The Reflex Sympathetic Dystrophy Syndrome - Outcome Medical of ...

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The Reflex Sympathetic Dystrophy Syndrome RSDS
III.
Efficacy
Scintigraphic Studies
Further Evidence for the Therapeutic
of Systemic Corticosteroids
and Proposed Diagnostic
Criteria
raphy.
iy done
isks
of
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FRANKLIN KOZIN M.D.
LAWRENCE M. RYAN. M.D.t
GUILLERMO F. CARERRA. M.D.
JAGMEET S. SOIN. M.D.
Milwaukee. Wisconsin
ROBERT L. WORTMANN M.D.
Wood. Wisconsin
Sixty-four patients
were evaluated prospectively for reflex sym
pathetic dystrophy syndrome RSDS
using quantitative clinical
measurements
high-resolution roentgenography and scintigraphy.
Five
separate groups
lowing us to distinguish
of the RSDS
as well as 16
were identified by
their clinical features
patients
with definite or incomplete forms
patients
with other disorders. Scintigraphy
was found to be useful diagnostic study that may also provide
method of predicting therapeutic responsa Systemic corticosteroid
therapy proved to be highly effective mode of treatment for up to
90 percent of the patients
with the R5DS.
al
ionic
ise.
ye
Arch
method
CO 1968
rombosis
1969
ilmonarv
tred
and
of deep
il
imped
Diagnosis
bedside
tolism an
1973 107
tive
tional
1973.
antico
Klan
trial.
Co
tim Versus
venous
prognosis.
Waltman
course of
1nd neg
126561.
From the Departments
of Medicine lRheunm
tologyl and Radiology. The Medical College
Wisconsin. Milwaukee County Medical Coot
plex. Milwaukee. Wisconsin and the Wood
Veterans
Administration
Hospital. Wood. Wis
consin. Requests
for reprints
should be ad
dressed to Br Franklin Kozin. Division of
Rheumatology. The Medical College of Wis
consin 8700 W. Wisconsin Avenue Milwaukee.
WI 53226. Manuscript accepted July 7. 1981.
Recipient of Clinical Investigator Atartl
JAM00483-Olj from the National Institutes
Arthritis. Metabolism Digestive Disease.
Recipient of Postdoctoral Research Fel
lowship from the National Arthritis
tion.
Founda
The reflex sympathetic dystrophy syndrome RSDSJ was first recog
nized over century ago Subsequent reports
have described
number of clinical variants
differing primarily in their associated
features
and predisposing factors
Unfortunately clear concept
of this
disorder has been frustrated by the lack of clear diagnostic cri
teria the myriad of terms
applied to the syndrome the absence of
proved therapeutic modalities
and our poor understanding of its
pathophysiology.
The classic features
of the RSDS
were vividly recorded
by Mitchell
Moorehouse and Keen and have been reiterated more recently by
DeTakats
and Stein brocker and Argyros
These consist of pain
and tenderness
usually in distal extremity associated with signs
or
symptoms
of vasomotor instability trophic
skin changes
and
swelling.
suspected predisposing event can be identified in up to 65 percent
of the cases
Roentgenographic evidence of acute patchy. bon
demineralization in the affected extremity is characteristic
Scintigraphy performed systematically in small number of patients.
revealed increased periarticular radionuclide activity suggesting
that this
technique may prove useful in patients
suspected of
RSDS.
The therapeutic modalities
available in the RSDS
RSD have been re
viewed recently Sympathetic blockade or systemic corticostero l
therapy appear to offer the greatest hope of success. especially when
employed early Our results
with systemic
corticosteroid treatment
in small group of patients
was encouraging and this
observation
is confirmed and extended here.
In the present prospective study 64 patients
were assessed for pos
sible RSDS. using clinical measurements
scintigraphy and high res
olution roentgenography. Diagnostic criteria that may help in further
defining
this
complex disorder were evaluated and the therapeutic
January 1981 The American Journal of Medicine Volume 70 23

ff
25
4.
IF EFI.EX SYMPATIF.TIC lYSTIti Pt lv svnut P.IF ki ri\ El .\l.
