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Transverse myelitis and optic neuritis in systemic lupus erythematosusa case report with magnetic resonance imaging findings.

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We describe a patient with systemic lupus
erythematosus who developed transverse myelitis and
optic neuritis. Magnetic resonance imaging showed the
presence of an abnormal signal in a normal-sized spinal
cord which corresponded to the patient’s neurologic
deficit. No abnormality was recognized in either optic
nerve. Magnetic resonance may prove to be a useful
imaging modality for the diagnosis of a transverse
myelopathy in systemic lupus erythematosus.
Transverse myelitis and optic neuritis are uncommon, though previously recognized, central nervous system (CNS) manifestations of systemic lupus
erythematosus (SLE) (1-8). Two previous reports
have shown an association of these 2 conditions (Devic’s syndrome) in SLE (9,lO).
We describe a patient with known SLE who
developed transverse myelitis and subsequent blindness secondary to optic neuritis. Magnetic resonance
imaging (MRI) delineated spinal cord abnormalities
consistent with a myelopathy. MRI failed to show
clear abnormalities of either optic nerve; however,
From the Department of Internal Medicine, Divisions of
Rheumatology and Nephrology, and the Department of Radiology,
Creighton University School of Medicine, Omaha, Nebraska.
Jay G. Kenik, MD: Assistant Professor, Department of
Medicine, Division of Rheumatology ; Kelly Krohn, MD: Resident
in Internal Medicine; Richard B. Kelly, MD: Resident in Radiology;
Martin Bierman, MD: Assistant Professor, Department of Medicine,
Division of Nephrology; Michael D. Hammeke, MD: Assistant
Professor, Department of Medicine, Division of Nephrology; John
A. Hurley, MD: Assistant Professor, Department of Medicine,
Division of Rheumatology.
Address reprint requests to Jay G. Kenik, MD, Department
of Medicine, Creighton University School of Medicine, 601 N. 30th
Street, Omaha, N E 68131.
Submitted for publication September 22, 1986; accepted in
revised form December 4. 1986.
Arthritis and Rheumatism, Vol. 30, No. 8 (August 1987)
focal cerebral lesions similar to those previously reported in SLE were identified (1 1,12).
Case report. SLE was first diagnosed in the
patient, a 27-year-old black woman, in December
1981. Her symptoms included pleuritic chest pain and
arthritis. Leukopenia and antinuclear antibodies were
present. In June 1982, she developed nephritis. A renal
biopsy demonstrated diffuse proliferative glomerulonephritis, and a regimen of 60 mg of prednisone per
day was begun. Azathioprine and prednisolone intravenous pulse therapy were later added to control her
nephritis. Her renal function gradually deteriorated:
Her creatinine level reached 5.0 mg/dl several weeks
before the present admission.
In January 1986, she presented to the emergency room with the complaint of vague abdominal
pain and a 6-hour history of progressive leg weakness.
On examination, optical fundi, visual acuity, and visual fields were normal. Neurologic examination revealed a clear mental status, and cranial nerves 2-12
were intact. A flaccid paralysis of her lower extremities was present, and the sensory deficit was consistent with a T3/T4 level.
The patient underwent immediate myelography ,
which showed no obstructive defect. Cerebrospinal
fluid (CSF) showed 1,870 fresh red blood cells/mm3,
1,590 white blood cells/mm3 with 89% polymorphonuclear leukocytes (PMN), protein level of 426 mg/dl,
and glucose level of 53 mg/dl (simultaneous serum
glucose 148 mg/dl). Gram stain of the CSF was negative for bacteria. Computed tomography (CT) of the
head, which was done without contrast injection,
showed no lesions or atrophy, and the results were
interpreted as normal.
Pertinent laboratory test results included:
Figure 1. T2-weighted magnetic resonance image, demonstrating the normal appearance and signal
intensity of the optic nerves (arrows).
Wesfergren erythrocyte sedimentation rate 13I mm/
hour, blood urea nitrogen 71 mg/dl, creatinine 7.0
mg/dl, white blood cell count 4,170/mm3 (70% segmented neutrophils, 2% bands, 15% lymphocytes,
12% monocytes, 1% eosinophils), hemoglobin 10.2
gm/dl, and hematocrit 30.2%. Serum complement levels were: C3 108 mg/dl (normal 88-244), C4 11 mg/dl
(normal 16-66), and CH5O 32 unitdm1 (normal 28-84).
The anti-native DNA percentage of binding was 23
analysis of CSF 8 hours later
(normal 4)Repeat
showed a white blood cell count of 1,430/mm3 (89%
PMN, 5% monocytes, 6% lymphocytes), glucose level
of 33 mg/dl (serum glucose 120 mg/dl), and protein
value of 705 mg/dl. Counterimmunoelectrophoresis to
detect bacterial antigens was negative. Routine cultures of the CSF, as well as acid-fast and fungal
cultures, cryptococcal antigen, and VDRL, were all
negative. N o further immunoglobulin profiling was
done on either CSF sample.
