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Cranial computed tomography in the diagnosis of systemic lupus erythematosus.

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Cranial Computed Tomography in the
Diagnosis of Systemic Lupus Erythematosus
F. Gonzalez-Scarano, M D , R o b e r t P. Lisak, M D , Larissa T. Bilaniuk, M D ,
Robert A. Zirnrnerrnan, M D , Paul C. Atkins, M D , a n d B u r t o n Zweirnan, MD
~
~~
T h e cranial c o m p u t e d tomograms of 29 patients w i t h systemic lupus erythematosus (SLE) were reviewed. T w e n t y two patients had a clinical course consistent w i t h central nervous system involvement. Of these, 20 had abnormal
C T studies d u r i n g the course of t h e i r C N S symptoms. The most common finding was sulcal enlargement, either
w i t h or w i t h o u t ventricular enlargement, a n d i t was p r o m i n e n t i n patients w i t h either psychosis or dementia.
Infarcts and intracranial hemorrhages were seen as well. Seven CT studies w e r e obtained in SLE patients without a
clear diagnosis of C N S involvement. O n l y o n e of these was abnormal.
Gonzalez-Scarano F, Lisak RP, Bilaniuk LT, et al: Cranial computed tomography in the diagnosis of systemic
lupus erythcmatosus. Ann Neurol 5 : 158-165, 1979
The second leading cause of death f r o m systemic
lupus erythernatosus (SLE) is central nervous system
involvement (CNS-SLE) [ 7 ] .H o w e v e r , t h e diagnosis
o f this complication remains a clinical one, based
u p o n t h e exclusion of o t h e r diseases or d r u g effects
as an explanation for organic CNS s y m p t o m s a n d
signs. Serological activity in blood is nondiagnostic,
a n d a t t e m p t s to utilize cerebrospinal fluid analysis,
arteriography, a n d isotope scanning have b e e n inconclusive [ 3 , 10, 12, 16, 22, 24, 261.
I n a n effort to d e v e l o p a n objective m e t h o d of
diagnosing CNS-SLE, we have retrospectively analyzed c o m p u t e r i z e d axial t o m o g r a m s obtained i n
patients w i t h SLE. A r e p o r t including part of t h e
present patient population has been published previously [4].
Patient S e l e c t i o n and Methods
We reviewed the records of all patients who had cranial
computed tomography (CT) who were referred to the Department of Radiology with the diagnosis of SLE, possible
SLE, or vasculitis involving the CNS. Additional patients
were obtained from the clinical files of two of us (R.P. L.
and B. 2 . ) and through individual contacts with other
members of the Departments of Neurology of the Hospital
of the University of Pennsylvania and the Children’s Hospital of Philadelphia. We identified 29 patients who were
judged to have active SLE by their physicians and who had
been evaluated one o r more times for the possibility of
C N S involvement. O n at least o n e occasion, C T scanning
was included in the investigation. Routine evaluation usually included lumbar puncture and assessment of the activ-
the Departments of Neurology and Radiology
(Neuroradiology) and the Division of Immunology, Department
of Medicine. University of Pennsylvania School of Medicine,
Philadelphia, P A .
From
158
ity of the systemic disease with serum antinuclear antibody
(ANA) assay, measurement of antibody to native D N A ,
and complement levels. Additional studies (arteriography,
electroencephalography, special roentgenography) were
performed when appropriate. Table 1 describes the findings in the patients with CNS-SLE. D N A binding in the
CSF was assayed on 6 patients chosen on the basis of
availability of fluid.
The third ( C 3 )and fourth ( C 4 )complement components
were measured by radial immunodiffusion (Melloy Laboratory, Springfield, VA). Serum was assayed for total hemolytic complement activity (CHSO) using sheep erythrocytes 1141. A N A was measured using indirect immunofluorescence of mouse hepatocytes [2] and antibodies
to native D N A using the radioimmunoassay as modified by
Tala1 and Pillarusetty 1271.