Figure
iltustra.
distingi..
with
thc
Figure 1. Fine-detait roentgenograms
of the hands
of patient with the PSOS. showing the normal left hand left and the
characteristic patchy demineratization in the affected right hand right.
regiuit.
at
se
efficacy of stellate ganglion blockade and systemic
corticosteroid administration was corn pa red.
METHODS
Patients.
All
patients
referred to tb Rhsunlitoligv
fr Consultation Service. The Nlilwiijkee Count Nt.dic.d
Center. in whom diagnosis
of RSDS
was seriously consider
were included in the study. Their clinical findings
wer
cifriled. and the most likely precipitating event was identifid
whenever possible. Prior treatment and subjective resiinsns
were noted.
Patients
with isolated proximal sites
of involv
ment e.g.. knee hip shoulder as the sole sit of involvement
were excluded because of the uncertainty that RSRS
fttrK
such
areas
Clinical Criteria. Patients
vere divided into five
ri1j
1115th
tipiin
theirclinical
findings.
Griaip
cuinsistisi
tifp.tifnt
ith pain and tenderness
in an extremity associated v.
mptoms
or signs
of vasomotor instalilitv particularl trio
pur.uturi
or color changes. and generalized swelling in ill
same extremity definite RSDS. Group
II consisted of if
ith pain and tenderness associated with vasuimotor instiliilip
itit
to Is
tr el hog in an extremity probable RSDSJ. Patients
in
Ill possible RSDS
had only pain and luisiiull tnulerne iii
in ci
f\trumitv which cojltl not Flu attributed tif
sjfuCific nerve injury. Grifilif IV lpfissilflf
rfcfnt irfi
RS
ist.ul
patients
who had vasomotfur instihui1it .ini stHIi
ii ut 11ff
pa in .ini ltisi ial lv no tenderness. In it if ii the patients
liel ffthfr disffrders
identified or did not fulfill the criteria for
inf.liision
in other groups.
lriiphic skin ch.inges. although
vie nit iseil as criterion since these changes
characteristic of the RSDS.
may lie asso
t.iiteil with chronic swelling of another etiology chronic dis
ease alone or other disorders such as scleruiderrna.
Clinical Measurements. Objective. scm iquantitative mea
sitrements were performed. as previously described 191.
clients
with upper extremity involvement before treatment.
it vi ri ins in te na Is rUt ring treatment nil alter treatment.
lii all cases
subjective estimate was made if the patients
1.111 response during
treatment. Patients who experienced
mi relief iii
over 75 percent were regarded as having an cx
ut res ffnsf
if 50 to
percent. got
itf t. fair response and less
tha ii
115ff.
in
if If ionse of 25 to
funen t.
poor
Roentgenographic Studies. Fine-i let.iil rf ff
ntgenographv of
tIlf hands
or feet was performed in all but one case. Roent
gfnfigriums
were examined blindly by three fibservers
and
red us isitive if diffuse or patchy fist 1100 in Figure
irsffnt in the clinically affected extrimitv
Scintigraphic Studies. Scintigraphv as performed in
.fjifrit\ fff titftifnts. using
technetiumttuuui tTc methvlene
lpiii fsfllffn.ftff \tDP. or occnsiiinally tiuthanethv
liff. t.IfhiphffcfhfunateEF-lDP. ftrruhii injection of ISmCi
image.
and
after
bleed
Scm
cc
Consit
of rad
Figure
genera
relati
scribet
prior
TREA
Twent
Severa
respnr.
gang11
jective
wereg
consist
60mg
in
foti
rapili
5mwf
were
24 January 1981 The American Journal of Medicine Volume 70
I..

TUE REFLEX SYMPATHETIC DYSTROPHY SYNDROMEKOZIN ET AL.
4j
Ri.
41t. e%
rt ...
.1111/31
13 13 13
AAUi 1%
Figure
2. Rapid-sequence radionuclide flow studies
are
illustrated. normal symmetrical flow pattern top is readily
distinguished from the asymmetrical flow bottom in patient
with the RSDS.
2ft and
the
Patients
riteria
the
nronic
for
RSDS
be asso
dis
itive mea
bed
rea tment
atrn.nt.
in
patients
p.rtenced
in an
cx
sc 01 25 to
ent.
.i
poor
lraihv of
.. Roent
ers
Figure
rnied
and
in
methylene
thinet-hv
iii of 15 mCi
of radionuclide.
scintigraphs
of the affected and contralateral
regions
were obtained as rapid-sequence blood flow studies
at sec/frame for minutes
and as 3-hour delayed static
image.
