J. Greenan,
Timothy
MD2
#{149} Robert
Cerebral Vasculitis: and Angiographic Cerebral vasculitis is an unusual disorder with numerous causes. One such entity, noninfectious granulomatous angiitis of the nervous system (CANS), is an extremely rare disease with a predilection for leptomeningeal and parenchymal arteries and veins. Isolated involvement of the central
nervous
system
Radiology
I
From
Philadelphia. requested 2 Current RSNA,
1992;
the
an-
182:65-72
Department
of Radiology,
Hospital
#{149} Herbert
I. Goldberg,
MD
MR Imaging Correlation’
C
vasculitis
EREBRAL
is a mare
One
ulomatous
angiitis
of the
In GCA, usually the temporal artery alone is involved (12). GCA is a granulomatous panamteritis predominantly
disor-
der with numerous causes such disease, noninfectious
(1). gran-
nervous
sys-
tern (CANS), was first described as a distinct entity by Cravioto and Feign in 1959 (2). CANS is an extremely rare disorder
of pamenchymal
meningeal
is characteris-
tic of GANS, which has also been referred to as primary angiitis of the central nervous system (PACNS). The results of magnetic resonance (MR) imaging and angiography in seven patients with presumed PACNS were retrospectively analyzed and correlated. MR images were positive in every case. Charactenstically, lesions were multiple, bilateral, and supratentonal. Both gray- and white-matter infarcts were identified in four of seven patients; infarcts were most common in the deep white matter. PACNS can also appear as primary parenchymal hemorrhage or simulate low-grade glioma. All lesions identified on MR images were associated with positive angiographic findings of cerebral vasculitis in the corresponding vascubar distribution. However, for 12 of 33 vascular distributions with angiographic evidence of cerebral vasculitis, no lesions were identified on MR images. These correlative observations suggest that some patients with proved PACNS may have normal MR imaging results.
Index terms: Arteritis, 13.2581 #{149} Cerebral giography. 17.124 #{149} Cerebral blood vessels, MR. 17.1214 #{149} Vasculitis, 13.2581
MD
I. Grossman,
affect
arteries
vessels
and
of any
predilection teriobes Histologic
and
(200-500 findings
pm
wall,
which
the vessel lymphocytes,
of similar
It can
(4).
nervous
cell arteritis (8,9).
Even
(GCA)
shown rarely to poral artery and (10,11), it seems and GCA do in tinct tends larger
of the
PA 19104. From the 1990 RSNA scientific assembly. Received June 3; revision received July 30; accepted August 12. Address address: Washington Imaging Center, Chevy Chase, Md. 1992
of
is infiltrated
by
large
(7). by 5evof giant-
(temporal GCA
arteritis)
has
been
that
PACNS
fact represent
two
3400 17, 1991; requests
Spruce revision to RIG.
can
giogram
ing,
dis-
classic
only
on
vas-
the
Segmental
basis
narrow-
angiographic
feature
of vasculitis, is nonspecific and is observed in numerous conditions, including PACNS, bacterial or viral am-
teritis,
the systemic
sarcoidosis,
vasculitides,
intravenous
drug
abuse,
atherosclerosis, neoplasm, and arachnoid hemorrhage. To our knowledge, no series tients with PACNS has scribed in the radiobogic and only scattered case
subof pa-
been deliterature, reports, pre-
dominantly in the nonmadiobogic literature, have documented the cornputed tomogmaphic (CT) findings in patients with proved PACNS (5-7,1319). The descriptions of these findings are vague and range from normal to single
or multiple,
bland three
small
or hemrnorhagic case reports have resonance
to barge,
infarcts. Only documented
(MR)
findings
in
patients with PACNS (20-22). We report a retrospective study the MR imaging and angiographic findings
bral
St,
be made
(5-7,13).
the
magnetic
involve both the ternintracranial vessels
April reprint
of cerebral
of positive brain biopsy results; a highly presumptive diagnosis of cemebral vascubitis, however, may be made on the basis of a positive cerebral an-
(PACNS)
of Pennsylvania,
diagnosis
(3).
nosobogic entities (2,3). GCA to involve systemic vessels than those affected by PACNS.