CT scanning was performed with an EM1 head scanner
using a 160 x 160 matrix. The study included the region
from the cranial base to the vertex. The studies were reviewed independently by two neuroradiologists (R. 2 . and
L. B.) who had no knowledge of the clinical histories. The
diagnosis of perisulcal atrophy was based on identification
of enlarged sulci in an amount disproportionate to the patient’s age (Fig l ) . The diagnosis of generalized cerebral
atrophy was based on the additional finding of ventricular
enlargement (Fig 2B).
Results
Clinicdl Symptoms
Thirty-six episodes o f clinical manifestations consist e n t with CNS-SLE w e r e identified i n 22 patients.
S e v e n patients were evaluated o n c e each and w e r e
t h o u g h t e i t h e r n o t to have any evidence for CNS-
Accepted for publication July 5 , 1978.
Address reprint requests to Dr Lisak, Department of Neurology,
Hospital of the University of Pennsylvania, 3400 Spruce St,
Philadelphia, PA 19104.
0364-5134/79/020158-08$01.25 @ 1978 by F. Gonzalez-Scarano
Table I . Clinical Findings in 22 Patients uiith CNS Lidpus
Patient
N o . and
Age (yr)
1, 14
Extra-CNS
Manifestations
of SLE
Arthritis; glomerulonephritis
CNS
Manifestations
Laboratory
Studies”
Seizures
CSF normal,
A N A l:lO,
anti-DNA
Diagnosis
C T Scan
Comments
C N S lupus
Perisulcal atrophy with
diffusely enlarged ventricles
Slight ventricular
enlargement
Treated with anticonvulsants (previously
o n steroids)
9%
2, 24
None
Seizures, psychosis
3,21
Pancytopenia; glomerulonephritis; discoid
lupus; +LE prep;
pleuritis; photosensitivity
Arthritis;
pleuropericarditis;
leukopenia; +LE
prep; oral ulcerations
Seizures, L
hemiplegia
CSF normal,
A N A 1:60,
anti-DNA
5157
CSF normal,
anti-DNA
657, C H 5 0
and C 3 low
C N S lupus
C N S lupus
Perisulcal atrophy and R
thalamic infarc t
Treated with azathinprine and steroids
Seizures
CSF normal,
A N A l:lO,
anti-DNA
60q
C N S lupus
Perisulcal at
rwhy
Arthritis; Raynaud’s
phenomenon; nephritis; rash; hemolyric anemia; thrombocytopenia;
alopecia; oral ulcerations; pleuropericarditis
Hemolytic anemia and
leukopenia; falsepositive RPR; arthritis; pleuritis;
alopecia; glomerulonephritis
Vertigo, episodic
confusion, visual field defect, seizures,
L hemiparesis
and sensory
defect, psychosis
Temporal lobe
seizures,
headaches.
elevated intracranial pressure and
papilledema,
episodic psychosis
CSF TP 86,
A N A 1:40,
anti-DNA
167
C N S lupus
R parietal infarct
with perisulcal
atrophy
Onset of seizures was
during therapy with
moderate doses of
steroids (prednisone,
4 0 mg) for systemic
problems; treated
with increased steroids
Treated with steroids
with good response
Pseudotumor Initial study
normal
cerebri
Arthritis; +LE prep;
leukopenia; thrombocytopenia and hemolytic anemia;
polyserositis
Sudden onset of
coma with a
background of
increasing
hypertension
CSF O P 576,
CSF T P
3 3 , WBC 6
lymphocytes,
A N A 1 10,
anti-DNA
58?,