In number of cases. the total counts
over the affected
and contralateral extremity were determined immediately
after the flow study to assess
the relative
blood
pooling.
Scintigraphs were reviewed by
rats
of uptake or
all of us. Flow studies
were
Considered positive when there was asymmetric appearance
of radionuclide in the affected and contralateral extremities
Figure 21. Static images
were positive when there was
general
increase in periarticular activity on the affected side
relative to the contralateral side Figure as previously de
scribe.d Increased radionuclide activity at the site of
prior trauma or fracture was excluded from analysis.
TREATMENT
Twenty
patients
were treated with stellate ganglion blockade.
their Several had received this
prior to referral. and
subjective
responses
were recorded. Other patients
were given steilate
study so that ob
ganglion blockade during the course of the
jective
measurements
were available. The majoritY of
patients
were given curticosteroids. Initial therapy
in most instances
consisted of prednisone. 60 to 60 mg for two to four days. 40 to
61 mg for two to four lays
and 30 to 40 mg for two to four lays
in four equally divided loses
subsequently the lose was
rapidly tapered as single morning dose of 40. 30. 20. tO and
mg
for Iwo or three clays
each. Occasionally higher doses
were given for up to two weeks. but
patienls
rarely
received
Figure 3. Static scintigraphs
are shown from normal
subject top and patient with RSOS
bottom. The increased
activity
dent.
more than three or four weeks
of systemic corticosteroid
therapy. Several patients
required retreatment because of
poor or initial
incomplete
response. or because of recurrence
of symptoms
weeks
or months
later.
RESULTS
Patients. The characteristics
and demographic data
of our patients
are shown in Table 1.
Twenty-one pa
tients
fulfilled criteria for RSDS. as they exhibited each
of the features
of the complete syndrome group their
condition was classified as definite RSDS. There were
it patients with probable group II and 16 patients
with
possible groups
III and IV RSDS. The associated dis
orders
which clinically and temporally appeared to
represent precipitating event are listed for groups
through IV in Table
suggested 2.
Sixteen
RSD group VI.
an RSDS
II using the categories
previously
patients 25 percent did not satisfy criteria for
in 14 88 percent of these
arthritis
two
tissues
of the affected hand is evi
in periarticular
Another diagnosis
was established
patients
including
infectious
rheumatoid arthritis
one patient
patients.
Reiters
syndrome one patient systemic lupus
er
January 1981 The American Journal of Medicine Volume 70 25

II Probable
II 11
II 11
Ill
III
TIlE IZEFLEX SYMPATHETIC DYSTROPHY SYNIROMEKO/.IN El Al..
TABLE
Characteristics
of Patients
in Each
of Five Groups
TABLE IV
Patients
Female Age Duration Involved Sitet
Group no. no. yr vik UE I.E
Grouc
21 14 52.4 13.7 25.1 23.2 14
48.5 17.9 84.2 155.0
III 43.3 17.4 152.7 148.2
IV 55.3 17.2 38.6 34.5
16 43.7 14.4 34.5 36.3 14
lv
Total 64 36 48.3 15.2 75.9 67.9 46 18
Values
given
indicate the mean standard deviation.
Total
liE upper extremity hand LE lower extremity foot.
TABLE Ii Frequency of Associated Conditions
in Patients
with Possible RSOS
Groups
through IV
IV
Possible Etloloc Factors
Peripheral Cerebral Spinal idIopathic
no.
Total
Subject
Nerve Myocardlal Disease or Cord or
Patients
Group no. Trauma Injury Fracture lschemla Hentiplegla Injury Miscellaneous
Total
III
IV
21 10 23
11
Total 48 10 15 lit 50
Percent 20 30 14 22 100
Two
patients
had peripheral nerve injuries
as
Three patients
had carcinomas
two breast one lung
shoulder brachial plexus
metastasis.
result of fractures.
TABLE Ill
Roentgenoiogic Findings
in the Five Groups
Group
Total
Patients
no.