University
Definitive culitis
Characteristically,
likely
nably, GCA characteristically affects persons older than does PACNS and has a more self-limited course (2).
a
been considered to be a variant though
wall is (2). Fi-
am-
cells,
system”
vasorum,
and
Isolated involvement of the central nervous system vascubatume is chamactemistic of GANS. Hence, subsequent to its original decription, GANS has also been referred to in the literature as “ isolated angiilis of the central nervous system” (3,5-6) and more recently as “primary angiitis of the cenPACNS has erab authors
media
of the vessel in PACNS
arteries
these vasculitic lesions result in multiple foci of ischemic or hemorrhagic infarction secondary to vessel wall proliferation with resultant luminal obliteration, thrombolic occlusion, or rupture of necrotic vessel walls.
tral
tunica
shows
cells, and giant cells (4). is usually, but not always, by granubomas composed cells
the
but
in diameter) include necrosis
plasma
mononuclear The vessel surrounded
veins.
size,
for small
bepto-
involving
while this layer relatively spared
in seven
vasculitis.
patients
One
patient
with
had
of
ceme-
bi-
Abbreviations: CSF = cerebrospinal fluid, GANS = granulomatous angiitis of the nervous system. GCA = giant-cell arteritis, HIV = human immunodeficiency virus, PACNS = primary angiitis of the central nervous system, SE = spin echo, SLE = systemic lupus erythematosus, TE = echo time, TR = repetition time.
65
opsy-proved PACNS. patients, clinical and failed to confirm the giographically verified Hence, by exclusion, diagnosis of PACNS
SUBJECTS
AND
In the 68-month
interval 1990, patients
of the University
of Pennsylvania,
and The
Children’s subjects
necessary,
between
April
of PACNS
Phila-
Hospital (five male
of Philaand two
was
made
only
after
exhaustive clinical and laboratory investigation failed to define the cause of the neurologic dysfunction or the angiographic abnormality. Patient evaluation included history, physical examination, chest radiography, complete blood cell count, routine blood chemistry analysis, urinalysis,
rheumatoid
panel
of tests,
mea-
surement
of serum angiotensin-converting enzyme, Lyme titer, lumbar puncture with microbiologic studies, head CT, MR imaging,
and
cerebral
angiography.
Conventional performed
(patient studied nique.
biplane
in every
angiography
case.
In one
was
patient
7), the left vertebral artery with a digital angiographic Three vessels were injected
was techin five
patients, and two vessels were injected in two patients (Table 2). Positive findings on angiograms were defined as a focal or diffuse, smooth or irregular, abnormal narrowing of a vessel, or an abnormal focal dilatation of a vessel (1). In each case, the extent of the vasculitic process was assessed
by recording
the
number
of vascu-
bar distributions involved (Table 2). All patients underwent MR imaging examination, six with a 1.5-1 superconducting magnet (GE Medical Systems, Mibwaukee) and one with a 1.0-1 unit (Siemens, Erlangen, Germany). Spin-echo (SE) sagittal 600/20 (repetition time [TR] msec/echo time [TE] msec) and axial 3,000/30, 80 images were obtained in each case. Gadopentetate dimeglumine, 0.2 mL/kg (Magnevist; Berlex Laboratories, Wayne, NJ) was
administered
postcontrast
in
two
images in the (600/27-30).
patients, axial
and plane
were obtained Positive findings on MR images were defined as high-signal-intensity foci on long-TR images, presumed to represent infarcts
or regions
of ischemia.
Lesions
were of greater magnitude than the cornmonly encountered tiny foci of high signal intensity
often
seen
long-TR images resent sequelae rotic disease in our patient
66
#{149} Radiology
in the white
matter
on
and were believed to repof small-vessel atheroscle-
(which would be uncommon cohort) or foci of demyelina-
tion. In one patient,
arbitrator.