CH5O and
c 3 low
A N A 1.320,
anti-DNA
12,55
Arthritis; glomerulonephritis; leukopenia
R hemiparesis
with
hypalgesia and
dysarthria, R
homonymous
hemianopia
13,46
Polyarthritis; leukopenia; L hemiparesis
hemolytic anemia;
and hemisenpleuritis; f L E prep;
sory loss,
alopecia; oral ulceraepisodic contions; massive profusion
teinuria
4,33
5,44
10, 16
11.59
C N S lupus
loo%,
CHSO, C3,
and C4 all
low
CSF O P 110,
CSF T P
300, RBC
5 5 00,
A N A 1 10,
anti-DNA
3 3 , c4,
C3, and
C H 5 0 low
CSF normal,
A N A 1 10,
anti-DNA
8656, C 3
and C4 low
Inrracerebra1 hemorthage
C N S lupus
with R
hemispheric
infarction
Steroids begun after
C N S evaluation
O n e year later developed perisulcal atrophy with slightly
enlarged ventricles
L ganglionic
hemorrhage with
intraventricular
rupture
L thalamic hemorrhage
Hemorrhage occurred
while on largc doses
of steroids given for
systemic vasculitis
Normal
Episode occurred while
on steroids; increase
did not affect mental
status
Neurological function
has returned to normal
Gonzalez-Scarano et al: CT Scanning in SLE
159
TabEe I . (Continued)
Patient
No. and
Age (yr)
Extra-CNS
Manifestations
of SLE
14,29
CNS
Manifestations
Laboratory
Studies"
Arthritis; malar rash;
pleuritis; +LE prep;
glomerulonephritis
Confusion, decrease in
memory, and
gait disorder
with incontinence
15.16
Arthritis; leukopenia;
+LE prep; glomerulonephritis; rash
Psychosis, headaches, (?) sixth
nerve palsy
16.44
+LE prep; arthritis;
1972, R
hemiparesis;
1975, confusion; 1976. L
hemiparesis,
confusion
Transverse
myelitis, psychosis, dementia
glomerulonephritis;
+latex fixation
17,29
Alopecia; malar rash;
hemolytic anemia;
nephritis; +LE prep
18.55
Leukopenia; rash; arthralgias; +LE prep;
pleural and pericardial effusion
22,27
Arthritis; malar rash;
photosensitivity;
Raynaud's phenomenon; glomerulonephritis
23, 15
Pericarditis; leukopenia;
glomerulonephritis
24, 58
Lupus anticoagulant
160 Annals of Neurology
Aphasia and R
arm weakness
associated
with hypertension, canfusion
(episodic), L
homonymous
hemianopia
Decrease in
short-term
memory,
paresthesias of
the hands,
proximal muscle weakness,
cotton wool
spots
Seizures, headache with
photophobia
L-sided hyperpathia with
changing deficits over a period of several
days
Vol 5
No 2
Diagnosis
CT Scan
Comments
CSF T P 115,
W B C 13
lymphocytes,
ANA 1 4 0 ,
anti-DNA
23';.
Complement lownormal
CSF normal,
C 3 and
C H 5 0 low,
ANA 1 8 0
CSF normal,
A N A 1 10,
anti-DNA
55c<,
C H 5 0 low
C N S lupus
Cortical atrophy
with ventricular enlargement and basal
ganglia
calcifications
See case report
C N S lupus
Marked perisulcal atrophy
Begun o n prednisone
and azathioprine
C N S lupus,
thrombotic
strokes
R ganglionic
hematoma,
cortical atrophy
Treated with steroids;
attempts to decrease
dose led t o increased
mental confusion
CSF W B C 12
lymphocytes, ANA
1 : 10,
anti-DNA
39ci
C N S lupus
See case report
CSF O P 129,
CSF TP
59, RBC
7200.
WBC 10,
A N A 1%.
