HISTORY of RSD/CRPS

"Perhaps few persons who are not physicians can realize the influence of which long-continued and unendurable pain pain can have upon both body and mind".

Silas Weir Mitchell "Nerve Injuries" 1864.

The first descriptions of CRPS were documented during the America Civil War (1861-65) by Silas Weir Mitchell MD, a young US Army contract physician, who treated soldiers with gunshot wounds. In his book "Gunshots Wounds and Other Injuries", he described pain which persisted long after the bullets were removed from the bodies of soldiers. He noted that the pain was characteristically of a burning nature, and named it "causalgia"(Greek for burning pain)which he attributed to the consequences of nerve injury. He observes:

"...a painful swelling of the joints....may attack any or all articulations of a member. It is distinct from the early swelling due to the inflammation about the wound itself, although it may be masked by it for a time:nor is it merely a part of the general edema....Once fully established, it keeps the joint stiff and sore for weeks or months. When the acute stage has departed, the tissues become hard and partial anklyosis results.."

Mitchell et al 1864

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The following is excerpted from a story which appeared in the Johns Hopkins Medical Institutions' publication, Brainwaves, and was written by Janet Worthington. It's an account of causalgia, a type of neuropathic pain, as described by Dr Mitchell:

"Under such torments, the temper changes, the most amiable grow irritable, the soldier becomes a coward, and the strongest man is scarcely less nervous than the most hysterical girl."

S. Weir Mitchell, M.D., a 19th century neurologist, was as perplexed by the phenomenon of sympathetically maintained pain (he called it causalgia), as his modern counterparts. In his 1872 book, "Injuries of Nerves and Their Consequences," he carefully documented case after case in which injuries resulted in:

"the most terrible of all the tortures which a nerve wound may inflict."

Most of his patients were Civil War veterans, otherwise healthy men whose lives had been forever changed by this peculiar, burning pain, described by one as a

"red-hot file rasping the skin."

In many, pain was associated with a mysterious glossiness in the skin.

"The burning comes first, the visible skin-change afterwards,"

Mitchell reported.

"Of the special cause which provokes it, we know nothing, except that it has sometimes followed the transfer of pathological changes from a wounded
nerve to unwounded nerves, and has then been felt in their distribution, so that we do not need a direct wound to bring it about."

 

The pain's location varied from patient to patient, but:

"its favorite site is the foot or hand...the palm of the hand or palmar face of the fingers, and on the dorsum of the foot; scarcely ever on the sole of the foot or the back of the hand. When it first existed in the whole foot or hand, it always remained last in the parts referred to...if it lasted long it was finally referred to the skin alone. The part itself is not alone subject to an intense burning sensation, but becomes exquisitely hyperaesthetic, so that a touch or a tap of the finger increases the pain."

Patients took obsessive lengths to avoid exposing the area to the air, Mitchell wrote.

"Most of the bad cases keep the hand constantly wet, finding relief in the moisture rather than in the coolness of the application."

The pain took its toll.

 

"As the pain increases, the general sympathy becomes more marked. The temper changes and grows irritable, the face becomes anxious, and has a look of weariness and suffering. The sleep is restless, and the constitutional condition, reacting on the wounded limb, exasperates the hyperaesthetic state, so that the rattling of a newspaper, a breath of air...the vibrations caused by a military band, or the shock of the feet in walking, gives rise to increase of pain. At last...the patient walks carefully, carries the limb with the sound hand, is tremulous, nervous, and has all kinds of expedients for lessening his pain."

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Another military surgeon, Rene Leriche MD, (1879-1955), treated many WW1 soldiers who also had nerve damage. He documented the classic signs of CRPS and tried to alleviate the pain with a sympathectomy:

"A few months previously I had unexpectedly encountered one of these cases. I was struck by the resemblance which the condition had to that of a sympathetic disorder; the cyanosis, the sweating, the paroxysmal nature of the pains, the effect on the general mental state, the reference of painful phenomena to a distance---all pointed in that direction. And, remembering that the sympathetic, in its distribution to the limbs, follows the course of the arteries, I asked myself whether, in those cases of nerve injury complicated by arterial wounds, it was not the injury to the sheath of the vessel that determined their painful and trophic features---the wound of the sympathetic---... Starting from this point, I asked myself whether, by acting upon the perivascular sympathetic, I might be able to succeed in modifying the causalgia."

He goes on to discuss a case study:

"I saw the patient on the 20th June;the upper limb was completely paralyzed---arm, forearm, hand and fingers....dominating everything, was an intense burning pain, concentrated particularly in the palm of the hand and on the pulp of the fingertips....On the 27th August, I exposed the brachial artery, which I found small and contracted. I removed its adventitia for a distance of 12 cm....By the next day it was obvious that the patient had less pain."

In conclusion, he thought that "novicain infiltrations of the paravertebral sympathetic chain" was an effective treatment for causalgia. He was tormented by the pain suffered by the brave soldiers and in 1937 he wrote "La Chirurgie de la Douleur" (Surgery of Pain) documenting his experiences.