Cerebral angiography (Table 2) in the seven patients demonstrated angiographic evidence of cerebral vascubilis in 33 separate vascular distributions. If only the five patients in whom bilateral carotid and a vertebral injection were performed are considered, then 27 vascular distributions were involved (median = 6, range = 1-8). The supratentoriab circubalion was involved in all seven palients, and angiographic evidence of disease was observed in the infratentoriab vasculature in two palients. Table 3 details the results of MR imaging, which were positive in all seven patients. A total of 36 lesions were identified. Multiple lesions were found in six patients. All lesions were
PACNS was diat the Hospital
the sole abnormal
supratentorial.
Bilateral
disease
was
present in six patients. Four patients had lesions involving both gray and white matter (Fig 1). In five patients, 14 lesions were in the deep white matter. Seven of these deep whitematter lesions in three patients were found in the penventricular white matter. Two of these three patients had additional lesions in gray matter incompatible
with
a diagnosis
of mul-
liple sclerosis. The third patient had a deep white matter lesion in the occipital lobe interpreted as a bow-grade glioma. This patient had another tiny white matter lesion adjacent to the atrium of the right lateral ventricle. Nevertheless, these findings were certainly not typical of multiple sclerosis. Thirteen cortical infarcts were present in three patients, and five deep gray matter lesions were idenlifled in four patients (Fig 2). Three infarcts in two patients involved cortex and subcorticab white matter, but only one isolated subcortical white matter infarct was seen. Hemorrhagic lesions, seen in three patients, included a caudate hemormhage (Fig 3), a hemorrhagic cortical infarct (Fig 2), and an isolated barge parenchymab hemorrhage (Fig 4). Gadopentetate dimeglumine, administered in two patients, revealed enhancement in five of eight lesions in one patient, all of which were cortical infarcts. In the second patient, one of three infarcts was enhanced. Thirty-two of 36 lesions identified
Data and Biopsy
Patient/Age (y)/Sex
RESULTS
female) ranged in age from 3 to 44 years (median, 27 years) (Table I). All patients were normotensive. Three patients underwent brain biopsy. In those patients who did not undergo brain biopsy, or in whom the results of brain biopsy were negative, a presumptive diagnosis
an independent
Table 1 Demographic
METHODS
1985 and December agnosed in seven debphia, delphia.
finding on MR images consisted of a large parenchymal hemorrhage. The median time elapsed between performing MR imaging and angiography was 5 days (range, 0-10 days). All studies were analyzed by two readers and, when
In the other six laboratory data cause of the anvasculitis. a presumptive was made.
Biopsy
Results Results
1/27/F
Negative
2/37/M 3/3/F
Negative Not performed
4/14/M 5/44/M 6/23/M 7/27/M
Not
performed
Not performed Positive Not
performed
on MR images could be correlated with positive angiographic findings in the corresponding vascular distribution. The apparent lack of correlation of four lesions resulted from the angiographic data not being available, as none of the patients in our cohort underwent raphy.
four-vessel Specifically,
cerebral angiogin five patients,
three vessels underwent injection, and in two patients, two vessels underwent injection. It should be emphasized that in 12 of 33 cases of vascubar distribution with angiographic evidence of vascubitis, no identifiable lesions
in the
were
corresponding
visualized
Of the seven graphic, clinical, ings diagnostic
territory
on MR images. patients with angioand laboratory findof PACNS who were
included in this study, three underwent brain biopsy. In two patients (patients 1 and 2), the right frontal lobe and its beptomeninges underwent biopsy with negative results. MR examination in a third patient (patient 6) revealed a moderate-sized lesion confined to the deep white matter of the left occipital lobe, which was
interpreted
as a possible
grade glioma (Fig of this lesion were bomatous angiitis.