anti-DNA
2@:f:
C N S lupus
White matter atrophy with
slightly enlarged ventricles and
prominent
sulci
R occipital
hematoma,
diffuse cortical
and subcortical atrophy
with ventricular enlargement
CSF normal,
A N A 1 10,
anti-DNA
78Cr,
C H 5 0 and
C 4 low
C N S lupus,
mild
proximal
myopathy
Perisulcal atrophy
CSF O P 330,
CSF T P
49, glucose
50, WBC
31, A N A
1:160,
C H 5 0 and
C 3 normal,
positive
India ink
and fungal
cultures
CSF T P 51,
W B C 18,
33'r
PMLs,
A N A 1:20,
anti-DNA
13?i
C N S lupus,
CrYPtococcal
meningitis
Perisulcal atrophy
Patient developed seizures several months
prior to onset of
headaches
C N S vasculitis
Small L caudate
and thalamic
infarct and
cortical atrophy
Steroids given
February
1979
Table I. (Continued)
Patient
No. and
Age (yr)
25,22
26, 17
Extra-CNS
Manifestations
of SLE
Arthritis; +LE prep;
thrombocytopenia;
hemolytic anemia;
alopecia; discoid
lupus; pleuritis
Arthritis; rash;
leukopenia; pleuritis;
+LE prep; hemolytic
anemia
27,39
Arthritis; alopecia
28.46
Pleuritis; +LE prep;
pericarditis; arthritis;
alopecia
29, 15
Sicca syndrome;
leukopenia; pericarditis; +LE prep;
+latex fixation
CNS
Manifestations
Laboratory
Studiesa
Seizures,
CSF T P 389,
pseudobulbar
glucose 50,
palsy with reWBC 5
current aspiration
Seizure disorder,
CSF O P 345,
elevated intraanti-DNA
cranial pres73%
sure
(?pseudotumor)
Migraine headCSF T P 120,
aches, transWBC 333.
verse myelitis,
RBC 8000,
visual field
ANA 1:40,
anti-DNA
defect
3096, C3
low
Aphasia, R
CSF normal,
hemiparesis, R
anti-DNA
homonymous
135%
hemianopia,
affective disorder
Bizarre behavior
CSF T P 150,
with slurred
glucose 40,
speech, deWBC 2,
crease in shortANA
1:1280,
term memory
anti-DNA
24%, C3
and CHSO
low
Diagnosis
CT Scan
Comments
CNS lupus
Cortical atrophy
with ventricular enlargement (3 yr
later)
Perisulcal atrophy and
top-normal
ventricles
Recovered from acute
episode
CNS lupus
Normal
Treated with ACTH and
prednisone with resolution of optic
neuritis
CNS lupus
L posterior frontal infarct,
cortical atrophy with
enlarged ventricles
Cortical atrophy
with enlarged
ventricles
CNS lupus
CNS lupus
Responded rapidly to
moderate doses of
steroids
"Total protein and glucose are given in mgldl, opening pressure in mm H,O
Note: The clinical course of some patients has been condensed.
ANA = antinuclear antibody titer; JX prep = lupus erythematosus preparation; CHSO = total hemolytic complement activity; C3, C4 =
third and fourth components of complement; T P = total protein; RPR = rapid plasma reagin test; OP = opening pressure; WBC = white
blood cells; RBC = red blood cells; PMLs = polymorphonuclear leukocytes.
SLE or to have insufficient evidence of organicity for
a conclusive diagnosis. The neurological symptoms
and signs in the patients with CNS-SLE are summarized in Table 2. Twelve patients (5596) had a
disorder of thought, affect, or cognition, accompanied by seizures in 3 and by corticospinal tract
signs in 6. These constituted the largest diagnostic
category. Intracranial hemorrhage, myelopathy, evidence of brainstem and cerebellar involvement,
pseudotumor cerebri, and visual disturbances represented the remaining CNS manifestations and occurred in a frequency similar to that in other studies
of CNS-SLE [8, 9, 13, 151.
CT Studies
A total of 36 CT scans were performed on all patients with and without clinical CNS-SLE. The abnormalities found in 23 of these studies are listed in
Table 3. In 20 of the 22 patients with CNS-SLE, the
CT study was abnormal during the course of their
CN S symptoms.
Sulcal enlargement with or without ventricular
enlargement was the most frequent finding in these
patients, occurring in 18 of the 22 with CNS-SLE.