Changes
No.
but this
appeared to be clinically implicated in the RSDS
in
Roentgenogram
Positive
Changes
RSD in only one patient with
on Scintigraph
Definite P505 21 21 81 18 83 16 69
Ill
P505 11 11 45 10 40 10 40
Possible PSDS
22 13
lVPossibleRSDS
57 28 43
Otherdiagnoses
16 15 29
Total 64 63 49 50 44 40 46
No.
Static
Images
Positive
No.
Flow
Study
Positive
affected
mal inter
72 in
Roentge
exhibite
bone
us
Howeve
was
con
as posi
RSDS
RSD
Roentg
the pati
foundir
group
percent
Ostec
as only
ceeding
of the
osteope
had
del
as earl
All If
flamrn
arthriti
ythematosus
lone patient. uncharacterized arthritides
two
patients
rotator cuff
tears
demonstrated
arthro
graphically two patients
peripheral neuropathy four
patients
and hysterical conversion reaction one pa
tient. Thus
almost half of these patients
had an in
flammatory arthritis
based upon synovial fluid findings
iiJ and considerable generalized edema often pitting
of their involved extremity. Careful examination always
disclosed localized site of tenderness and pain de
spite diffuse edema. Absence of pain and tenderness
the hand or foot when there is extreme wrist or ankle
RSD
pain is strong evidence against an RSDS
in
Clinical Measurements. Initial measurements
were
available for 63 percent of the patients
with upper cx
tremitv involvement 86 percent of those in groups
Il and confirmed our previous
observations
grip strength in the affected hand was reduced 136.2
and
The
16.8 mm Hg mean SENt 18 when compared with
the contralateral hand. Similarly the tenderness
score
was 95.5 8.5 10 greater in the affected hand
and all but one patient. who had essentially equal ten
derness
bilaterally. had greater scores
on the affected
side. Finally ring
sizes
were 12.9 2.1 mm 17 patients
greater in the proximal interphalangeal joints
of the
agnosti
Scintig
78 pert
cent
jT
quenc
positi
signifi.
rical
II
Half
showe
studies
the all
side.
26 January 1981 The American Journal of Medicine Volume 70

II
22
THE REFLEX SYMPATHETIC DYSTROPHY SYNDROMEKOZIN ET AL
TABLE IV
Responses
of Patients
to Steilate Ganglion Blockade or Systemic Corticosteroid Therapf.
Siti
LI
Patients
Response
Treated
Poor Fair Good Excellent
Group no. %J
to
II 100
Stellate Ganglion Blockade
71 28
tIl
100
18
IV 100
67 33
Total 20 85 15
Systemic Oral Ccrtlcosteroid Therapy
17 11 29 53
tlt 100
11 22 44
IV 33 33 33
100
Total 35 29 23 40
Subiective
responses of pain relief were graded as discussed in text.
Otis
Total
23
11
50
100
ie patient with
Study
Positive
69
40
43
46
ments
were
th UPlF cx
groulls
ifl5
iced 136.2
and
The
iipared with
erness
score
ected
hand.
equal tenthe
affected
17 patients
oints
of the
affected
hand. Only two patients
had increased proxi
mal interphalangeal
72 mm.
joint sizes
on the contralateral
hand
Roentgenographic Studies. Many roentgenograms
exhibited the characteristic patchy dernineralization of
bone usually associated with the RSDS
Figure 1.
However the presence of unilateral diffuse osteopenia
was considered sufficient for roentgenograms
to be read
as positive as this
may be observed in patients
with
RSDS
of greater than six to 12 weeks
duration
Roentgenograms
were positive in approximately half
the patients
in the study Table III. Osteopenia was
found in 81 percent of the patients
with definite RSDS
group I. 45 percent with probable group II and 57
percent with possible RSDS
RSD group Ill.
Osteopenia was not related to duration of symptoms
as only 25 percent of the patients
with complaints
ex
ceeding one year had this
finding. In contrast 28 percent
of the
patients
with complaints
osteopenia. Most of these
of 12 or fewer weeks
had
subjects
12 of 18. 67 percent
had definite or probable RSDS. Osteopenia was found
as early as five to eight weeks
in four patients.
All five patients
group with osteopenia had in
flammatory
three patients
or infectious
two patients
arthritis
which may have contributed to the initial di
agnostic confusion.