low-
5). Results of biopsy positive for granu-
DISCUSSION Prompt diagnosis sential. If untreated,
of PACNS is esthis disease is
rapidly
and
progressive
frequently
fatal. Patients are usually middleaged, and there is no sex-related predominance (7). Headache is the most frequent complaint, and signs of both global and focal neurobogic dysfunction are usually identified (7). Symptoms
such
as fever,
myalgia,
arthral-
gia, and arthritis, which occur frequently in other vasculitic syndromes, are conspicuously absent in PACNS (4). The erythrocyte sedimentation rate is elevated in two-thirds of patients (7). Cerebmospinal fluid (CSF) analysis
characteristically
shows January
a 1992
Table 2 Angiograph
ic Evidence
of Arte
Involvement
ritic
ACA
ICA Vessel Injected
Patient
MCA
R
L
R
L
R
SCA
Vert
PCA
L
R
L
R
L
R
L
LCCA,LV
-
-
-
+
-
-
-
-
+
+
+
+
-
+ +
-
+
+ +
-
BCCA,LV
-
-
-
-
3 4 5 6
BCCA,LV BCCA,LV BCCA,LV BCCA,LV LCCA,LV
-
-
+
+
+
+
-
+
-
-
-
-
-
-
+
+
+
+
-
-
+
-
-
5 6
-
-
+ +
+
+
+
+
+
-
-
+
+
8
-
-
-
-
-
-
-
+
-
-
-
-
-
-
-
+
-
+
-
+
-
-
-
-
Note.-ACA = anterior cerebral artery, BCCA = bilateral common carotid arteries, ICA = supraclinoid mon carotid artery, L = left, LV = left vertebral artery, MCA = middle cerebral artery, = no evidence evidence of involvement, R = right, SCA = superior cerebellar artery, vert = vertebral.
of Lesions
Patient
.
SC
DWM
0 0 0 0 0 1 0
6 2 0
I 1* 1
j*
0
3 2 0
2 0 0
3 8
1
14
5
36
1 0 5*
4 5 6
0 0 0
7
7
0 0 0 0 2 0 1
13
3
Total
Total
C/S
1 2 3
weakening
Note-C = cortex, C/S = cortical/subcortical white matter, SC = subcortical white matter. *One lesion was hemorrhagic.
white
matter,
DGM
0GM
deep
=
gray
(35). The angiographic rebral vascubitis are
8 3 6 1
can be seen
7
matter,
DWM
with
an
elevated
(7). Prednisone for
treatment
for
drug
PACNS.
of For
of corticosteroid-resistant
or progressive PACNS, cyclophospharnide can be added to the therapy
The known,
exact cause although
of PACNS numerous
(5).
is unreports
suggest a possible viral cause. The association of herpes zoster ophthalmicus and delayed contralateral hemiparesis is well known (23,24).
Cerebral tients culitis
angiogmaphy shows most
findings cornrnonly
in these
the Ml and A2 segments dle and anterior cerebral
phy
and
logic
history High antibody
vasto
In most of there is also a
of a patient
and used clear
electron microscopy has been to identify herpeslike intranuviral particles in another patient
182
#{149} Number
PACNS
virus from the
CSF
Volume
with
1
enter
into
the
diagnosis
radio-
of cerebral
(29),
to be eccentric
and
involve
segments (36). That
orrhage
produces
who
Willis. Subarachnoid hemorrhage rarely occurs as a consequence of muptume of an arteritic vessel (1). Physical
was
semonegative
munodeficiency virus yet HIV was isolated and CSF (19). Electron
for human
wall
and
within
granubomatous
a patient with ing is supported
im-
(HIV) infection, from the brain microscopy has
mycoplasmabike
in the
traburninab
of the midarteries,
of lymphorna. levels of anti-varicella have been isolated
hence
differential
shorter lesions
tures
respectively (25,26). Histopathobogically, these patients have a granubornatous vasculitis identical to that of PACNS (27,28). Diffuse granubornatous vascubitis has also been described in patients with recent herpes zoster
infection (14-16,27-31). these cases, however,
of noninflarn-
with PACNS (32). The association of PACNS with other viral or viruslike illnesses has also been seen. Specificabby, granubornatous angiitis has mecently been described in a patient
demonstrated
pa-
of cerebral localized
in a variety
of ceand
vasculitis. Atherosclerosis is the most common cause of arterial narrowing seen at angiography. The stenotic lesions of atherosclerosis, however,
protein
is the
therapy
findings nonspecific
to
hemorrhage, and neoplasm may mimic cerebral vasculitis at angiogradeep
=
vessel
secondary process
matory and inflammatory disorders. Noninflammatory vascubopathies such as atherosclerosis, subamachnoid
tend pleocytosis
of a damaged
wall (1) or vasoparabysis an adjacent inflammatory
on MR Images
C
1 3
segment of internal carotid artery, LCCA = left cornof involvement, PCA = posterior cerebral artery, + =
from
Table 3 Distribution
level
3 7
I 2
7
choice
No. of Arteries Involved
structhe
in
PACNS (33). This findby the work of Tho-
mas et al (34), who injected turkey poults with a neurotropic strain of Mycoplasrna gallisepticutn and pro-
duced
a fatal
necmotizing
cerebral
vas-
tions.
