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Hemiplegia and rheumatoid hemiarthritis.

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Hemiplegia and Rheumatoid Hemiarthritis
OLLOWING hemiplegia, poorly underF s t o o d syndromes may appear with pain,
swelling and disability on the paralyzed
side. The most common event is the
shoulder-hand syndrome described by
Friedman et al.,I or, less frequently, the
thalamic pain syndrome of Head and
Holmes.2 Not widely appreciated and of
great biologic importance is the “good that
may accompany hemiplegia. Hemiplegia
followed by rheumatoid arthritis or generalized osteoarthritis with Heberden’s nodes
is often associated with a striking protective
effect on the paralyzed side. The degree of
protection is approximately proportional to
the magnitude of the paralysis.
Such a patient with hemiplegia and
rheumatoid arthritis is the subject of this
The patient was a 50-year-old policewoman who
had hypertension and sustained a right internal
carotid artery thrombosis and left hemiparesis on
December 28, 1951. A rehabilitation program was
promptly initiated in the hospital. In four months
she was able to walk with a brace and crutches
and carry out the activities of daily living. She
had partial use of the left arm and hand, though
they remained quite weak. Five months after the
stroke she had abrupt onset of pain, swelling and
redness of the second metacarpophalangeal joint
of the right hand lasting three days. Each two to
three weeks thereafter she had episodes of pain,
heat and redness in the wrist, metacarpophalan-
geal and proximal interphalangeal joints of the
right hand. These episodes became severe and
disabling, episodic for two to three years, finally
becoming constant. The right shoulder, elbow,
wrist, metacarpophalangeal and proximal interphalangeal joints became involved; the right knee,
ankle and metatarsophalangeal joints were also
swollen and tender.
Although she also complained of severe pain in
the left hand, described as “air blowing through
holes in the bones of the hand,” the joints of the
left upper and lower extremity (the paralyzed side)
did not become inflamed, and except for a lesion
of the left ulnar styloid process, showed no roentgenologic evidence of erosion or destruction. She
was treated with 4 to 6 gm. of aspirin daily,
prednisone, physical therapy, rest and exercises.
Gold treatment was not helpful.
Surgical procedures included a hysterectomy in
1940, removal of a left ureteral calculus in 1949,
fracture of left femur in 1953, cholecystectomy and
repair of a perforated peptic ulcer in 1956, triple
arthrodesis of the left foot, and hand surgery for
the third and fourth di,gits of the right hand in
1960. Chronic pyelonephritis developed due to E.
Serial examinations revealed the vital signs to be
normal. The weight was 135 lbs. The ocular fundi
showed grade 2 arteriolosclerosis and slight bilateral corneal opacities. The heart and lungs were
normal. The abdomen contained a palpable but
non-tender liver.
The left side was hemiparetic. The left hand
was claw-like, though there was no joint inflammation. The left shoulder and elbow were normal.
The left knee contained a small effusion. The left
foot was abducted and inverted. Tenderness could
be elicited in the foot but this was not localized to
the joints. The cervical spine was within normal
From the University of Vermont College of Medicine, Department of Medicine, Rheumatism Research Unit, Burlington, Vermont.
This work was supported by U.S.P.H.S. Training
Grant AM-05087.
JOHN H. BLAND,M.D.: Associate Professor of
Medicine, Deparfment of Medicine, Unioersity of
Vermont College of Medicine; Director, Rheumatism Research Unit. WINSTONM. EDDY, M.D.:
Assistant Professor of Clinical Medicine, Department of Medicine, University of Vermont College
of Medicine.
11, No. 1 (February 1968)
Fig. 2.-The hands on 15 January, 1960. Left: This is a “claw” hand, but the
joint spaces remain open a n d uninvolved in any radiologically detectable rheumatoid process. Right: This hand shows more extensive disease, with gross subluxation,
massive erosions a n d absorption of bone a n d loss of carpal bones.
limits. The right metacarpophalangeal and proximal interphalangeal joints were all thickened, swollen and warm. They showed gross subluxation and
deformity. Swan-neck deformities were present. Interosseous atrophy was evident. The right elbow
and shoulder were tender and swollen; motion was
limited by pain. There was a large effusion and
marked thickening of synovium and capsule. The
right ankle and metatarsophalangeal joints were
swollen, warm and tender.
