H. Hooshmand, M. D.

DIPLOMATE AMERICAN BOARD OF PSYCHIATRY AND NEUROLOGY

BOARD CERTIFIED IN ELECTROENCEPHOLOGRAPHY

BOARD CERTIFIED IN ELECTROMYOGRAPHY  

BOARD CERTIFIED IN AMERICAN BOARD OF ELECTODIAGNOSTIC MEDICINE

INTRACTABLE NEUROLOGY

EPILEPSY, PAIN, MS

An International Referral Center dedicated to Treatment, Education and Research

RSD PUZZLE #18
Bilateral Involvement Of Complex Regional Pain Syndrome (CRPS)                                                                             

(Revised on 2/14/2002)

INTRODUCTION

S. Weir Mitchell, M.D., the father of CRPS (RSD) diagnosis, in his book "Injuries of Nerves and Their Consequences(1872)[1]" made the following statement on the spread of RSD.

"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."

"Your Complex Regional Pain Syndrome (CRPS) has caused involvement of the brachial plexus and the hand on the right side. There is no way you can develop the same thing on the left side."

Two principle questions are brought up in this puzzle.

1. Involvement of the brachial plexus. Even though in occasional cases, brachial plexus injury can result in CRPS, practically any pathology that causes CRPS involving the hand, wrist, shoulder, or elbow area, causes constriction of blood vessels in the distribution of brachial artery branches. As a result, the patient invariably develops poor circulation to the nerves that get the supply of the blood from the brachial artery. The main nerve trunk is the brachial plexus with resultant poor oxygenation and as a result, the patient shows a clinical picture of weakness, pain, and spasm in the distribution of the brachial plexus. This is part and parcel of CRPS involving the upper extremity.

2. CRPS temperature changes are practically invariably bilateral in nature, by virtue of the fact that the temperature regulation at the spinal cord level is modulated at the central gray matter of the spinal cord. In addition, the chains of sympathetic ganglia on each side of the spine(Figure 1) is connected vertically as well as horizontally through the sympathetic plexi in the anterior aspect of the spine (such as cardiac plexus, mesenteric plexus, etc.) [2]. However, clinically it becomes obvious in about 1/5 of patients usually the manifestation is far more prominent on one side. This phenomenon[3-16] has been noted in animal experiments when the animal sustains injury to the right front paw and then the left front paw, the right and left back paw also manifest inflammation and the pathologic changes typical of CRPS.

FIGURE. 1
From: Chronic Pain: Reflex Sympathetic Dystrophy- Prevention and Management  (CRC Press, 1993)
H. Hooshmand, M.D.

Temperature changes have been identified in CRPS by Kurvers, et al [8,9]. Three stages of acute (warm), subacute (warm and cold), and chronic (cold) CRPS. They concluded that at spinal cord level there is a mirror imaging [15,16] antidromic vasodilation of bilateral nature.

The same phenomenon of bilateral[3-17] involvement causes confusion in regard to diagnosis with bone scan test. The bone scan test is abnormal somewhere between 55-65% of CRPS patients just because the disease involves both sides and the bone scan cannot discriminate the abnormality between one versus the other side. The same is also a problem in thermography tests and thermography tests frequently show the CRPS hypothermia being on both sides rather than on one side.

The main reason for the CRPS becoming bilateral and spreading to other extremities is because in contrast to the somatic nervous system, the sympathetic nervous system has bilateral innervation. In the somatic nervous system (usual sensation and motor function) the abnormalities in dermatome in a specific nerve root distribution, whereas in CRPS the abnormality is distributed among the blood vessels, distribution of nerves (thermatomes) and to the sympathetic ganglia and their across the midline collections, the condition reflects itself on both sides rather than one side of the body. This bilateral manifestation through the sympathetic plexi across the midline explains the patient's problem with headache, dizziness, tinnitus, chest pain, and abdominal manifestations of CRPS (gastritis, diarrhea, cramps) and spread of CRPS to other extremities.

In treating CRPS, even if the opposite extremity looks normal, the treatment should be given to both extremities because of this principle of bilateral innervation. The enclosed graph shows the bilateral innervation of sympathetic nervous system.

H. Hooshmand, M.D.