Clinical Case Report

ReflexSympathetic Dys,rophy:A CaseofTotalBodyPain

Van Chen,NP

JohnKelly,MD

Reflexsympathetic dystrophy (RSD),also known as complex regional painsyndrome type I (CRPS 1), is a chroniccondition characterized by burning

and aching pain; hyPeresthesia andallesthesia; motor disturbance and soft

clear, studies have shown that RSD is

associated with many different med-ical conditions including traum".,

drugs, and unidentified pr~cipitatingfactors.3 Previously reported RSD pa-tients had localized pain or pain in-volving only limited areas of thebody.4 Systemic cases are rare. The fol-lowing report examines a patient with

During the examination, the response to mild sensory

stimuli may produce severe pain.

tissue change; vasomotor and auto-nomic changes; and psychosocial dis-

turbance.] Early signs and symptomsof the disease usually include inflam-

matory signs in the affected limb andmuscular paresis with easy fatigue.2

RSD can be difficult to diagnose

and often requires excluding otherconditions that produce similar symp-toms. A thorough history and neuro-logical examination is important for

accurate identification. During the ex-amination, the practitioner may no-tice that response to mild sensorystimuli produces severe pain. Physical

examination involves observing theskin color and temperature; swelling

and vascular reactivity; overgrownand grooved nails; swollen and stiff

joints; and muscle weakness and atro-phy. Other conditions are ruled outwith appropriate testing that may in-clude a magnetic resonance imaging

scan, a full laboratory panel, and elec-trophysiological studies of the nervesand muscles.

While the etiology of RSD is not

8 TheNursePractitioner' Vol.32, No.9

global pain that affected all of her ex-tremities and had multiple medicalcomplications associated with RSD.

II PatientCharacteristics

The patient, a 52-year-old African-Am~rican woman, was diagnosedwith RSD in 1992. She had several

medical conditions before her diagno-sis throughout a specific stretch oftime, which may have predisposed herto RSD. These included:. a long history of pain in her back

and legs since 1973,managed with avariety of indicated regimens.a fracture of the sacral spine in 1973at agescoliosis, cervical, and lumbar

spinal stenosis diagnosed in 1973. arachnoiditis since 1974 from com-

plications of several myelograms.laminectomies from L4 to Sl forherniated discs in 1975

. "'fa.cetdenervations by ablation and"'w,>epidlll.;jUinjections in the lumbarand cervical spine for pain treat-ment in 1976

.. Harrington rod fusion, T8 throughsacrum, for scoliosis and spinal in-

stability in 1978\ ,

'''Harrington rod removal in 1980. restrictive lung disease and respira-

tory insufficiency since her late 20s,diagnosed in 1983

. galactorrhea-amenorrhea syn-drome between early 1992 and1993.

In 1992, she began to experience

burning pain, excessivehair growthcompared to the opposite extremity,and swelling in the left arm, which

were considered early signs and symp-toms of RSD.She was diagnosed withbrachial plexitis and treated withphysical therapy that includedacupuncture, desensitization proce-

dures, warm compresses, and range ofmotion exercises.The treatments were

ineffective, and the patients' symp-toms advanced to her right upper ex-tremity and bilateral lower extremitiesover a period of 1 month. Later, thepain spread to her torso, including herback, abdomen, and chest.

The pain was described as a deep

aching and burning sensation. She ex-

perienced hyper§,t;l1sitivitywithi"''''marked allesthesia of the skin in local

areas. She also suffered tremors, my-

oclonic jerks, and mood changes that

included anxiety and depression. De-spite intensive treatment, her pain syn-

drome became chronic. She developedmuscle atrophy and contractures ofthe extremities, especially at the fourthand fifth fingers of her hands. She hasbeen unable to walk since 1993.

Continuous abdominal pain oc-curred, along with ileus, which re-

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quired total colectomy with ileorectal

anastomosis in 2002. Dumping syn-drome emerged soon afterwards.Since the total colectomy, she devel-oped partial ileus of the small bowel,

and menopause began in 2000. Fur-thermore, the patient has a history ofangina and suffered a myocardial in-farction (MI) in 1996.

At present, the patient still hasconstant, generalized pain, chronic

ileus, muscle spasms, and atrophy ofall four extremities. She uses an elec-

tric wheelchair for mobility and haslived in a nursing home since 1993.

II DiseaseManagementCurrently, the patient's pain is reason-

ably controlled with a self report ofpain between 4 and 6 out of 10. Pain

is treated utilizing a multimodal ap-proach, which includes morphine(Avinza), gabapentin (Neurontin),prednisone, oxcarbazepine (Trileptal),

and lidocaine via patch. Various medi-cines are used for other conditions

(see Table:"Medical Conditions andAssociated Medications Used"). Treat-

ments used in the past included other

analgesics, tricyclic antidepressants,sympathetic blocks, desensitization

techniques for pain, and therapies for

preventing muscular atrophy or con-tracture.

In 1993,the patient underwentmultiple sympathetic nerve blocks forboth upper and lower extremities and

surgical sympathectomies for upperextremities without significantchange. Her pain was reported as con-stant, with a self-reported severity av-eraging 8 to 10 out of 10 prior to hercurrent treatment regimen.

