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Transverse myelitis and acute central nervous system manifestations of systemic lupus erythematosus.

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BRIEF REPORT
TRANSVERSE MYELITIS AND ACUTE CENTRAL NERVOUS SYSTEM
MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS
ROBERT W. WARREN and DEBORAH W. KREDICH
Central nervous system (CNS) manifestations
of systemic lupus erythematosus (SLE) in adults and
children are common, occurring in up to 45% of
patients (1). Conversely, transverse myelitis in SLE is
rare, and with the current therapy, usually is fatal or is
followed by prolonged paralysis (2,3). This case report
is the first that describes an SLE patient with transverse myelitis successfully treated with extremely
high-dose steroids.
Case report. A 14-year-old girl came to Duke
University Hospital in 1979 with panniculitis, fever,
and hepatosplenomegaly; SLE was diagnosed with
additional findings of thrombocytopenia, alopecia,
encephalopathy, elevated serum anti-DNA antibody
(12 pg DNA bound/ml serum, normal <9), and a
positive fluorescent antinuclear antibody (1 :40). Specific antibodies to SS-A, SS-B, Sm, and RNP were not
found.
Over the next 2 years she had seizures and
migraine headaches, pericardial effusion, vasculitic
skin lesions, arthritis, hypertension, dysfibrinogenemia, malar erythema, and proteinuria up to 5.7 gm/
day. Drug therapy included daily prednisone, a brief
course of azathioprine, hydroxychloroquine, and aspirin.
From the Section of Pediatric Rheumatology, Department
of Pediatrics, Duke University Medical Center, Durham, North
Carolina, and the Division of Rheumatology and Immunology,
Department of Pediatrics, University of North Carolina, Chapel
Hill.
Supported in part by USPHS training grant 2T32 AI07217.
Robert W. Warren, MD, PhD; Deborah W. Kredich, MD.
Address reprint requests to Robert W. Warren, MD, PhD,
Department of Pediatrics, Clinical Sciences 229H. University of
North Carolina at Chapel Hill, Chapel Hill, NC 27514.
Submitted for publication September 27, 1983; accepted in
revised form April 12, 1984.
Arthritis and Rheumatism, Vol. 27, No. 9 (September 1984)
When admitted to Duke Hospital in October
1981 she was receiving 50 mg prednisone per day.
Findings included normal vital signs, arthritis, moderate proteinuria, malar erythema, moderate hepatosplenamegaly, and hypertension. The mental status
examination results suggested depression and tangential and dissociative thought patterns. No appreciable
muscle weakness or sensory loss was noted. Initial
examination revealed hematocrit 37%, white blood
count 1,400, Westergren erythrocyte sedimentation
rate (ESR) 13 mm/hour, and anti-DNA 0 pglml with
serum total hemolytic complement (CH50) 56 units
(normal 32-46). Her creatinine clearance was normal,
and her urine was without sediment abnormalities.
Electroencephalogram (EEG) results were normal.
Cerebrospinal fluid (CSF) examination was remarkable for slightly depressed glucose levels and fourth
component complement (C4) (see Table 1); C S F bacterial, fungal, mycobacterial, and viral cultures were
then, and subsequently, negative. A brain computerized tomography (CT) scan revealed perisulcal atrophy, mildly increased from prior studies.
Initially, therapy was not altered, but 10 days
after admission the patient became lethargic and dysarthric, and 1 day later she was found stuporous,
paraplegic, and incontinent. Findings included a TI0
sensory level, and bilateral sustained ankle clonus and
Babinski responses. ESR was then 10 mmhour, antiDNA 7 &mi; CH5O and muscle enzyme levels were
normal. CSF studies, given in Table 1, revealed a
further decrease in glucose. The brain CT result was
unchanged; myelogram results were normal. EEG
showed diffuse slowing at 6-7 Hz, consistent with
severe encephalopathy .
Intravenous (IV) methylprednisolone, 250 mg
every 6 hours, was started. Within 36 hours of initia-
1059
BRIEF REPORTS
Table 1.
Cerebrospinal fluid (CSF) studies of patient with systemic lupus erythematosus transverse
myelitis
Date
1/13/81
10/29/81 (admission)
11/9/81 (with onset of
m yelopath y)
1211818 1 (discharge)
CSF
white
blood
cells/
mm’
1
I
0
1
CSF
glucose,
mu0
Serum
glucose,
mgYc
CSF
protein,
mg%
CSP
c4
units
Serum
c4
units
41
34
95
83
26
26
160
128,000
24
80
93
158
22
35
320
64.000
tion of IV steroids, voluntary toe movement and bowel
and bladder control had returned. Lower extremity
weakness slowly diminished, and 1 week later the
patient was ambulating with assistance. Her dysarthria, sensory loss, and previous aberrant behavior
concomitantly disappeared. Subsequent findings included a negative assay for anti-DNA antibody, improvement in CSF test values (Table l ) , and resolution
of the diffuse slowing on EEG by 4 weeks.
The steroid was tapered very slowly until an
oral dosage of prednisone 40 mg twice a day was
reached. Azathioprine 100 mg daily was added before
she was discharged.
Discussion. Approximately 50% of the patients
with SLE have CNS symptoms, with frequent reports
of headache, psychosis, and seizures. While seizures,
coma, or transverse myelitis immediately suggest a
diagnosis of CNS lupus, neurologic symptoms are less
dramatic in most SLE patients and may also be
attributable to situational or steroid-induced psychiatric problems, or to infectious etiologies. Laboratory
studies may not be helpful, particularly since antiDNA antibody and CH5O alterations may not reflect
CNS lupus activity (1). This case is a dramatic example. Indeed, laboratory tests sensitive and specific
enough for CNS lupus remain elusive. The significance of EEG, brain scan, brain CT, as well as spinal
fluid protein, cellular CSF, anti-DNA, antineuronal
antibody, and C4 abnormalities have been recently
reviewed by Hughes (4) and found to be relatively
inconclusive. This SLE patient’s increased perisulcal
atrophy and depressed CSF C4 were strongly suggestive of the diagnosis of a CNS lupus flare; no other
explanation for her CNS symptoms is likely.
