Complex Regional Pain syndrome Type I (RSD) and Type II (Causalgia) is new terminology (taxonomy), developed by the International Association for the Study of Pain, World Institute of Pain and others as a more encompassing term to include:

• Reflex Sympathetic Dystrophy
• Causalgia
• Sudeck's atrophy
• Algodystrophy
• Post-traumatic Sympathetic Dystrophy
• Shoulder-hand Syndrome
• Chronic Traumatic Edema
• Post-traumatic spreading Neuralgia
• Neurovascular dystrophy
• Sympathalgia

Chronic severe pain is the main symptom, consisting characteristically, of burning, lancinating pain that is more severe than would be expected from the initiating event or the apparent clinical injury. Pain must be present for the diagnosis and it must be accompanied by painful sensations to touch or other non-painful stimuli (allodynia), or pain that is more severe than it should be from a normally painful sensation (hyperalgesia). Tremor and dyskinetic movements or weakness may be present. Also autonomic (sympathetic nervous system) abnormalities, of temperature, changes in vascularity, hair growth, skin changes and nail growth may be present but are not necessary for the diagnosis. These changes may be used in staging the severity of the disease.

Stage I: Acute, 1-3 months (edema, increased or decreased temperature, increased hair and nail growth, disuse secondary to pain which is burning and not confined to a particular nerve.
Stage II: Dystrophic, 3-7 months (progressive edema, decrease temperature, excessive perspiration, hair loss, nail rigidity and breaking, color changes [cyanosis], disuse-immobilization, weakness, tremor, spasticity.
Stage III: Atrophic, 7 months (skin dehydration, smooth glossy skin, cyanotic or pale skin, lymphedema, muscle wasting, flexion contractures, ankylosing joints, disuse-immobilization, atrophy, spasm, dyskinetic movements and tremor.
Stage IV: End stage, often combined with stage III (frozen, useless extremity, and severe depression, possible suicidal.

DIAGNOSIS: Neuropathic pain and other associated disorders frequently are difficult to diagnose, partly because doctors are not aware of the disease. Pain, particularly pain that presents in the distal part of the extremity, is important in the diagnosis of CRPS, however, the definition does allows for involvement of other regions, such as the face or trunk. CRPS Type I is a syndrome that develops after some minor injury or noxious event. Spontaneous severe pain, and pain from non-painful stimuli, or severe pain from a mildly painful stimuli, is present and is not limited to the territory of a single peripheral nerve, and is out of proportion to the injury. Depending on the stage of the disease there is evidence of edema, skin blood flow abnormality, hair and nail changes, and temperature changes in the painful area since the injury. The diagnosis of CRPS Type I is excluded by the presents of other conditions that could account for the pain. CRPS Type II is a syndrome that develops after a known nerve injury and is in all other respects like Type I.

Treatment: 1) There is no question in my mind that the main stay of management of CRPS Type I and Type II is sympathetic blockade and physical therapy. Most patients respond well to sympathetic blockade and permanent recovery is possible if the therapy is started, early in the course of the disease, before irreversible changes occur. A series of sympathetic blocks should be carried out and continued until minimal pain persists. For the upper extremity Stellate ganglion or brachial plexus blocks are effective and can be done on an out patient basis. For lower extremity lumbar sympathetic blocks and lumbar epidural sympathetic blocks are effective. My personal preference is lumbar epidural sympathetic blocks. 2) A treatment method that has been useful in my practice, particularly in late stage disease, is the IV regional block technique (Bier block). Various drugs have been used, including reserpine, guanethidine, bretylium, clonidine, nefedipine (calcium channel blockers) and vasodilators. We have had very good experience with Bier blocks using lidocaine and bretylium. 3) I advocate physical therapy and an exercise program as well as electrical nerve stimulation (TENS). 4) Oral medications used during the course of treatment, include: anti-depressants, sedatives, anti-convulsants, muscle relaxants, non-steroid anti-inflammatory agents, non-narcotic analgesics, and corticosteroids.

IMPORTANT:
Recognition of the disease in its early stages.
Early proper treatment:

• Sympathetic blockade
• Physical therapy
• Oral medications

CASE REPORT: Use of Bier Block with Bretylium to Treat a Patient With Complex  Regional Pain Syndrome Type I (Reflex Sympathetic Dystrophy)
Author: Robert V. Plehn, M.D., DABA, FACA