Scintigraphic Studies. Scintigraphs
were obtained in
78 percent of the patients
and were positive in 44 per
cent Table III and Figures
and 3. Although the fre
quency of positive flow studies was
positive static scans
less
than that of
this
was not statistically
significant. Only one patient group IV with asymmet
rical flow had normal static scan.
Half of the patients
in groups
through IV 18 of 36
showed asymmetrical radionuclide activity on the flow
studies. In 15 of the 18 patients
flow was increased on
the affected side suggesting enhanced perfusion on that
side.
Forty-nine percent 21 of 43 of the patients
in groups
through LV had both positive roentgenograms
and
scintigraphs. whereas
in 33 percent both studies
were
negative.
whose
each
Scintigraphs
were positive in two patients
roentgenograms
in groups
and IV.
showed no abnormalities
abnormalitie one
Assuming that patients
in groups
and II indeed had
RSDS
whereas
those in groups
III and IV did not the
scintigraph proved to be more specific study than the
roentgenogram 86 percent versus 71 percent
the
whereas
sensitivities
were virtually identical 68 percent
versus
39
percent
Treatment. None of the 20 patients
receiving
stellate
ganglion blockade had good response Table IV. Ten
were referred ofter failure of stellate ganglion blockade
and therefore represented selected group the re
maining 10 received stellate ganglion blockade while
under our care either because of personal preference
two patients
prior failure of corticosteroid therapyfive
patients
or presence
of medical disorders
contraindi
cating high-dose systemic steroid therapy such as dia
betes
mellitus
three patients. One such case is illus
trated in Figure this
patient had both subjective and
objective improvement during series
of stellate gan
glion blockade followed by worsening of her condi
tion. She had rapid and sustained improvement with
systemic corticosteroid therapy.
In contrast to the data with stellate ganglion blockade.
13 percent of the patients
had good to excellent re
sponse to systemic corticosteroid therapy and this
figure
was increased to 82 percent and 66 percent in the sub
sets
with definite and probable RSDS
respectively
tither combinations
actually enhance this
difference
rtt.tr the sensitivity and specificity of the rnentgenogrLims
it is likely
enstimated in this study because of the careful selection of
1utients
anti their referral for possible RSDS. thereby eliminating
Ikitients
with simple disuse osteoporosis lace 61 and 1311.
are
January 1981 The American Journal of Medicine Volume 70 27

THE REFLEX SYMPATHETIC DYSTROPHY SYNJDROME-Ktu/.INJ EF AL.
Figure 4. The clinical measurements
and subjective re
sponses
of patient with the ASOS
are shown for the affected
hand and contralateral hand 0. The patient was treated
initially with four stellate ganglion blocks
arrows
which
provided mild transient improvement. Administration ot
systemic corticosteroids
subsequently resulted in an excel
lent sustained response.
Table IV. Of the Ii patients
who were treated with
stellate ganglion blockade first one had an excellent
Figure four good one fair and five poor re
sponse to the administration of systemic corticosteroids.
Conversely none of five patients
who were given stel
late ganglion blockade after corticosteroid therapy im
proved. The duration of symptoms
prior to the admin
istration of corticosteroids did not seem to affect the
response.
270 RING S/Zr PIP JOINTS
260
mm 250
80 PEOMSCNE
mg/day
401 iTcF.n..
Doys
rb
eb
Objective
measurements
compared favorably with
patients
subjective responses
Table V. Interestingly.
objective improvement was present in all but one pa
tien who received corticosteroid therapy. particularly
in the tenderness
Patient
compared
percent
scores.
responses
to corticosteroid therapy also were
to results scintigraphic studies. Ninerv
of the patients with positive scintigraphs
showed
good or excellent improvement. Conversely. 64 percent
of the patients with negative scintigraphs
showed poor
to fair response to corticosteroid therapy. Thus
presence
of positive scintigraph is significantly correlated with
good to excellent therapeutic response. 0.011. x.
COMMENTS
The present study has defined several aspects
of the
RSDS
that previously have created diagnostic and
therapeutic
confusion.
Diagnosis
and Differential Diagnosis. Patients
were
divided into five groups
based upon strict clinical cri
teria. In 25 percent of the patients initially suspected of
having RSDS. insufficient evidence was found to
support this
diagnosis
group V. An alternative diag
nosis
was established in 1488 percent providing some
insight
RSDS.