The patient was treated with parenteral methylprednisolone, 100 mg/day, in divided doses. Her motor
and sensory deficits stabilized to approximately the T3
level during the first week of admission. One week
after ,admission, she had a sudden loss of vision in the
right eye. On examination, her right pupil did not react
to light. The fundus was normal. Visual evoked potentials showed no visual impulse transmission in the
right eye and delayed transmission in the left eye,
which was consistent with a prechiasmal defect. On
the fifteenth day of hospitalization, she developed a
similar loss of vision in the left eye. Cyclophosphamide (100 mg/day) was added to the steroid
regimen, and the azathioprine was discontinued. An
MRI study of the head, spinal cord, and optic nerves
was obtained. Results of the study of the optic nerves
were normal (Figure 1). MRI evaluation of the brain
(Figure 2) demonstrated multiple small areas of abnormal signal intensity in the periventricular white matter,
which were believed to represent microinfarctions. A
large area of abnormal signal in the left parietal occipital region was consistent with a typical corticocerebra1 infarction. MRI of the spinal cord demonstrated a
zone of abnormal signal in the lower cervical and
upper thoracic region (Figure 3), encompassing the
level of the patient’s deficits.
The patient’s motor deficits have persisted
without improvement, but her vision has shown almost total recovery over a 2-month period.
Discussion. Transverse myelitis and optic neuritis are both uncommon, and potentially devastating,
manifestations of SLE. The incidence of transverse
myelitis has been reported to be <4% of those patients
with neuropsychiatric manifestations of SLE (13). In
these patients, the acute onset of major motor deficits
has been shown to be caused most often by cerebral
infarctions or hemorrhage. The CSF findings in our
patient, which consisted of a pleocytosis, elevated
protein value, and reduced glucose level, are well
recognized in transverse myelitis (2). The marked
elevations in the CSF white blood cell count and
protein levels in this patient were suggestive of
The presentations of transverse myelitis range
from mild paresis and sensory deficits to complete
sensorimotor paralysis. In a review (3), 50% of patients initially diagnosed as having an acute myelopathy or myelitis were shown to have an anatomic mass
Figure 3. T2-weighted midline sagittal image of the spinal cord,
demonstrating a zone of abnormal increased signal in the lower
cervical and upper thoracic region (between arrowheads) encompassing the level of the patient’s transverse myelitis. The signal
intensity of the cord below this region (arrow) appears normal.
Figure 2. T2-weighted magnetic resonance image of the brain at the
level of the lateral ventricles, demonstrating 4 separate areas of
increased signal (arrows), presumably representing vasculitisinduced infarctions.
lesion; thus, the need for early diagnostic studies is
clear. Those patients presenting with symptoms of
back pain associated with motor loss tended to have a
poorer neurologic recovery.
In a recent study, MRI has shown altered signal
intensity in the spinal cords of 4 patients with multiple
sclerosis (14). In that same study, 2 patients who, on
the basis of clinical and CSF findings, were suspected
of having myelitis had normal MRI results. We found
no previous report documenting MRI findings in transverse myelitis in SLE. It is not surprising that ischemic
insults to the spinal cord result in an altered signal
intensity similar to that found in the brain. The pies‘If an
in a
renders the Possibility of a surgically treatable lesion
Before the advent of MRI, CT had been the
imaging method of choice in investigation of CNS
manifestations in patients with SLE. Two preliminary
reports suggest improved detection, sensitivity, and
accuracy in delineating lesions in the brain with the
use of MRI, as compared with CT (11,12). In several
patients with normal results on CT scans (including
our patient), MRI demonstrated lesions, and in those
patients with abnormal CT scan results, MRI demonstrated lesions more accurately and revealed additional lesions that' were not seen on CT. Typical
images that are recognized include large and multiple
small areas of increased activity in the cerebral white
matter. In addition, focal areas of increased MRI
intensity can be seen in the cortical gray matter.
Interestingly, in 2 patients (1 I), gray-matter lesions
resolved over several weeks, which suggests that
some lesions may not represent true infarctions.
In a review of 12 cases of optic neuritis in SLE
(8), an association with transverse myelitis was noted
in 6. The reason for the concurrence of these 2
uncommon manifestations of SLE is unclear. In addition lo loss of visual acuity, sluggish or absent pupillary responses were often seen. The fundi usually
show typical disc atrophy or edema, but were normal
in 2 of the 12 patients.
The lack of abnormal findings of MRI of our
patient's optic nerves is consistent with the only
previous report of MRI evaluation of optic neuritis, in
which 3 patients all had normal study results (15).
MRI, however, does play an established role in evaluating multiple sclerosis, a common cause of optic
neuritis. As MRI technology evolves, with better
surface coils, improved imaging sequences, and thinner slice capabilities, the diagnostic yield in optic
neuritis may improve.
In conclusion, MRI may prove to be a very
valuable diagnostic method in patients with suspected
transverse myelopathy, and may obviate the need to
use more invasive diagnostic procedures.
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report, systemic, erythematosus, neuritic, findings, optics, lupus, magnetic, imagine, myelitis, case, resonance, transverse
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