II Multiple ComplicationsRSD is a rare syndrome that is diffi-

cultto diagnose and is often initiallymissed by practitioners not familiarwith it. Its incidence is unknown both

in the United States and internation-

ally.The reported prevalence of RSD

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is 1% to 2% after various fractures,

2% to 5% after peripheral nerve in-

jury, and 7% to 35% in prospectivestudies of Colles fracture. The likeli-

hood of developing RSD is higher ifthe lesion is distal or the nerves areaffected.

RSD affects all races, with no

racial predilection, and both sexes(the female to male ratio is 2:1). The

mean age ofRSD patients is approxi-mately 40 to 42 years. The highest in-

cidence of the disease is in adults aged40 to 49 years. RSD has been foundoccasionally in children, but its inci-dence is much lower than in adults.

This patient is consistent with thosestatistics.s She has all the common

manifestations of RSD,including se-vere pain, altered sensation, motor

disturbance (spasm, restriction), vaso-motor or autonomic changes (skincolor, temperature, swelling andedema, and sweating or trophic

changes), and altered psychosocialfunctions.

As with other reported cases, thecourse of this patient's disease in-

cluded three stages: 1) acute or hyper-emic; 2) ischemic; and 3) atrophic.Although pain in the patient's back

and neck began in 1973 following thefracture of her sacral spine, definite di-agnosis was not made until 1992,

when she began to experience burning

Clinical Case Report

pain, hair growth, and swelling in theleft arm. The key differential diagnosisof this case is brachial plexitis,which

was made prior to RSD diagnosis.Her total body pain, however, is

unique, as are the multiple severecomplications resulting from her con-dition. While diagnostic criteria forRSD are mainly focused on many dif-ferent aspects of localized sensory andautonomic features,3 this patientmanifests pain and motor dysfunction

involvingthe entirebodyexceptthe ":head and face.A Medline search from

1994 showed only three cases with in-volvement of more than nvo limbs,

and only one other case involved the

whole body.4Additionally, investiga-tors searched PubMed, which covers

the remaining years after this litera-

ture review, and found no reportedcases involving the whole body. This ~case expands the diagnostic criteria

for CRPS I and challenges the validityof the term "complex regional painsyndrome."6 While indeed complex,RSD is not necessarily localized.Moreover, in this particular patient,

RSD involved the entire body and hadconsequences for many systems.While a causal relationship between

RSD and her varying complaints can-not be proven, they occurred after the

confirmative diagnosis in a gradualmanner with no additional explana-

The Nurse Practitioner' September 2007 9

Clinical Case Report

tion. These nontypical RSD complica-tions are believed to include: chronic

intestinal pseudo-obstruction (result-ing in removal oflarge intestine) andileus of the small bowel, restrictive

lung disease with respiratory insuffi-

ciency; angina, MI, transient ischemicattack, hypertension, hypothyroidism,multiple seborrheic cysts on the back,

and occasional bouts of anxiety anddepression, which greatly improved

with better pain control.The pain associated with RSD is

difficult to manage, but can generallybe controlled at a tolerable level with

multiple drugs and other therapies.At present, the patient is able to at-

tend social activities, including con-certs and church with the use of an

electric wheelchair. She can sleep

fairly well. Because there is no uni-

form treatment protocol for RSD pa-tients with total body pain, medica-tions and their combinations were

researched. Minimal effective dosages

were used, which, although nominal,is beyond regular uses.

II The Verdict

Ultimately, the patient was diagnosed

with total body RSD.This implies thatpain can involve more than just as-sorted extremities or regions. The dis-ease became chronic and caused pain,physical disability,and complications

involving multiple systems.To improveher treatment result, multiple drugsand therapies are needed. For more in-

formation, clinical practice guidelinesare available from the International Re-

search Foundation for RSD/CRPS at

http://www.rsdfoundation.org. ~

REFERENCES

J. Schwartzman R), Popescu A. Reflex sympathetic

dystrophy. Current Rheumatology Reports. 2002;(4):165-169.

2. Schutzer SF, Gossling HR. The treatment of re-flex sympathetic dystrophy syndrom (Currentconcepts review). J Bone Joint Surg Am. 1984;66:625-629.

3. Turner-Stokes L. Reflex sympathetic dystrophy-

a complex regional pain syndrome. Disability andRehabilitation. 2002;24( 18):939-947.

4. Teasel! RW, Potter P,Moulin D. Reflex sympatheticdystrophy involving three limbs: a case study. ArchPhys Med Rehabil. 1994;75(9):1008-1010.

5. Allen G, Galer BS, Schwartz L. Epidemiology ofcomplex regional pain syndrome: a retrospectivechart review of 134 patients. Pai". 1999;80(3):539-544.

6. Bruehl S, Harden RN, Galer BS. External valida-

tion of IASP diagnostic criteria for Complex Re-gional Pain Syndrome and proposed researchdiagnostic criteria. International Association forthe Study of Pain. Pain. 1999; 81(1-2):147-154.

ABOUTTHEAUTHORS

Yan Chen is a Nurse Practitioner at The Washing-ton Home and Hospice, Washington, DC. Dr. JohnKelly is a Neurologist and Chairman at the Depart-ment of Neurology at George Washington Univer-sity Medical Center,Washington, DC.