Pain
into which disorders
disorder may
be confused
with the
tenderness
swelling and warmth vasomotor
instability were present in the distal extremity of
seven
patients
with arthritis. Although these findings
were unilateral careful examination revealed local
tenderness
isolated to the involved joints
rather
than
the diffuse tenderness
so characteristically found in the
RSDS
Two cases
of unsuspected septic arthritis
were found. Four additional patients
had specific pe
ripheral nerve injuries
with sensory and/or motor
changes
in the expected distribution but with none of
the other
features
of RSDS.
TABLE ComparIson of Serial ObjectIve Measurements
and Subjective Responses
of Patients
Treated with Systemic Corticosteroids
TABLE
Definite
Pain
Signs
Swei
nence
Dystr
Probabl
Pain
At.
Vascr
OF
Swell
Dystr
PossIble
Vascr
OF
Swer
Nope
tDyst
Doubtfu
tjnex
of pati
in gro
incor
In
associ.
four
fering
II in
Tablc
Table
tigra
sign if
pareci
also
study or in number
Steinbrocker and Argyros
have suggested that
circumscribed forms
of the RSDS
exist such as iso
Based
jectivt
lated Dupuytrens
contractures. We found no evidence
swelli
to support this
concept either in this
Table
Roen
Roen
with the RSDS
was ii
it was
chara
Objective
Response
patch
Subjective
Response
Grip
Strength
mm hg
Tenderness
Ring Size
mm
Excellent 46.4 7.2 24.0 5.0 84.9 7.7 2.9 4.6 13.2 2.1 4.7 2.1
Good 98.0 83 32.7 33.3 58.0 24.9 21.5 17.2 7.3 2.2 4.5 2.5
Fair 30.0 17.6 1.7 19.6 24.7 23.1 4.0 10.8 3.0 1.6 2.7 1.8
Poor 60 20 16 11.4 14 10 3.5 2.5 4.0
Combined 46.3 14.8 21.0 7.5 61.2 0.7 7.4 5.0 9.1 1.6 4.2 1.3
Predominantly affected hand contralateral hand. Changes
represent differences
week after corticosteroid therapy.
Mean
SEM.
in measurements
from before to at least one
descr
disus
umer
osteo
nostie
As
speci
perc
versti
to be
28
January 1981 The American Journal of Medicine Volume 70

TUE REFLEX SYMPATFIETtC DYSTROPHY SY4DROMEKOZtN ET AL.
.lilV ith
restinglv
inc j.a
rticularlv
also were
es.
Ninety
is showed
ed
percent
poor
presence
lated
UO1
cts
ostic
vith
of the
and
.ents
were
tinical
.spected
found
itive
cri
of
to
diag
ding some
ti
ith the
vasomotor
tremity
of
findings
aled
local
ather than
Ic
und
in the
arthritis
pecfic
br motor
.th none
1ested
.uch
is
pe
of
that
as isoevidence
number
RSDS
4.7 2.1
4.5 2.5
2.71 1.8
4.0
at
least one
TABLE Vt Proposed Clinical Diagnostic
RSDS
Definite RSDS
Pain and
RSD
tenderness
in the distal extremity
Signs
and/or symptoms
of vasomotor instability
Swelling
nence
Criteria for
in the extremityoften with periarticular promi
Dystrophic skin changes
Probable RSDS
RSD
Pain and tenderness
AND
Vasomotor
OR
Swelling
instability
Dystrophic skin
Possible RSDS
RSD
Vasomotor
OR/AND
Swelling
No pain
Dystrophic skin
Doubtful
instability
usually present
changes
often present
but mild-moderate tenderness
RSDS
Unexplained pain and tenderness
may be present
changes
occasionally present
in an extremity
of patients
observed previously. Nonetheles
patients
in groups
IV may indeed have partial or
incomplete forms
of the syndrome.
In the remaining 48 patients
associated with the RSDS
clinical findings
were
RSD and they could be divided into
four groups. Only group II appeared distinctive dif
fering in the frequency
of associated conditions
Table
II in the roentgenographic and scintigraphic data
Table Ill and in response to corticosteroid therapy
Table Differences
in the roentgenographic scm
tigraphic and therapeutic findings were statistically
significant. 0.00i Chi-square analysis
when com
pared with those in group I. Therapeutic responses
were
also significantly better in groups
II and IV 0.05.