Most (11/12) of the SLE patients who had mental
abnormalities (affective or cognitive) as part of their
CNS manifestations showed enlarged sulci on C T
scan. In addition, generalized cerebral atrophy was
documented in an elderly woman with organic brain
syndrome consistent with either senile dementia or
CNS-SLE. Half (9/18)of the patients with large sulci
had seizures, either with or without accompanying
dementia, and about one-tenth (2/18) had rapidly
changing neurological symptoms and signs referable
to widespread areas of the neuraxis.
The development of large sulci as seen on C T scan
occurred in as short a period as five months and was
present within days to weeks after the onset of CNS
Gonzalez-Scarano et al: CT Scanning in SLE 161
Table 2. Central Nem’ous System Inilolwment
in 22 Patients w i t h Systemic Lupu.r Erythematosus
No. of
Patients
CNS Manifestations
F i g 1. CT scan rezeals diifUse siilcaleizlarXet)Ie)Iti n Patient 29.
a 15-year-old girl u’ithabnormal hehaisior and decrease it2
.short-term viemory.
Psychiatric disorder (including organic
brain syndrome)
Seizures
Corticospinal tract signs
Hemianopia
Intracranial hemorrhage
Myelopathy
Brainstem signs and symptoms
Intracranial hypertension (pseudotumor)
Cerebellar syndrome
Optic neuritis
Sensory abnormalities
12
9
8
4
4
3
2
2
1
I
1
Table 3. Feutares of CT Scans in 2.3 Patients ulith SLE”
CT Findings
Patients
with CNS-SLE
Perisulcal atrophy
Generalized atrophy
Infarct
Intracerebral hemorrhage
9
9
4
4
“Some of the scans showed more than o n e diagnostic abnormality.
O n e of the patients was not diagnosed as having CNS SLE.
manifestations in 5 patients. The progression of sulcal enlargement was noted in 3 patients who had
more than o n e study performed, the first being normal and subsequent studies showing perisulcal atrophy (see the case reports that follow).
In addition to sulcal enlargement, 8 patients
showed either infarcts or intracerebral hemorrhages.
Three of the 4 patients with infarcts on CT scan had
an acute neurological syndrome resembling a stroke,
Large sulci were seen in these patients as well. Two of
the 4 patients with intracerebral hemorrhages were
the only patients in this series with thrombocytopenia (Table 2 ) . O n e of the four hemorrhages
occurred in an unusual location (occipital).
Serological and CSF Findings
Findings in serum and CSF studies were not helpful
in defining C N S involvement in these SLE patients.
Fi,@. CT.rcgn.r in a 29-year-old womatz (Patient 17). ( A )T h e
initialexamination is normal. (Bi C T txamination one year
later .ibnws su1(caland t,entrimIdre n h r g e m e n l .
162 Annals of Neurology Vol 5
No 2
February 1979
Thus, patients with CNS-SLE did not show a greater
incidence of serum antibody to native DNA or depression of serum complement (C3, C4, or CH50)
with SLE patients without CNS
when
manifestations.
Elevations of CSF total protein (above 50 mgldl)
occurred in 39% (14/36)of episodes, and mild CSF
pleocytosis was noted in 4276 (15/36), usually in
conjunction with other CNS signs (see the case reports).
We could not demonstrate elevated DNA-binding
antibodies in any of six unconcentrated cerebrospinal
fluid specimens examined, including those of patients
with very high D N A binding activity in simultaneously obtained serum.
Case Reports
The following cases illustrate the progression of CNS
manifestations and perisulcal atrophy in patients with
CNS-SLE.
Patient 14
A 29-year-old woman was well until November, 1971,
when she developed migratory arthralgias and a malar
rash. She was evaluated and found to have antinuclear antibodies, with D N A binding of 45C4. A renal
biopsy showed diffuse membranoproliferative glomerulonephritis. Small doses of steroids (30 mg of prednisone
every other day) were administered through June, 1974,
when she developed a subacute psychosis. During evaluaF i g 3. C T scans in a 29-year-old ioonian (Patient 14). (A)
The
initialexamination is normal. ( B , CT examination after an
inter?al o f f se nionth.c demorfstvates luteral rentricular enlurgrme ii I .
tion in January, 1975, a CSF pleocytosis (13 white cells/
mm3, 95c; mononuclear cells) and a CSF protein of 115
mgldl were noted. T h e CT scan was normal (Fig 3A). Extensive viral, fungal, and bacterial cultures were negative,
and moderate doses of steroids were administered. She
improved initially, then deteriorated again. A decrease in
the dose of steroids was accompanied by improvement in
her mental status.