On the left there was an extensor plantar response. ankle clonus, and hyperactive deep tendon
reflexes. The deep tendon reflexes on the right
were normal. Rheumatoid nodules were noted on
the extensor surface of the right forearm only.
The left dorsalis pedis and posterior tibia1 pulsations were not palpable; those on the right could
be easily felt. Both femoral pulses were palpable.
Laboratory data included the following: hemoglobin, 9 gm. per cent; RBC, 3.9 million/cmm.;
WBC, 7,00O/cmm. with a normal differential; a
trace to I+ albuminuria (on repeated testing); sedimentation rate, 37, 44 and 31 mm./hr. (Wintrobe);
latex fixation test was negative repeatedly; total
protein, 5.9 g per cent with albumin 2.6 g per
cent and globulin 3.3 g per cent. Synovianalysis
(right knee) showed fluid of low viscosity with
poor mucin clot and a leucocyte count of 20,500
per cmm.; blood urea, nitrogen, cholesterol and
glucose were normal. The electrocardiogram at the
time of the cerebrovascular accident was normal.
An EEG was non-specifically abnormal.
Roentgenologically, there was gross osteoporosis,
typical rheumatoid erosions, subluxation, and cartilage destruction in the right carpal, metacarpophalangeal, and proximal interphalangeal joints.
The left hand, by contrast, showed osteoporosis
and evidence of marked atrophy, but erosions only
in the ulnar styloid process Even the joint spaces
were intact. (See Figs. 1 and 2) Serial radiograms
of the hands showed increasing destructive changes
on the right and gradual development of mntrac-
Fig. &-Lateral radiogram ( 9 June, 1958) of the left (hemiplegic) foot showing
marked atrophy and osteoporosis but with maintenance of joint spaces and articular
surfaces. The P-A view showed advanced osteoporosis but no evidence of rheumatoid
erosions or joint destruction.
tures and claw hand on the left. Fig. 3 illustrates
x-ray films of the left foot.
In September, 1964 the patient entered the hospital following an accident at home in which she
fractured the proximal left tibia and fibula. The
blood urea nitrogen at that admission was 70 mgm.
per cent and she was found to be in renal acidosis.
She died suddenly in acute pulmonary edema.
Post-mortem examination was refused.
Coste and Forestier? reported two hemiplegic patients who developed Heberden’s
nodes nine and ten years, respectively, following strokes; the nodes appeared only
on the non-paralyzed side. Forestier? in the
same year, reported a woman who developed Heberden’s nodes only eight months
following a stroke; the nodes were restricted to the norma1 hand. Winter5 also
reported such a case. Jacquelines described
a boy who developed a hemiparesis of the
right arm and leg at age two years; thirtynine years later he developed rheumatoid
arthritis involving only the unparalyzed
side. At age fifty-one the arthritis became
very severe but still did not involve the
paralyzed side. This patient had been fully
ambulant and employed. Thompson and
Bywaters‘ reported four patients who had
strokes preceding onset of rheumatoid arthritis in whom the joints of the paralyzed
side were clearly spared. Fifteen months
separated the stroke and arthritis onset in
one instance; two years, thirty-two years,
and six years in the other three. Radiologic
evidence of characteristic rheumatoid erosions, cartilage destruction, and subluxation
were present on the non-paralyzed side and
absent on the paralyzed side in each
instance. The sheep cell agglutination test
for rheumatoid arthritis was positive in all
patients. In one case quadriceps muscle and
synovium were biopsied from both thighs
and both knees. Histologic findings consistent with rheumatoid arthritis were seen in
both biopsies from the right (unparalyzed)
side and very little histologic change on the
paralyzed side. This phenomenon supports
the view that rheumatoid arthritis is a
diffuse disease with a tendency to local
inflammation in synovium; whatever factors
lead to this are effectively opposed in a
paralyzed arm or leg.
