. Regional infusions are usually effective [12] [13].
Other treatments such as non steroidal anti-inflammatory drugs (NSAIDs) may be used. Corticosteroids are highly effective in reducing pain and allowing for physical therapy. Surgical methods may be used and include thoracic or lumber sympathectomy. This is indicated in cases resistant to therapy. Surgery should be considered before there is significant joint deformity. Chemical sympathectomy with alcohol has also been done.
Prognosis
The course of the disease is variable, but if caught early and treated appropriately, many patients do not progress on to the later stages of the disease. There is little data on mortality, but morbidity may be significant.
Etiology
Reflex sympathetic dystrophy usually occurs after injury, which can be traumatic or postsurgical. In some cases there may be no identifiable incident. In some patients an event such as myocardial ischemia or a hemiplegic stroke, may be the inciting event. Common surgical procedures such as arthroscopy of the knee and other joints may be the cause in some patients. There also have been cases in diseases such as pancreatic and pancoast tumors [2] [3].
Epidemiology
35% of patients have no identifiable inciting event. There is an estimated 5% prevalence in people with a previous upper extremity injury. The syndrome was prevalent (12%) in patients with hemiplegic stroke, but this has significantly decreased with the advent of early mobilization. There is no gender or race predilection, but it has a peak incidence between 30 to 60 years [4].
Pathophysiology
There appears to be a need of three conditions to be present for reflex sympathetic dystrophy to occur:
- Abnormal sympathetic reflex
- Persistent painful lesion
- Predisposition
The true mechanism of this complex regional pain syndrome is unclear, but appears to involve the formation of a new reflex arc after the inciting event. The arc follows a sympathetic pathway and is then modulated by cortical centres and produces vascular changes. The pain occurs possibly due to the triggering event causing nerve damage with increased sensitivity to sympathetic neurotransmitters such as epinephrine.
There is thought to be prolonged and constant release of pain inciting peptides from peripheral nerves causing nerve inflammation with subsequent pain and allodynia. Neuropeptide Y and substance P have been implicated in this. There appears to be a genetic predisposition. A few human leukocyte antigen (HLA) associations have been found, a few examples include HAL-A3, and DR2 [5] [6].
Prevention
Summary
There are two types of a complex regional pain syndrome (CRPS):
- Type 1, when the complex regional pain syndrome is not caused by an identifiable nerve lesion. Names include reflex sympathetic dystrophy, Sudeck's atrophy and reflex neurovascular dystrophy.
- Type 2, when there is an identifiable lesion. It was previously known as causalgia [1].
Both types are chronic systemic diseases characterized by severe pain, swelling, and vasomotor dysfunction.
Patient Information
- Definition: Reflex sympathetic dystrophy is a disease whereby there is a problem with a certain area of your body, usually a hand or leg. There is pain and abnormal sensation in a certain area with abnormal sweating and swelling.
- Cause: The cause is unknown, but it is thought that inciting events such as trauma and surgery may be the cause. In many people no cause is found. The triggering event causes there to be abnormal sensation in the affected area.
- Symptoms: The most common symptom is pain which can be burning or tingling. Some complain of throbbing pain which can also be severe. The affected area is sensitive to touch or cold, there is swelling and the skin become thick and rough. The muscles in the area become weak and smaller.
- Diagnosis: Most of the diagnosis is made from asking you questions and examining you. They skin temperature and the amount of sweat in the area will be recorded.
- Treatment: The treatment involves blocking the nerves and physical therapy. You may also be given drugs to help with the pain and swelling.
- Prevention: Quick physical therapy after an injury or stroke is advised to reduce the occurrence of the disease.
References
- Stanton-Hicks M, Jänig W, Hassenbusch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995; 63:127.
- Pak TJ, Martin GM, Magness JL, Kavanaugh GJ. Reflex sympathetic dystrophy. Review of 140 cases. Minn Med 1970; 53:507.
- Sebastin SJ. Complex regional pain syndrome. Indian J Plast Surg. May 2011;44(2):298-307
- Goebel A. Complex regional pain syndrome in adults. Rheumatology (Oxford). Oct 2011;50(10):1739-50
- van Laere M, Claessens M. The treatment of reflex sympathetic dystrophy syndrome: current concepts. Acta Orthop Belg 1992; 58 Suppl 1:259.
- Kozin F. Reflex sympathetic dystrophy syndrome. Bull Rheum Dis 1986; 36:1.
- O'Brien SJ, Ngeow J, Gibney MA, et al. Reflex sympathetic dystrophy of the knee. Causes, diagnosis, and treatment. Am J Sports Med 1995; 23:655.
- Bonica JJ. Causalgia and other reflex sympathetic dystrophies. Postgrad Med 1973; 53:143.
- Todorović-Tirnanić M, Obradović V, Han R, et al. Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy? Eur J Nucl Med 1995; 22:1187.
- Cappello ZJ, Kasdan ML, Louis DS. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am 2012; 37:288.
- Cepeda MS, Carr DB, Lau J. Local anesthetic sympathetic blockade for complex regional pain syndrome. Cochrane Database Syst Rev 2005
- Perez RS, Kwakkel G, Zuurmond WW, de Lange JJ. Treatment of reflex sympathetic dystrophy (CRPS type 1): a research synthesis of 21 randomized clinical trials. J Pain Symptom Manage 2001; 21:511.
- Olcott C 4th, Eltherington LG, Wilcosky BR, et al. Reflex sympathetic dystrophy--the surgeon's role in management. J Vasc Surg 1991; 14:488.