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Relapse of lupus transverse myelitis mimicked by vertebral fractures and spinal cord compression.

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BRIEF REPORT
RELAPSE O F LUPUS TRANSVERSE MYELITIS MIMICKED BY
VERTEBRAL FRACTURES AND SPINAL CORD COMPRESSION
ANN K. HENRY and CAROLYN M. BRUNNER
Neurologic involvement during the course of
systemic lupus erythematosus (SLE) has received
increasing attention during recent years. Transverse
myelitis is one of the less frequently encountered
neurologic syndromes; it is rarely mentioned in the
larger series, and there are only 37 cases reported in
the literature (1-7). During the past 10 years, we have
had the opportunity to study 5 SLE patients who
developed spinal cord syndromes, I of whom had
multiple abscesses in the cord at postmortem examination. The remaining 4 patients have had fluctuating
neurologic courses that are similar to those that have
been reported in other patients and that have been ,
independent of other clinical and serologic evidence of
lupus activity. We describe here a patient who had II
transverse myelitis, with relapse after gradual improvement, and an unexpected, non-disease-related
complication that mimicked transverse myelitis.
Case report. A 47-year-old white woman with
SLE was referred to the University of Virginia Hospital on November 27, 1978, because of progressive
weakness in her lower extremities during the previous
48 hours. The diagnosis of SLE was originally made in
May 1958, based on clinical symptoms of polyarthritis,
Raynaud’s phenomenon, and facial rash, with leukopenia and thrombocytopenia. Lupus erythematosus
(LE) cell preparation showed negative results.
From the Department of Internal Medicine, University of
Virginia, Charlottesville.
Ann K. Henry, MD: Fellow, Division of Rheumatology;
Carolyn M. Brunner, MD: Professor of Medicine, Division of
Rheurnatology .
Address reprint requests to Carolyn M. Brunner, MD,
Division of Rheurnatology, Box 412, University of Virginia Hospital, Charlottesville, VA 22908.
Submitted for publication August 6, 1984; accepted in
revised form April 1.5, 198.5.
Arthritis and Rheumatism, Vol. 28, No. 11 (November 1985)
She responded well to treatment with corticosteroids and had no further difficulty until May 1970,
when she suddenly lost vision in her left eye. The
dosage of steroids was increased by her local physician. One month later, she returned for treatment of
pleuritis; no retinal changes were noted at that time.
However, a test for antinuclear antibody (ANA) gave
strongly positive results.
In October 1975, she presented with bitemporal
hemianopsia and paracentral scotomata. Normal results were obtained on the following evaluations:
electroencephalogram (EEG), cerebrospinal fluid
(CSF) analysis, erythrocyte sedimentation rate (ESR),
and CH5O. ANA (1:4) was 4+ and anti-double-stranded DNA (anti-dsDNA), by counterimmunoelectrophoresis, was l + . Again, after the steroid dosage was
increased, her condition improved.
In February 1977, she developed right hemiparesis and dysarthria. Results of EEG, brain scan, CSF
analysis, CH50, and Clq precipitins were normal, and
anti-dsDNA and cryoglobulins were negative. The
ESR of 23 mm/hour at the time of admission decreased
to 4 mrn/hour after a week of treatment with higher
doses of prednisone (60-80 mglday). She gradually
regained her strength, and her speech pattern normalized. She was maintained with prednisone thereapy,
15 mglday.
Two days before admission to our hospital on
November 25, 1978, she developed cramping discomfort in her lower abdomen, which became severe
epigastric pain radiating to her back. Within 24 hours,
she noted unsteadiness of gait, which rapidly progressed to paralysis of her lower extremities, disappearance of abdominal pain, and inability to void. She
denied having symptoms of arthralgias/arthritis, rash,
BRIEF REPORTS
Figure 1. Lateral view of the lumbar spine, showing compression fractures of L1 and L2. Arrow
indicates the posterior border of the displaced L1 vertebra.
or pleuritic chest pain. On examination she had a
flaccid paraplegia with absent deep tendon reflexes,
positive Babinski’s responses bilaterally, and a sensory level at T4 (absent pain and temperature sensation,
with preservation of proprioception and light touch
sensations).
CSF contained 405 mg% of protein, 40 mg% of
glucose (serum glucose 2 hours earlier was 147 mg%),
and 940 white blood cells (WBC)/mm3 (80% polymorphonuclear leukocytes), with negative results on Gram
stain, India ink preparation, and cultures. Wright’s
stain showed many LE cells. Cervicothoracolumbar
myelogram result was normal, although lateral plain
films of the dorsal spine showed partial collapse of T8.
The only abnormal serologic test result was Raji cell
binding of 310 pg equiv of aggregated human gamma
globulin (AHG)/ml (normal <15). The following results were also obtained: anti-dsDNA was negative,
CH5O was 43 units (normal 34-48), urinalysis and
renal function findings were normal, WBC count was
7,200/mm3 (with 9% lymphocytes), hematocrit was
43.7%, and ESR was 30 mm/hour.