Between August and November, 1975, she developed
an unsteady gait and numbness and paresthesias of the
lower extremities. She was incontinent of urine. Neurological examination showed pseudobulbar features, a
spastic-ataxic gait, and decreased proprioception in the
toes.
She also demonstrated inattention, decreases in shortand long-term memory, and a reduced fund of knowledge.
A complete myelogram and serum level of vitamin B12
were normal. The CT scan (Fig 3B) showed generalized
atrophy. Steroids were readministered, and her mental disorder became more prominently one of flattening o f affect
rather than difficulty with memory and judgment. There
was no effect on her motor symptoms. A six-week trial of
azathioprine was without benefit.
Patient 17
A 2y-year-old woman had the onset of migratory arthralgias in late 1975. Over the next three months she developed alopecia, a malar rash, weight loss, and a gait disorder. She was evaluated at the Hospital of the University of
Pennsylvania in January, 1976, and a diagnosis of SLE was
made. Neurological examination showed unsteadiness of
Gonzalez-Scarano e t al: CT Scanning in SLE
163
gait and finger-to-nose dysmetria. The CSF examination
showed 12 lymphocytes/mms with negative cultures. The
CT scan (Fig 2A) was unquestionably within normal limits.
She was treated with prednisone for her systemic complaints and showed rapid improvement.
In May, 1976, she was admitted with the sudden onset of
paraparesis and urinary incontinence; a sensory level was
demonstrated in the thoracic region. The CSF examination
and myelography were normal. Her steroid dose was increased, and she showed some improvement in strength
over several months. She remained in stable condition until
January, 1977, when she became increasingly weak again.
She demonstrated indifference to her disability and a flat
affect. Both short- and long-term memory were highly impaired. The CT scan showed generalized atrophy (Fig 2B).
Controls
Six patients with SLE who had systemic clinical and
serological features similar to those of these 2 patients were evaluated for complaints ranging from
headaches to episodic paresthesias and were judged
not to have active CNS-SLE. The CT scan showed
top-normal ventricular size in 2 patients and was
completely within normal limits in the remaining 4.
None of the patients had enlarged sulci.
Discussion
CNS manifestations of SLE have been noted since
the disease was first described, but the accurate diagnosis of CNS involvement remains difficult. The
clinical syndrome is varied and can include dementia
and psychosis, as well as seizures, hemiparesis,
chorea, pseudotumor cerebri, aseptic meningitis, and
rnyelopathy [ I , 5 , 8, 9, 13, 15, 17, 20, 21, 251. Because they are not specific for CNS-SLE, these
symptoms could be due to steroid therapy, hypertension, or coincidental C N S disease (infection, tumor)
in the patient with known SLE. Although none of the
current modes of therapy for CNS-SLE are universally accepted as beneficial, the distinction between
accompanying disease and CNS-SLE may be useful in
the prognostication of the course of the disease.
Therapeutic trials also necessitate accurate diagnosis.
None of the laboratory tests in current use demonstrate any abnormalities that are specific for CNSSLE. Large series have shown that moderate increases
in the amount of total protein in the CSF are common [lo, 261, and low CSF glucose has been described in SLE patients thought to have a related
rnyelopathy [l]. O n e group [ 2 2 ] has reported low
levels of C4 in the CSF of SLE patients with CNS
involvement. However, these authors themselves
have recognized the instability of C4 in CSF, which
makes the assay impractical for clinical use and may
explain the inability of others to confirm these
findings [ 121. Additional attempts at utilizing the CSF
164 Annals of Neurology Vol 5
No 2 February 1979
in CNS-SLE have included immunoglobulin determinations and assays for antibody to D N A .