When
alternate
with the focal dilatations, is a “ string-of-beads” appearThe vascular narrowings in ceangiitis may be due to spasm,
theme ance. rebrab
edema,
cellular
vascular
infiltration
narrowings
or prolifem-
of the vessel wall, or compression by surrounding thickened meninges from exudate or fibrosis (i). Similarly, focal dilatations may result ation
usual
of an aneurysm
examination,
is well
cause
is rup-
of the circle
CT appearance,
of
and
CSF
in identifyhemor-
rhage. Neoplasms can also produce vascular narrowings and irregularities that resemble arteritis at arteriography.
cubitis. The characteristic angiographic features of cerebral vasculitis consist of vascular narmowings and focal dilata-
the
ture
vasospasm
The
studies should be useful ing cases of subarachnoid
in-
reaction
established.
than do vasculitic subarachnoid hem-
These
findings
may
(meningioma
metastasis
(37) or diffuse or meningeal
(lymphoma, carcinomatosis).
be focal
or glioma) leukemia, CSF
evaluation and MR examination with administration of contrast material will usually indicate the presence of neoplastic disease. Cerebral vasculitis can be of infectious or noninfectious cause. Unfortunately,
cannot other
these
numerous
be distinguished solely on the basis
graphic cerebral nantly
criteria. vasculitis bacterial
Infectious include and viral
rulent
bacterial
arteritis
a complication
of severe
conditions
from one of angiocauses pmedomidisease.
is most
anof Pu-
often
bacterial
Radiology
#{149} 67
Figure
1. Patient
(a) SE 3,000/80
1.
axial
MR
and
image
arteriographic
reveals
images
numerous
obtained
foci of high
in a 27-year-old
signal
patient
intensity
compatible
with
PACNS
with
infarcts
and
multiple
gray
and
white
in the left perisylvian
cortex,
matter
infarcts.
left putamen,
left posterior parietal white matter, and right external capsule (arrows). (b) SE 3,000/80 axial image at a level slightly superior to that in a shows foci of high signal intensity indicative of infarction adjacent to the body of the left lateral ventricle and in the white matter of the left parietal lobe. There is a tiny extraaxial collection in the right frontal region compatible with recent brain biopsy. (c) Early lateral view from an arteriogram of the left common carotid artery shows focal segments of narrowing (arrows) involving the pericallosal and callosomarginal branches of the anterior cerebral artery, which is compatible with vasculitis. In addition, there is nonfilling of multiple middle cerebral branches secondary to extensive vasculitic involvement.
due to Haeinoinfection (38). The most common finding is involvement of the arteries at the base of the brain, as this is the predominant location of purulent arachnoiditis (1,39). More peripheral branches may show vary-
litis, and drug-abuse vasculitis. Periarteritis nodosa is the most common of the systemic necrotizing vascubitides associated with cerebral vascubitis (3).
of SLE developed months later. Central nervous ment in sarcoidosis
The
3%-5%
ing degrees of diffuse Acute bacterial arteritis
sis, sis of
meningitis,
philus
usually
influenzae
narrowing (1). may result in
formation of mycotic aneurysm (39). The arteritis associated with tubercubus meningitis also has a marked tendency to involve arteries at the base of the brain, particularly the supraclinoid portion of the internal carotid artery and the horizontal portion of
the
middle
quently branches.