Findings were believed to be compatible with
an anterior spinal artery syndrome secondary to active
SLE, and the steroid dosage was increased to 120 mg
of methylprednisolone/24 hours, in divided doses. The
patient was transferred to the spinal cord unit where
she received appropriate physical therapy, and the
steroid dosage was tapered (after 8 weeks, prednisone
dosage was 30 rnglday). The patient’s strength gradually increased, sensory level improved to T7, CSF
protein decreased to 35 mg% within 2 weeks after the
initial evaluation, and within 6 weeks, Raji cell binding
was consistently within normal limits.
In early February 1979, the patient noted diminished strength in both legs and urinary incontinence.
Differential diagnostic considerations included drug
toxicity from baclofen (an antispasmodic), infection,
or exacerbation of SLE. CSF contained 285 mg% of
protein, 29 mg% of glucose (simultaneous blood glucose 60 mg%), and 10 WBCs/mm3 (all mononuclear).
Although the prednisone dosage was increased again,
worsening of her lower extremity weakness with sensory level stable at T7 prompted further investigation.
BRIEF REPORTS
Roentgenograms of the lumbar spine showed
new compression fractures of L1 and L2 (Figure 1). A
myeilogram, with dye introduced from both the C1-2
and L3-4 interspaces, revealed complete block of the
subarachnoid space at T12-L1 due to posterior displacement of the compressed L1 vertebra (Figure 2);
there was no compression at the site of the old T8
fracture. On February 16, lumbar laminectomy was
performed, with placement of a Harrington rod to
stabilize the compression fractures. The patient’s subsequent course was that of slow improvement and
gradual tapering of the steroid dosage.
The patient is currently doing well, although
neurologic deficits remain. She is able to walk unassisted for only short distances, and she uses a walker
or canes. Sensory level (absent pain and temperature
sensation, with decreased light touch and vibratory
sensation) remains at T7; bladder and bowel tone are
decreased.
Discussion. Central nervous system (CNS) involvement in SLE is quite common, with neuropsychiatric manifestations reported in 25-75% of lupus patients. Transverse myelopathy , however, is a rare
manifestation of CNS involvement, with only 37 cases
having been reported in the literature (1-7). In 4 of
these cases (5,8,9), a diagnosis of SLE was made only
by the presence of LE cells or ANA, tests that are now
not considered diagnostic without other manifestations of the disease; the remaining information on
these patients does not allow for a positive diagnosis of
lupus. In 1 other case (lo), a patient with an atypical
picture of slowly progressive, spastic paraparesis
without sensory deficits, the diagnosis of transverse
myelopathy is not clear. Unfortunately, many of the
remaining reports do not provide sufficient data to
adequately review the diagnosis, treatment, and
course of the disease.
Transverse myelitis is defined as “inflammation
involving the entire thickness of the spinal cord, but of
limited longitudinal extent” (Stedrnan’s Medical Dictionary, 23rd edition, 1976). This is a pathologic definition, however, and unless the spinal cord is fully
viewed at autopsy, this entity cannot always be distinguished from other processes. Cord compression by
hematoma, abscess, or tumor (or displaced bone, as
was seen in this case) can present with a clinical
syndrome identical to that of transverse myelitis.
Therefore, the term “transverse myelopathy” is usually more appropriate unless a pathologic study has
confirmed the presence of inflammation.
The diagnosis of CNS lupus presents problems
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Figure 2. Lumbar portion of myelogram (cisterna rnagna dye placement), demonstrating a block caused by the posterior displacement
of the compressed L1 vertebral body (arrow).
that have yet to be resolved, especially when psychiatric manifestations appear without neurologic abnormalities, or when neurologic abnormalities are not
concurrent with extra-CNS manifestations of SLE.
Researchers have proposed the use of a variety of
serologic, CSF, and radiologic tests to confirm a
causal relationship (antineuronal antibodies, lymphocytotoxic antibodies, EEGs, brain scans, oxygen 15
brain scans, computed tomography scans, CSF immune complexes, IgG levels, or complement levels),
but as yet, there are no parameters that are considered
diagnostic.
The diagnosis of transverse myelopathy as a
specific manifestation of CNS lupus is no exception to
this dilemma and depends on the appropriate clinical
picture in a patient with SLE; non-disease-related
etiologies, such as cord compression and CNS infection, need to be excluded by CSF examination and
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myelogram. In at least 3 of the reported cases, transverse myelopathy in SLE was secondary to cord
compression, Irregular filling defects seen on myelogram correlated with clots in the venous system at
autopsy in 1 subject (11). In 2 other subjects (12,13),
cord compression by subdural hematomas was belatedly found at autopsy; myelograms had not been done.
As was seen with the patient presented here, exclusions of other processes must be made with each
episode of myelopathy.
Serologic data which might support the presence of lupus activity, such as serum immune complexes, are not given in the previously reported cases.