A lower motor neurone lesion preceding
development of Heberden’s nodes protected the involved side in cases reported
by McEweq8 Hench,” and Stecher and
Karnosh.lo Most recently Kamerman’l reported the case of a 57-year-old woman
who sustained a comminuted fracture of the
right elbow, ultimately resulting in complete ulnar nerve palsy and incomplete
radial nerve palsy; six years later she developed rheumatoid arthritis, but no signs
of the disease appeared on the right,
though there was diffuse involvement elsewhere. The sheep cell agglutination test for
rheumatoid factor was positive and there
was radiologic evidence of disease of the
left hand.
Our case confirms that joints in hemiplegic limbs do not develop rheumatoid
arthritis as do those of non-hemiplegic
limbs. There is no evidence that stroke will
affect rheumatoid changes already present
when hemiplegia occurs.
The mechanism by which hemiplegia
protects against local development of rheumatoid arthritis in the paralyzed limb is not
clear. It cannot be due wholly to forced immobilization of paralysis; our patient was
partially ambulatory to the time of her
death. Ambulation was maintained over the
thirteen years’ followup with the aid of a
long leg brace, canes and crutches. With
help at home and strong rehabilitative support she could carry out most activities of
daily living. Even though Thompson and
BywatersY7case 3 had been ambulatory and
working full time for 32 years since his
stroke, protection against rheumatoid arthritis was still complete. Jacqueline’sa patient had been active and working for 49
years following his stroke; his protection
was complete. Eva, if the limb is completely paralyzed, gravity continues to act on it;
thus, a paralyzed limb is never weightless.
Spastic paralysis may, in fact, induce more
than normal forces on joints, ligaments and
tendons. Vascular tone and vessel size are
commonly compromised; loss of skeletal
muscle tone may occur, reducing mechanical forces operating on joints.
The effectiveness of optimum rest programs and therapeutic splinting suggests
that diminished, if not absent, motion plays
a therapeutic role. Complete disuse of an
extremity may be the central factor in preventing arthritis. Connective tissue metabolism is grossly altered by immobilization.
Fibroblasts de-differentiate, losing their
long processes and endoplasmic reticulum,
becoming morphologically the fibrocyte, a
nucleus, a thin cytoplasm and a few
processes. Normally, with wounding or
gross mechanical stimulus, fibroblasts increase metabolic activity, rapidly developing a large endoplasmic reticulum that
initiates synthesis of fresh collagen and
polysaccharide. Relative metabolic inertia
of an immobilized limb could play a part in
the response to an inflammatory stimulus;
thus, the disease may be minimally expressed on the immobilized side and
maximally evident on the non-paralyzed
Some of these cases provide indirect support for the view that rheumatoid arthritis
is a diffuse disease of small blood vessels,
capillaries, venules and arterioles, and that
the synovial involvement is a localized,
intense, symptomatic and destructive feature, perhaps attracting attention and
thought from the real etiologic issues. In
our patient dorsalis pedis and posterior
tibia1 pulsations were absent on the left
(paralyzed) side, though they were readily
palpable on the non-paralyzed side. Bywaters12 showed in an arteriographic study
that patients with nodular, seropositive
rheumatoid arthritis frequently manifest
blocks and obstructions in small vessels in
finger pulp tissue and in distal small vessels,
again supporting the concept that the early
and sustained lesions of rheumatoid arthritis are capillaritis, venulitis and arteriolitis, as proposed by Sokoloff.13 Bywaters12
did an arteriogram in his case 1 and showed
expected diminished caliber of the large
vessels on the paralyzed side only (secondary to loss of innervation); there were,
however, clearly small vessel blocks and
failure of filling of finger pulp tissue on
both sides, suggesting that both sides are
involved by the primary lesion but only the
normal side by the localized joint manifestation. Hess and Z814 have shown
that rheumatoid macroglobulin (rheumatoid factor) may complex on vascular endothelium and possibly produce changes
in small vessels. Further such studies are
important in patients with stroke and rheumatoid arthritis, irrespective of the order
in which the diseases are acquired.