cerebral also involves The proclivity
artery the
(1), but convexity of syphilis
invade the vasculature is quite dent in tertiary meningovascular
freto
evidis-
ease.
other
bitides, yasu
by necrotizing
and
is usually
the disease tients with
inflammation
ported patients
by Ford and with central
symptoms
tremely
a late
manifestation
(3). In the periarteritis
had
topathobogically,
of
disease,
from
PACNS (3). Cerebral vasculitis has been described to be associated with several of the collagen vascular diseases, including systemic lupus erythematosus
first
(50).
uncommon.
In their
findings
cases of SLE, Ellis and Verity found evidence of cerebral in only 7% . In SLE, as with collagen vascular diseases, vasculitis generally occurs
with disease genhuis
previously
diagnosed
review
in 57 (41) vasculitis
the
other
cerebral in patients
systemic
(41,43-45). Sanders and Ho(42) reported a case of cere-
bral
vasculitis
tom
of SLE.
as the
Systemic
presenting
manifestations
system involveoccurs in only (3). Cerebral angiin association is exceedingly et al (49) mefindings in a vasculitis. His-
the
symp-
described
evidence occlusion of
in
In 1971,
leagues
(SLE) (41-43), scberoderma (44), and rheumatoid arthritis (45), but is exthe neumopathobogic
16
form
of
granubo-
angiitis seen in sarcoid is very to PACNS (4). The two diseases are best distinguished by the presence of systemic samcoidosis and leptomeningitis, which have been seen in all reported cases of angiitis neurosarcoid (3,46-49). CSF analysis and MR examination with contrast material administration will usually demonstrate the presence of leptomeningeal disease as the cause of an arteritis. Illegal drug-abuse vasculitis was
Siekert (40), all nervous system
distinction
patients
patient
matous similar
series of 114 panodosa re-
systemic
enables
of
itis has been described with sarcoidosis but rare (46-48). Lawrence ported angiographic patient with samcoid
primarily of muscular arteries (1). Central nervous system involvement in periarteritis nodosa has been meported to occur in 46% of cases (40)
analysis of CSF will indicate the bactenab cause of the arteritis. A large number of viruses display neurotropism (39). With the exception of herpes
#{149} Radiology
vascu-
however,
acterized
which
68
necrotizing
include GCA, Takaarteritis, Wegener granubomatoand bymphomatoid granulomato(3). Pemiarteritis nodosa is a disease multiple system involvement char-
In syphilitic angiitis, both arteries and veins are affected and there is a predilection for branches of the middle cerebral artery (39). Luetic artemiab lesions are generally diffuse and affect all leptomeningeab vessels, causing difficulty with collateral circubation (39). Results of microbiologic
zoster, however, viruses generally affect vessels of microscopic cabiber(1). The noninfectious vascubitides are numerous. In addition to PACNS are the systemic necrotizing vasculitides, vasculitis associated with collagen vascular disease, neurosarcoid vascu-
systemic
in their
(51)
1970
Rumbaugh reported
by
Citron
and
et
ab
cob-
angiographic
of cerebral vasculitis in 19 addicts who
and/or mostly
used methamphetarnine. Methamphetamine or “speed” arteritis has subsequently been described by numerous
other
investigators
(52-54).
Cerebral vasculitis has also been meported to be associated with heroin (55) and, more recently, cocaine abuse (56,57). The exact pathogenesis of drug-abuse vasculitis is unknown, but a hypersensitivity reaction to the drug and/or to impurities has been postulated
(55). January
1992
tious cause of the vasculitis patient. Physical examination, sis of CSF, and MR and CT
revealed titers history
‘
c-.
were .
C..
#{149}.::
.. CA
...; 1,
Si#{149}
::
a.
patient zostem
b.