Our patient had an interesting pattern of immune
complexes by the Raji cell assay. At the time of initial
presentation, when she was presumed to have had a
lupus-related process, the assay showed immune complexes to be very high at 310 pg equiv of AHG/ml. This
level progressively declined with steroid therapy and
was consistently within the normal range prior to and
at the time of a recurrence of symptoms (this time,
secondary to cord compression). One could speculate
that in this patient, the Raji cell assay was actually
measuring lymphocytotoxic antibodies (14) that were
cross-reacting with neuronal tissue (IS), thus causing
the CNS disease. However, the lymphocytotoxic antibodies identified by the Raji cell assay have been
predominantly IgG, whereas those that cross-react
with neuronal tissue have been reported to be IgM. In
addition, this patient’s peripheral WBC count was not
decreased (although the lymphocyte count was only
648), and results of a Raji cell assay of the CSF were
normal.
Andrianakos et a1 (1) commented on the presence of hypoglycorrhachia during the first 24 hours of
transverse myelopathy in the 3 patients they described
in 1975. Gibson and Myers (2) also noted decreased
glucose in the CSF in 4 of 80 patients with CNS lupus.
In only 6 of the other 30 cases of myelopathy (4,7)
were CSF glucose levels reported; although the levels
all appear to be normal, no simultaneous blood glucose
levels were given, and the time relationships to the
onset of symptoms are unclear. Similarly, it is difficult
to interpret our patient’s CSF glucose levels, which
were obtained after the first 24 hours of symptoms,
without simultaneous serum glucose levels.
Other CSF parameters are not particularly helpful in differentiating lupus myelitis from other myelopathies. Protein levels are usually moderately elevated, and pleocytosis is present in various degrees
(predominantly lymphocytes); these patterns are con-
BRIEF REPORTS
sistent with nonspecific cord damage. In none of the
other case reports were there comments on CSF
immune complexes (our patient had normal levels by
the Raji cell assay, despite an elevated serum level),
CSF IgG (our patient had a normal IgG value of 6.5%
of the total protein), or CSF complement levels (not
measured in our patient). Since the pathologic findings
in this entity have been noted to usually be consistent
with ischemia and to only rarely show a true vasculitis
(infiltration of the vessel wall by inflammatory cells),
immunologic activity in the CSF may well not be
expected to be increased.
The side effects of long-term steroid therapy are
many and can be quite debilitating. The patient presented in this report not only developed osteoporosis
and vertebral collapse, but the resulting spinal cord
compression created a difficult diagnostic problem by
mimicking the transverse myelitis that was initially
seen.
REFERENCES
1 . Andrianakos AA, Duffy J, Suzuki M, Sharp JT: Transverse myelopathy in systemic lupus erythematosus:
report of three cases and review of the literature. Ann
Intern Med 83:616-624, 1975
2. Gibson T, Myers AR: Nervous system involvement in
systemic lupus erythematosus. Ann Rheum Dis 35:398406, 1975
3. Feinglass EJ, Arnett FC, Dorsch CA, Zizic TM, Stevens
MB: Neuropsychiatric manifestations of systemic lupus
erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease. Medicine
(Baltimore) 55:323-339, 1976
4. April RS, van Sonnenberg E: A case of neuromyelitis
optica (Devic’s syndrome) in systemic lupus erythematosus: clinicopathologic report and review of the literature. Neurology 26:1066-1070, 1976
5. Kewalramani LS, Saleem S, Bertrand D: Myelopathy
associated with systemic lupus erythematosus (erythema nodosum). Paraplegia 16:282-294, 1978
6. Thakarar P, Greenspun B: Transverse myelopathy in
systemic lupus erythematosus. Arch Phys Med Rehabil
60~323-324, 1979
7. Hachen HJ, Chantraine A: Spinal cord involvement in
systemic lupus erythematosus. Paraplegia 17:337-346,
1979
8. Granger DP: Transverse myelitis with recovery: the
only manifestation of systemic lupus erythematosus.
Neurology 10:325-329, 1960
9. Penn AS, Rowan AJ: Myelopathy in systemic lupus
erythematosus. Arch Neurol 18:337-349, 1968
10. Fulford KWM, Catterall RD, Delhanty JJ, Doniach D,
Kremer M: A collagen disorder of the nervous system
presenting as multiple sclerosis. Brain 95:373-386, 1972
BRIEF REPORTS
1 1 . Sinkovics JG, Gyorkey F, Thoma GW: A rapidly fatal
c,ase of systemic lupus erythematosus: structure resembling viral nucleoprotein strands in the kidney and
activities of lymphocytes in culture. Texas Rep Biol
bled 27:887-908, 1969
12. Weil MH: Disseminated lupus erythematosus with massive hemorrhagic manifestations and paraplegia. Lancet
7.53358-360, 1955
13. Clinocopathologic conference. Bull Johns Hopkins Hosp
118:423-437, 1966
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14. Anderson CL, Stillman WS: Raji cell assay for immune
complexes: evidence for detection of Raji-directed
immunoglobulin G antibody in sera from patients with
systemic lupus erythematosus. J Clin Invest 66:353-360,
1980
15. Williams GW, Bluestein HG, Steinberg AD: Brainreactive lymphocytotoxic antibody in the cerebrospinal
fluid of patients with systemic lupus erythematosus:
correlation with central nervous system involvement.
Clin Immunol lmmunopathol 18:126-132, 1981
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mimicked, spina, lupus, cord, vertebrate, compression, myelitis, fractured, transverse, relapse
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