Based upon these data as well as the presence of ob
jective clinical changes
of vasomotor instability and/or
swelling we propose the diagnostic criteria outlined in
Table VI.
Roentgenographic and Scintigraphic Studies.
Roentgenographic evidence of bony demineralization
was most frequent in patients
with definite RSDS
but
it
was not uncommon in the other groups
Table III. The
characteristic roen tgenogra phic appearance of acute
patchy osteoporosis
in the RSDS
has been extensively
described It cannot be differentiated from
disuse osteopenia except possibly by doc
umenting its early development For these reasons.
osteopenia must not be considered reliable diag
nostic
criterion
As already stated scintigraphy proved to be far more
specific than roentgenography 86 percent versus
71
percent with no sacrifice in sensitivity 68 percent
versus
69 percent. Furthermore. scintigraphs
appeared
to be useful guide to therapy. Ninety percent of the
patients
with positive scintigraphs
experienced good
or excellent response to corticosteroid therapy com
pared with 34 percent of the patients
with negative
scintigraphs. 0.001. These results
indicate that
scintigraphy may be useful both as diagnostic aid and
as predictor of therapeutic responses. As the numbers
of patients
are relatively small this
observation
viewed cautiously until confirmed
The presence of asymmetric
must be
radionuclide flow studies
in half the patients was also interesting Table III Figure
2. It
appears
to be an indication of asymmetric blood
flow and if so would be consistent with previous
physiologic studies
showing enhanced local blood flow
in the affected extremity of patients
with the RSDS
In several
but within
patients
scintigraphs
were positive initially
normal limits
following corticosteroid ther
apy. suggesting that scintigraphy may demonstrate an
active potentially reversible process
in certain subjects.
These data are the subject of separate report.
Treatment. The present study confirms our prior ob
servations
regarding the efficacy of corticosteroid
therapy in the RSDS
and extends
them to patients
with
incomplete varieties
of the syndrome groups
II and
IV. Seventy-six percent of the patients
with
definite
group 14 of 17 probable group II six of nine and
possible group IV two of threel RSDS
experienced
good or excellent subjective response. Interestingly
when the clinical measurements
are included in this
assessment of therapeutic response data not shown 90
percent of the patients
given systemic corticosteroids
had good to excellent therapeutic response mm
provement in both tenderness
and swelling. These
response were not affected by the duration of symp
responses
toms
prior to corticosteroid
administration.
Stellate ganglion blockade may be effective in the
RSDS
However sustained responses
are not
as frequent as commonly believed 12822-25. Our ex
perience here was discouraging. But it
must be appre
ciated that this
study was not designed to compare
stellate ganglion blockade with corticosteroids
since all
patients
who failed corticosteroid therapy were given
stellate ganglion blockade whereas
the converse was
not true. Appropriate studies
are needed
in this
area.
In summary the present study has provided some
insight into the differential diagnosis
of the RSDS. Ar
thritis
of the wrist. carpal ankle or subtalar joints
may
mimic the syndrome in the hand or foot. Careful atten
tion to localizing symptoms
and
signs
as well
as
to the
proposed criteria may help in diagnosis. Changes
on the
roentgenogram are not helpful diagnostic finding since
involutional disuse or inflammatory osteoporosis
may
be present in number of patients
suspected of an
RSDS. Scintigraphy proved to be valuable examina
tion both in diagnosis
and in predicting which patients
would respond to corticosteroid therapy. Finally this
study
firmly establishes
the efficacy of systemic corli
r.osteroid therapy in patients
with the RSDS.
January 1981 The American Journal of Medicine Volume 70 29

itE xvtx syxtpvrt IET1t tYS1tU IPI lv SYNER r.tE KOZJN El Al..
REFERENCES
I. Mitchell SW. et at. Gunshot wounds
and other injuries
of
nerves. Philadelphia LB Lippincott. 1864.
2. Kozin The painful shoulder and reflex sympathetic dys
trophy vndrome. In McCarty Of ott. Arthritis
and allied
ind turns. 9th ed. Philadelphia Lea Febiger. 1979
11111120.
DeTakats
Reflex dystrophy of the extremities. Arch .Sctrg
1937 34 939-956.