Our patient developed a rheumatoid
nodule on the extensor surface of the nonparalyzed forearm only. This clinical observation suggests either that the paralyzed
side was not as subjected to micro-traumata
as the right side, or that the tissues were
not as responsive to the forces producing
rheumatoid nodules. Sokoloff proposed that
rheumatoid nodules are reflections of the
small vessel disease.13 The latex hation test
in our patient was repeatedly negative, an
unusual finding in nodular rheumatoid arthritis. However, we noted in a previous
study four instances of nodules in rheumatoid arthritis in seronegative patients.15
Since Kamerman’s reportll we know that
a lower motor neuron lesion will also protect against rheumatoid arthritis. The site
of the neurological deficit thus does not
seem to be the decisive factor.
The normal hand in the paralyzed hemiplegic patient must take on much more
work. Excessive use of the connective tissues of the normal hand might accelerate
and intensify the process in joints on that
side, suggesting that the protective effect
of the paralysis is due to disuse rather than
absent innervation. No known neurological
or vasomotor effect explains the protective
force. We believe it is due to disuse and
immobilization, directly or indirectly.
A thirteen-year followup study is presented of a case of right cerebral thrombosis
with left hemiplegia followed in four months by development of severe nodular, seronegative rheumatoid arthritis. The joints of the paralyzed limb did not develop rheumatoid arthritis, while those of the normal side became severely involved. It is suggested
that the sparing, protective effect of hemi legia on rheumatoid arthritis is most likely
due to the enforced immobility of the Em.! Possible mechanism by which hemiplegia
protects and its biological implications are discussed.
Very important etiologic and therapeutic implications may be “hiding in plain
sight” in the interpretation of data from such experiments of nature.
Es presentate un tudio catamnestic de 13 annos de duration relative a un caso de
thrombosis dextero-cerebral con hemiplegia sinistre, sequite quatro menses plus tarde
per le disveloppamento de sever nodular arthritis rheumatoide a seronegativitate. Le
articulationes del paralysate extremitate non disveloppava arthritis rheumatoide, sed
le articulationes del latere normal esseva gravemente &cite. Es suggestionate que
le effecto protective de hemiplegia contra arthritis rheumatoide es probabilissimemente
le effecto del forciate immobilitate del extremitate. Es commentate le possibile subjacente mechanismos e lor inherente significationbiologic.
I1 pare ben possibile q u e irnportantissirne connexiones de signification etiologic e
therapeutic es “celate in plen vista” i n le interpretation de datos a b tal experimentos
del natura.
1. Friedman, H. H., Schwartz, S., Trubek, M.,
and Steinbrocker, 0.: The “Pararheumatic” Arthropathies. Ann. Intern. Med. 38: 732-758, 1953.
2. Head, H., and Holmes, G.: Sensory disturbances from cerebral lesions. Brain 34: 102-254,
3. Coste, F., and Forestier, J.: Hemipligie et
nodosites d’Heberden controlaterales. Bull. SOC.
Med. Hop. Paris 51: 772-776, 1935.
4. Forestier, J.: Rheumatism d’Heberden, chez
une hemiplegique lesions articuliares limitbes au
cBtb sane. Rev. Neurol. 63: 442-448, 1935.
5. Winter, S.: Unilateral Heberden’s nodes in a
case of hemiplegia. New York J. Med. 52: 349359,1952.
6 . Jacqueline, F.: A case of evoluted polyarthritis with localization controlateral to a hemiplegia.
Rev. Rhum. 20: 323-324,1953.
7. Thompson, M., and Bywaters, E. G. L.: Unilateral rheumatoid arthritis following hemiplegia.
Ann. Rheum. Dis. 21: 370-377,1962.
8. McEwen, C.: Heberden’s nodes: Heredity in
hypertrophic arthritis of the finger joints. (A Discussion). J. A. M. A. 115: 2024, 1940.
9. Hench, P. S.: Heberden’s nodes: Heredity in
hypertrophic arthritis of the finger joints. (A Discussion). J. A. M. A. 115: 2025, 1940.
10. Stecher, R. M., and Karnosh, L. J.: Heberden’s nodes. VI. The effect of nerve injury upon
the formation of degenerative joint disease of the
fingers. Amer. J. Med. Sci. 213: 181-191, 1947.