During WWII, William K. Livingston MD, (1892-1966),a military doctor, was working at Oakland Naval Hospital where he treated peripheral nerve injures of soldiers who had chronic pain. He wrote about the "vicious circle of pain as similar with vasoconstriction and atrophy". He compared this pain to "circus movements in the heart muscle." He also talked about "mirror images" of pain or sympathetic pain in which the limb contra lateral to the injury becomes sympathetic. Modern research has found interneuron connections that not only ascend and descend the pain pathways but result in abnormal neurotransmission also to the contralateral side.

Here follows a soldier's narrative:

"It is as if a rough bar of iron were thrust to and fro through the knuckles, a red, hot iron placed at the junction of the palm, and then an eminence with a heavy weight on it and the skin was being rasped off of my finger ends".

Livingston 1947

Moreover, Dr Livingston suggests that the concept of receptor specificity with only four methods of cutaneous sensitivity (touch, pain, heat,cold) is too inadequate to explain phantom limb pain or CRPS and pain syndromes. Pain sensations are governed by higher cortical centers in the brain and emotional factors. He described all pain as psychic perception with a marked psychological component. This idea has formed the basis of chronic pain as the multi-faceted process we are familiar with now.

All three of these physicians contributed a great deal to what we know about causalgia (CRPS-2) and RSD (CRPS-1) today.

GERMAN SCHOOL

In 1900, Sudeck, a surgeon from Hamburg, discovered a new twist: "acute patchy osteoporosis" as a complication of infection in limbs. The radiographic changes started as "patchy osteoporosis of the small bones of the hand or feet and the distal metaphysis of forearm or tibial bones".Eventually the osteoporosis becomes diffuse. Hence the name "Sudeck's atrophy", due to the patchy osteoporosis findings.

Another German doctor, Keinbock confirmed the osteoporosis findings and also reported "acute bone atrophy and atrophy due to inactivity". In 1936, one of Sudeck's students, Reider, suggested that the disease be named "limb dystrophy" due to the bone tissues breaking down. The term "reflex" originated with the observations that the syndrome was caused and maintained by a reflex , travelling through the nervous system.

Sudeck proposed that RSD could be caused by an "exaggerated inflammatory response after injury or operation of an extremity". The signs and symptoms of acute inflammation were rubor, calor, dolor, tumor and functio laesa. This theory forms the basis of modern Dutch research and treatment; that RSD begins as inflammation.

FRENCH SCHOOL

In 1890, Charcot observed the disease as "non-pitting edema, changes in color, changes in skin temperature, tenderness of the skin and pain". Charcot thought the cause was self suggestion. Leriche, a neurosurgeon, introduced the name "algodystrophie". He thought it was caused by an increase of activity in the sympathetic nervous system and introduced "surgical sympathectomy" as a treatment. This soon became a popular treatment for CRPS.

AMERICA AFTER WW II

In 1947, Steinbrocker renamed the disease "shoulder-hand syndrome". He also began using oral corticosteriods as treatment in 1953. Meanwhile, Serre in France and Kozin in USA, used nuclear scans e.g. bone scans using technetium labelled methylenediphosphonate and found diffuse disturbances in peri-articular areas. Later Kozin implemented the three phase nuclear scan which is still used today. Hannington-Kiff began using the intravenous regional blockade of the sympathetic nervous system with guanethidine in 1974. This treatment is still used today along with physical therapy.

NAMES FOR RSD/CRPS

Following these and other developments, the confusion over the names of this syndrome increased. Algodystrophie was used primarily in France, Sudeck's atrophy referred to the osteoporosis part of the disease. When the disease began as a result of nerve injury, it was called "causalgia" after Mitchell. If it began with non-nerve injuries, it could be called "mimocausalgia" or "minor causalgia." "Posttraumatic dystrophy" (Dutch term) referred to the specific event which caused RSD.

Other names surfaced in the literature such as "vasospastic", "neurotrophic" "neurovascular" or "reflex". The most common was "sympathetic". Roberts introduced the name "sympathetically maintained pain" in 1986 and it has become popular with pain researchers. This term is a condition where pain and hyperalgesia are relieved with a blockade.

Some common names in the literature for RSD/CRPS are:

In 1995, Michael Stanton-Hicks and a committee of RSD researchers decided to give RSD (Reflex Sympathetic Dystrophy) a new name:
Complex Regional Pain Syndrome. Why? If one name was decided upon, then further research could be done and confusion would be eliminated.

Now we have CRPS type 1 which is "RSD" and CRPS type 2 which is "causalgia." What is the difference? Both have the same exact symptoms however, RSD has no identifiable cause and causalgia is due to nerve injury. This should have eliminated the confusion but now patients with this disease still call it "RSD" and the medical community refers to it as "CRPS". Both names are used interchangeably.

Source: Am Soc Anesth. Newsletter October 2002 page 11-13

Source: Veldman PHJM MD Clinical Aspects of RSD Thesis Nijmegen CIP-Data Royal Dutch Library, The Hague ISBN 90-9007712-X