4. Stein brocker 0. Argvros TG The shoulder-hand syndrome
prcsentstatus
as diagnostic and therapeutic entity. Mcii
din North Am 198 42 1533-1553.
5. Herrmann LG. Reineke HG. Caldwell JA Post-traumatic
painful osteoporosis
clinical and roentgenological entity.
Am Roentgenol 1942 47 353361.
Genant HK. Kozin F. Bekerman C. McCarty The
6. Dl. Sims
Sim
reflex sympathetic dystrophy syndrome comprehensive
analysis
using fine-detail radiography photon ahsorp
tiometrv and bone and joint scintigraphy. Radiology 1975
117 2132.
7. Kozin F. Genant HK. Bekerman C. McCarty DL The reflex
sympathetic dystrophy syndrome. II. Roentgenographic
and scintigraphic evidence of bilaterality and of periarti
cular accentuation. Am Med 1976 60332338.
8. Rosen PS. Graham The shoulder-hand syndrome his
torical review with observations
on 73 patients. Can Med
Assoc 11958 77 8691.
9. Kozin F. McCarty DJ. Sims
J.
Genant HK The reflex sym
pathetic dystrophy syndrome.
I. Clinical and histologic
studies
Evidence for biiaterality. response to corticoste
roids. and articular involvement. Am Med 1976 60
321331.
10. Bekerman C. Genant HK. Hoffer PB. Kozin F. Ginsberg MH
Radionuclide imaging
Radiology 1976 118 652-659.
of the bones
and joints
of the hands.
11. McCarty Dj Synovial fluid. In McCarty Dl.
ed. Arthritis
allied conditions. 9th ed. Philadelphia Lea Febiger. 1979
51-69.
and
12. Micsi AT. Xliii lager TA Epic lent ohigv cci the rheitma tic is.
oases. In NliGa rtv Dj.
ec Arthritis am all id inc I. lit ii ins.
9th ed. Philadelphia l.ea Febiger. 1979 It 12
t. toes
Rat lii clccgital at cpea rances
clil SI IS strc cats. Cl in
Rad el 169 20 345353
14. to rrma E.G. Ga Idwel jA Din goosis .10 treat nt of
xcstrhei imatic cisteup critsis. Sit rg 194 1131
1140.
15. Rrailsford jE Pathological changes
ditced by injury. Br Med 1936 657663
in bones
anti tunIs
uric.
iii. Steinbach Fit. The roentgen appearance of osteoporosis.
Rail ioi Gun North Am t964 197207.
17. Feist jH The biologic basis
of radiologic
findings
in
bone
disease recognition and interpretation
of cbnormal bone
architecture. Radiol dIm North Am 1970 8183-206.
8. Miller OS. DeTakats
Post-traumatic dystrophy
of the ex
tremities
SudecVs
atrophy. Surg Gyn cccii Obstet t94 1125
558-582.
19. Stoite 5K. Stolte JB. Leyten JF Die pathofysiologie von ist
shoulder-hand syndrone. Ned Tijdschr Geneeskd 1970
11412081215.
20. Lehman El Traumatic vasospasm study of cases
vasospasm in the upper extremity. Arch Surg 1934 29
92- 107.
21. Rassmussen TB. Freedman Treatment of causalgia an
analysis
of 100 cases. Neurosurg 1946 26 165-173.
22. Steinbrocker 0. Neustadt OH. Lapin Shoulder-hand
syndrome sympathetic block compared with corticotropin
and cortisone therapy. JAMA 1953 153 788-791.
23. Evans
IA Reflex sympathetic dystrophy report of 57 cases.
Ann Intern Med 1947 26 417-426.
24. Drucker WR. Hubay CA. Hoiden WD Pathogenesis
of
post-traumatic sympathetic dystrophy. Am Surg 1959 97
454465.
25. Patman RD. Thompson jE. Perrson AV Management of
post-traumatic pain syndrome report
Surg 1973 177 780786.
of
of 113 cases. Ann
Ch
Pat
G. KU
K. S.
R. C.
Alinzqu
From
ogy.U
icine.
AMAt
from ft
grant
ft
as the
and
the
beadd
versity
BCMC
87131.
Prc
logy.
of Tror
30 January 1981 The American Journal of Medicine Volume 70