Conff icting information has been reported from
different centers regarding CSF total immunoglobulin levels [26, 161, and we have been unable to
confirm a recent report [26] of elevated D N A binding antibodies in the unconcentrated CSF of patients
with CNS-SLE.
In the current series, 64% of the patients who
had a CT scan during their first unequivocal episode
of C N S involvement had abnormal findings in that
study. Patients with a sudden episode of neurological
involvement may have a major cerebral infarction or
an intraparenchymal hemorrhage. Large vessel occlusion has been documented with angiography in
patients with SLE [28]. The C T scan may show a
focal region of decreased density (low attenuation
coefficient) that enhances following the injection of
meglumine iothalamate (Conray) in infarcts that are
between one and four weeks old [GI. Intraparenchymal hemorrhages may occur in sites considered
unusual for the hemorrhages associated with hypertension. The CT scan is particularly sensitive in detecting intracranial hemorrhages [ 171.
Patients with SLE who have a chronic or subacute
neurological illness, particularly those with abnormalities of mental status, are likely to demonstrate
perisulcal or generalized cerebral atrophy by CT
scan. The development of sulcal enlargement is
not specific for CNS-SLE; other causes, including
trauma, alcoholism, Alzheimer disease, and changes
due to the aging process must be taken into account.
During the period that we collected these SLE cases,
10 additional young patients showed a similar picture. Four of them had vasculitis, either systemic or
limited to the intracranial vessels (diagnosed by angiography), but did not meet the criteria for SLE [23];
others had diagnoses of Wilson's disease, accelerated
hypertension, anorexia nervosa, protein-losing enteropathy, multiple sclerosis, o r "unknown collagen
vascular disease."
The variety of disease represented suggests that
the pathological correlate of sulcal enlargement as
seen o n C T scan is not uniform. Previously published
reports of the neuropathological findings in SLE indicate that gross cerebral atrophy is less common than
microinfarction or vascular changes [ l l , 13, 18, 201.
In our series, 2 patients with generalized atrophy by
CT scan who were autopsied had areas of microinfarction but no gross atrophy. O n e SLE patient, not
included in the series because she had a posterior
fossa meningioma, also demonstrated small infarcts
in areas remote from the tumor, and her C T scan
showed large sulci as well.
This evidence implies that extensive microinfarc-
tion may decrease the amount of cortical brain
parenchyma sufficiently to change the attenuation
coefficient of the tissue. Some of the neurological
complications of SLE, particularly those, like dementia, which cannot be localized to a single anatomical
site, may be explained on the basis of a greatly reduced neuronal population throughout the cortex.
The pathogenesis of the vascular changes that presumably precede microinfarction remains to be
elucidated.
In this series we have noted an association between
sulcal enlargement and CNS-SLE that is frequent
enough to suggest that the finding of such changes on
CT scan may be of diagnostic importance. Nevertheless, in many of our patients the diagnosis of
CNS-SLE was evident on clinical grounds alone, and
the presence of sulcal enlargement was used both to
confirm the clinical impression and as a means of
following the progression of the disease. We suspect
that perisulcal and generalized atrophy are not reversible processes, and in our limited follow-up experience we have not seen restoration of a normal pattern
on C T scan. However, at least 1 patient (No. 14)
improved following the use of steroids in spite of
widespread changes on CT; thus, other factors are no
doubt involved in the pathophysiology of CNS-SLE.
In addition to the morphological changes in the
brains of these patients, there may be metabolic abnormalities due to either fluctuations in blood flow or
to other factors that have not been investigated. At
this time there is no controlled study to show that
therapeutic intervention can successfully alter the
course of CNS-SLE. We propose that the problem is
more likely to be resolved with arrest of the systemic
disease prior to the development of CNS changes.
Supported in part by a grant from t h e Lupus Foundation of Delaware Valley.
References
1. Andrianakos AA, Duffy J, Suzuki M, et al: Transverse
myelopathy in systemic lupus erythematosus. Ann Intern Med
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