The coexistence of severe brain dysfunction
with transverse myelitis has been reported in only I
other SLE patient younger than 18 years of age, who
rapidly succumbed (5). Indeed, while the occurrence is
undoubtedly greater than that in published reports,
only 30 cases of well-documented transverse myelitis
with SLE have been previously reported; 4 patients
were less than 18 years old (1,2,5,6). A review of 26
of these cases has been presented elsewhere (2),
but a compilation of the reported neurologic findings
of all patients (including the present case) is given in
Table 2. Evolution of the neurologic deficit is rapid
and often complete within a few hours. The only
consistent neurologic finding is paraplegia with loss of
sphincter control. Hypoglycorrachia in the absence of
infection may be suggestive, but certainly is not a
sensitive test or predictor of transverse myelitis in
SLE (2). Treatment for transverse myelopathy has
almost always included steroids, though Hachen and
Chantraine (3) concluded that cortisone therapy does
not affect the spinal cord disease; Andrianakos et a1 (2)
suggested benefit if steroids are started within 24 hours
of onset of the myelopathy.
Indeed, despite prednisone therapy ranging to 3
mg/kg/day, 10 of 30 patients died within 6 weeks of
diagnosis; vasculitis with ischemic necrosis and myelomalacia of the cord were prominent postmortem
Table 2. Reported neurologic findings in systemic
erythematosus patients with transverse myelitis*
Finding
Weakness and sensory loss
Impaired sphincter control
Extensor plantar responses
Hyperretlexia
Normal myelogram
Cerebrospinal fluid protein elevation (>40 mg%)
Low cerebrospinal fluid glucose (<30 mg%)
Cerebrospinal fluid leukocytosis (>20 cells)
Cerebrospinal fluid complement depression
lupus
All patients
(n = 31)
31/31
23/23
8118
4/17
819
14/17
6/12
10114
212
* Compilation of data from references I , 2, 5 , 6, and the present
case.
1060
findings (2). Only 4 other patients recovered completely. Of the 4 previously reported pediatric patients, 2
died within 1 month of hospitalization and 2 were
incompletely rehabilitated. Compared with these histories of rapid demise, or very slow and usually
incomplete functional recovery, the experience with
this patient is remarkable. This patient’s rapid recovery from a critical, deteriorating condition was clearly
associated with massive-dose methylprednisolone
therapy. Such a rapid recovery from transverse myelitis with SLE has not been previously reported, though
lupus nephritis (7) and encephalopathy (8) have improved with IV pulse steroid therapy.
The use of very high-dose steroids for acute
idiopathic transverse myelitis has been recently described (9). Two of 3 adult patients treated with
massive doses of IV methylprednisolone had substantial improvement within 96 hours. Like SLE transverse myelopathy, idiopathic myelitis usually carries a
high morbidity, with less than 15% of patients ambulatory by 3 weeks (10). Parallel rapid improvement in
both myelopathies suggests that the initial mechanism
of steroid activity may be to reduce tissue edema and
inflammation, perhaps preventing further secondary
damage from the primary process. Suppression of an
aberrant immune response would be an expected
mode of steroid activity in SLE transverse myelitis.
Underscoring the high morbidity and mortality
of transverse myelopathy in lupus, this report raises
the possibility that very high-dose IV steroid therapy
may improve prognosis. Given the low incidence of
transverse myelitis in SLE, a multicenter prospective
controlled study will be required for further assessment.
BRIEF REPORTS
Acknowledgments. We thank Drs. Darrell Lewis and
Andrew Hodson for neurologic consultation and Ms Maureen Oakes for secretarial assistance.
REFERENCES
1. Yancey CL, Doughty RA, Athreya BH: Central nervous
system involvement in childhood systemic lupus erythematosus. Arthritis Rheum 24: 1389-1395, 1981
2. 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
3. Hachen HJ, Chantraine A: Spinal cord involvement in
systemic lupus erythematosus. Paraplegia 17:337-346,
1979
4. Hughes GR: Central nervous system lupus-diagnosis
and treatment. J Rheumatol 7:405-411, 1980
5. Penn AS, Rowan AJ: Myelopathy in systemic lupus
erythematosus. Arch Neurol 18:337-349, 1968
6. Scharf I, Nahir M, Hemli J: Transverse myelitis with
systemic lupus erythematosus. J Neurol 215:23 1-232,
1977
7. Kimberly RP, Lockshin MD, Sherman RL, McDougal
JS, Inman RD, Christian CL: High dose intravenous
methylprednisolone pulse therapy in systemic lupus
erythematosus. Am J Med 70:817-824, 1981
8. Eyanson S, Passo MH, Aldo-Benson MA, Benson MD:
Methylprednisolone pulse therapy for nonrenal lupus
erythematosus. Ann Rheum Dis 39:377-380, 1980
9. Dowling PC, Bosch VV, Cook SD: Possible beneficial
effect of high-dose intravenous steroid therapy in acute
dernyelinating disease and transverse myelitis. Neurology 30:33-36, 1980
10. Ropper AH, Poskanzer DC: The prognosis of acute and
subacute transverse rnyelopathy based on early signs
and symptoms. Ann Neurol 451-59, 1978
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