Figure
2. Patient 3. Multiple gray matter infarcts in a 3-year-old patient with PACNS. (a) SE 3,000/80 axial image reveals high signal intensity compatible with infarction in the left thalamus and bilateral anterosuperior insular cortex. (b) SE 3,000/80 axial image reveals abnormal high signal intensity in the medial aspect of the left frontoparietal cortex, which is indicative of infarction. A focus of low signal intensity within the infarct represents hemorrhage.
A
Lyme had oph-
In addition, there of systemic disease Results of rheumatoid
unremarkable. and
enzyme #{149}54
,...
disease.
graphs
%Jk0::
-0’
no meningeal were negative. No of recent herpes
thalmicus. evidence patient. #{149}
in any analystudies
Chest
a
was no in any panels
radio-
angiotensin-converting
levels
were
normal.
No pa-
tient had a history of intravenous drug abuse. Although by these exclusionary criteria, PACNS does seem the plausible diagnosis, it is certainly
most con-
ceivable that the observed vascubitic changes could be the initial presentation of one of the systemic vascubitides, such as periarteritis nodosa, or one of the collagen vascular diseases. As previously discussed, a single case of SLE appearing as cerebral vasculitis in the absence of antecedent or synchronous
systemic
disease
reported (42). All patients hort responded favorably with prednisone and/or phamide. At this writing,
were
in remission
has
been
in our coto therapy cycbophosall patients
and
no patient
had
developed any manifestation of a systemic disease. Finally, it is highly unlikely that sarcoidosis was the cause of vascubitis in any of our patients because angiitic neurosarcoid has been reported only in patients with systemic sarcoidosis, which we were able to successfully exclude in our cohort
(2,46-49).
a.
b.
Figure
3. Patient 2. Images obtained in a 37-year-old patient with PACNS and a caudate hemorrhage. (a) SE 600/20 axial MR image reveals a focus of high intensity in the left caudate nucleus compatible with subacute hemorrhage. In addition, there is abnormal low signal intensity within the splenium of the corpus callosum, indicative of infarction. (b) Anteropostenor image obtained early during arteriography of the left common carotid artery reveals focal areas of narrowing involving the supraclinoid internal carotid artery (arrowhead) and the proximal segments of the anterior cerebral artery (solid arrow) and middle cerebral artery (open arrow), compatible with vasculitis.
Cababrese and Mallek (7) found the results of cerebral angiography to be abnormal in 84% of patients with PACNS, with classic findings of arteritis observed in 65%. False-negative angiograms in patients with PACNS are not uncommon, because this disease primarily affects the precapillamy arterioles (58), which are below the resolving capacity of cerebral angiography. Numerous cases of biopsyproved PACNS associated with nor-
mal
cerebral
One of the patients reported herein had biopsy-proved PACNS. Prior to establishing a presumptive diagnosis of cerebral vasculitis in the remaining
had lymphoproliferative disease or a history of malignancy. Because of the nonspecific nature of the angiographic findings in vascubi-
been
six patients, we excluded nonvasculitic vasculopathies to the best of our diagnostic capabilities. Specifically, our cohort was normotensive, and CSF analysis and MR and CT examination revealed no evidence of sub-
tis, no definite causal diagnosis could be made for these six patients without a brain biopsy. Nevertheless, after thorough clinical and laboratory investigation, PACNS was suggested as the presumptive diagnosis on the basis of exclusion. To be more specific, theme was no evidence of an infec-
infectious
arachnoid or
hemorrhage,
leptorneningeal
Volume
182
brain
disease. #{149} Number
1
tumor,
No
patient
artemiograms
been reported (20-22,59,60). to afflict small
in the Despite vessels,
have literature its predilection PACNS has
histopathobogically
shown
to
involve barge branches of the main cerebral arteries (2,61). In fact, for case 1 in their original description of nongranubomatous
the nervous Feigin tous
system,
(2) stated process
angiitis
Cravioto
“a severe
involved
of
and granuboma-
arteries
and
of varying caliber, from the die cerebral artery to the intraparenchymal vessels.” Cerebral angiographic findings in our six patients veins
Radiology
mid-
#{149} 69
with tient
presumed PACNS and one pawith proved PACNS were corn-
patible
with
those
in previous
reports
in the literature (4,6,13). Angiography revealed segmental, diffuse, bilateral disease rial and
that involved infratentorial
the supratentocirculations
mize
biopsy specimens from the frontal hemisphere
morbidity.