11. Kamerman, J. S.: Protective effect of traumatic lesions in rheumatoid arthritis. Ann. Rheum.
Dis. 25: 361-363. 1966.
12. Bywaters, E. G. L.: Peripheral vascular obstruction in rheumatoid arthritis and its relatienship to other vascular lesions. Ann. Rheum. Dis.
16: 84-103, 1957.
13. Sokoloff, L.: The pathophysiology of peripheral blood vessels in collagen diseases. In Orbison, J. L. and Smith, D. E., Eds.: The Peripheral
Blood Vessels. Baltimore, Williams & Wilkins,
1963, pp. 297-325.
14. Odone, D. T., Wilson, A. F., and Engleman,
E. P.: Annual meeting of the American Rheumatism Assoc. Bull. Rheum. Dis. 11: 227-229, 1960.
15. Bland, J. H., and Brown ,E. W.: Seronegative and seropositive rheumatoid arthritis. Ann.
Intern. Med. 60: 88-94, 1964.
demand critical interpretation: they consist often of chance coincidences and in the study of such a coincidence it
is important to be sure that the peculiar phenomenon it is said to exemplify is not itself merely
the chance choice of an obsessive viewpoint.
This interesting case justifies itself, first, because of
the symmetrical nature of non-hemiplegic rheumatoid arthritis, and secondly because there is a
background of other observed cases, all pointing
in the same direction. The ideal study is one in
which all known instances of such a coincidence
are considered together: the recent account by
Glick (1967) of 13 cases of poliomyelitis later affected by rheumatoid arthritis falls into this category. While there was no absolute protection in
the paralyzed joint, there was enough evidence in
all his examples to conclude that “joints controlled
by paralyzed muscles show less involvement and
destruction” and also that “subcutaneous nodules
did not appear on the paralyzed limbs,” although
they appeared in 3 out of the 5 seropositive cases
in the unaffected areas. Even in rheumatoid arthritis without hemiplegia the elbow’ nodule occurs
always on the side of the least affected shoulder.
The nodule is the most specific lesion of rheumatoid arthritis and may be regarded as “rheumatoid
arthritis in miniature.” What is odd about the
present case is that latex fixation tests for rheumatoid factor were negative: in our experience this
is a rare and always transient phase.
In this case and in others, function has been regained to a considerable extent on the paralyzed
side and yet it remains relatively (but not always
entirely) free from the overt clinical and radiological manifestations of rheumatoid arthritis, both in
upper motor neurone lesions, in poliomyelitis and
in the unique case of peripheral nerve injury recently recorded by Kamerman (1966). As Kamerman, Glick and Thompson and Bywaters (1962)
have each concluded, i t is the relative disuse of
the affected limb which seems to protect it, rather
than any obscure neurological mechanisms. How is
this mediated? It cannot be lack of motion: Glick
suggests that decreased intra-articular pressure may
be an important factor. This depends on muscle
action and may reach well over arterial pressures
(Caughey and Bywaters, 1963) being responsible
for the gross radiological changes with intraosseous
cyst formation and Baker’s cysts seen in “people
who have little pain, strong muscles and heavy
jobs” (Bywaters, 1964), a view confirmed by Castillo et al. (1965.)
Even so, it is no therapeutic solution to splint
and immobilize the rheumatoid joint: a vegetable
existence may be unilaterally inescapable for a
hemiplegic, but his life and his function depend
on his moveable side, rheumatoid or not.
Taplow, England
Glick, E. L.: Asymmetrical rheumatoid arthritis
after poliomyelitis. Brit. Med. J. 3: 26, 1967.
Kamerman, J. S.: Protective effect of traumatic
lesions on rheumatoid arthritis. Ann. Rheum. Dis.
25: 361, 1966.
Thompson, M., and Bywaters, E. G. L.: Unilateral rheumatoid arthritis following hemiplegia.
Ann. Rheum. Dis. 21: 370,1962.
Caughey, D. E., and Bywaters, E. G. L.: Joint
fluid pressure in chronic knee effusions. Ann.
Rheum. Dis. 22: 106, 1963.
Bywaters, E. G. L.: In Radiological Aspects of
Rheumatoid Arthritis, 1964, p .52.
Castillo, B. A,, E l Sallab, R. A., and Scott, J. T.:
Physical activity, cystic erosions and osteoporosis
in rheumatoid arthritis. Ann. Rheum. Dis. 24: 522,
THE OCCURHENCE of pauci-articular and of unilateral rheumatoid arthritis raises the perplexing
question of the localization of rheumatoid inflammation, and reminds us how difficult it is to speculate meaningfully concerning pathogenesis of a
disease when one is ignorant of its etiology. But
since I agree that cases such as the one so well
described by Drs. Bland and Eddy may provide
an important clue “hiding in plain sight,” I shall
seek out an arm-chair and proceed.
It seems to me crucial to determine just how
normal the clinically uninvolved junctures of the
hemiplegic arthritic really are. We are all aware
that histological examination of a number of different joints (e.g. the distal inter-phalangeal,
sterno-clavicular) often reveals impressive arthritic
changes despite the fact that there may have been
little or no trouble with these parts intra uitem.
If one were to find evidence of at least minor
involvement of the joints on the hemiplegic side
(which will be the case, I suspect) then we have
an experiment of nature which seems to tell us
that relative inactivity of a joint is in some way
(?vascular, ?chemical, ?physical) capable of dampening the local inflammatory response characteristic
of rheumatoid arthritis. This should not be sur-
prising, perhaps, in view of the frequency which
patients report a transient worsening in symptoms
following strenuous exertion.
If, on the other hand, the joints on the hemiplegic side are entirely normal histologically, then
the mystery is even deeper, since we do not know
whether there is a causative micro-organism in RA
which is actually present in the joints, or if arthritis may not be the result of some secondary
(?immunologic) disturbance such as has been postulated to be responsible for lupus nephritis and
rheumatic carditis. I t is possible that the localization of rheumatoid inflammation may be connected
with peculiarities in the structure of synovial blood
and lymphatic vessels (a mechanical factor leading
to entrapment of particulate matter). Alternatively,
rheumatoid inflammation may occur where it does
because there is a chemical or immunological bond
between the synovium and some microorganism or
its products. To b e suie, there are several other
hypotheses, (equally attractive and equally unburdened by any facts), including, very likely, the
correct one.
Pittsburgh, Penna.
for the astute comments of
Professors Bywaters and Rodnan and agree with
Professor Bywaters that to practice and promote
inertia in the unilaterally rheumatoid joints of the
hemiplegic is without merit. The patient’s clinical
problem is not that of the non-stroke patient.
Movement and optimum use of his rheumatoid
non-paralyzed extremities are his salvation. Professor Bywaters cautions that a peculiar phenomenon
may be merely a reflection of the preconceived observation, the made-up mind; oppositely, tonguein-cheek may lead to foot-in-mouth.
Click‘s report of sparing of joints in patients
with rheumatoid arthritis with preceding residual
paralysis of poliomyelitis suggests the protective
paralysis is nonspecific. Six of his eleven cases
were seronegative and nodules formed only in
the non-paralyzed side, as in our case.
Deo uolente, we hope for another opportunity
to study a similar patient and “next time” we
would do the following studies:
1. Synovianalysis on fluid from both knees;
search for “ragdcytes” in joint fluid.
2. Latex fixation tests on both sera and joint
3. Biopsy synovial membrane from joints on
both sides and study by light and electron microscopy:
a. Magnitude of plasma cell proliferation and
development of lymphoid germinal centers from
both sides.
b. Degree of development and proliferation
of villi and “buds.” We think this approaches
specificity in rheumatoid arthritis.
c. Proliferation of A and B cells of synovium
with search for the electron-dense round bodies
(residual bodies) described in Barland, P., Novikoff, A. B., and Hamerman, D.: Electron microscopy of the human synovial membranes. J.
Cell. Biol. 14: 207, 1962.
The phenomenon is an important biological
event, worth making small waves about (not unsound waves). The understanding of sparing of the
joints on the paralyzed side is probably less than a
light-year away.
Biirlington, Vermont
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hemiarthritis, rheumatoid, hemiplegia
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