The
MR imaging,
are usulobe of the to mini-
leptomeningeab
which
revealed
a moderate-sized lesion in the deep white matter adjacent to the occipital horn of the left lateral ventricle (Fig 5). The lesion was not enhanced with contrast material on CT scans. Results of angiography at the time of MR irnaging were interpreted as negative. In retrospect, subtle regions of focal narmowing were detected in the distribution of the left posterior cerebral amtemy. Hence, this constellation of findings at the lime was suggestive of a bow-grade glioma. Brain biopsy of this lesion was then performed. Tissue analysis of the specimen revealed granubomatous angiilis of the cerebral vessels. 70
#{149} Radiology
by Valvanis case
case of tumor was
et ab (18).
mass
lesion
This
of granulomatous was seen on a CT
scan as a homogeneously and
biopsy is important in that cerebral vascubitis often has a predilection for the beptomeningeal vasculature. Although PACNS is a diffuse disease, it is segmental; hence, brain biopsy mesults can be negative. Three patients in the present study underwent brain biopsy, the results of which confirmed PACNS in only one. The false-negative results in two patients can be considered a sampling error. Both of these patients underwent right frontal lobe and leptomeningeal biopsy. It is interesting that MR examination in one of these patients (patient 2) revealed disease limited to the left hemisphere. Angiography in this patient did reveal vascubitis on the right side that was limited to the supraclinoid segment of the internal carotid artery and proximal segments of the anterior cerebral artery and middle cerebral artery. In the second patient (patient 1), MR imaging also revealed much more extensive disease on the left side. In this case, however, the right carotid artery was not studied. The patient in our cohort who did have positive brain biopsy results (patient 6) was initially evaluated with
CT and
reported
dramatic a brain
biopsy-proved angiitis originally
vessels of all sizes. Only one patient had involvement in a single vascular distribution. It is our opinion that brain biopsy should be reserved for the group of patients in whom thorough clinical investigation, MR imaging, and angiography fail to explain an aquired neurologic deficit. Brain and leptomeningeab ably taken nondominant
An even more PACNS simulating
enhanced
involving
the
cortex
and
subcomtical white matter of the right temporal-occipital lobe. In addition, Johnson et ab (21) described the MR findings in a case of biopsy-proved PACNS that was seen as bilateral parietooccipitab mass lesions that crossed the splenium of the corpus callosum.
All patients were examined strength case
in the with
MR yielded
present study high-field-
imaging,
which
positive
findings.
in each A me-
dian interval of 5 days elapsed tween performing MR imaging performing
cerebral
five
of seven
was
performed
In no case
beand
angiography.
In
patients,
MR imaging
prior
to angiography.
were
steroids
or cytotoxic
agents administered before cerebral angiography. Steroid therapy was begun in only one case after angiography but before MR examination. It is unlikely that in these five patients the severity of cerebral vascubitis, as assessed at the time of angiography, was greater than at the time of MR imaging (a mean of 5 days earlier). Conversely, in the one untreated pa-
lient who underwent MR tion subsequent to cerebral
examinaangiogra-
phy, it is also unlikely that the brain lesions changed significantly in the interval. In addition, in this patient,
all of the ages
lesions
were
tamed
evident
present
several
on
days
on MR ima CT
prior
scan
ob-
to cerebral
angiography.
MR examination tive in all patients sistent
with
the
of PACNS, ease.
results were in this study, known
a diffuse
Common
pathogenesis
segmental
MR
dis-
findings
in our
patient cohort were numerous dian, six), bilateral, supratentorial farcts involving both gray matter. The exact mechanism farction, such as ischemia
nab encroachment
(mein-
and
white of infrom lumi-
or secondary
thrombotic occlusion, not discernible.
On
posicon-
was
MR images,
infarcts
obviously
were
most
commonly
seen in the deep white matter, including the corpus callosum and capsular tracts. Contrary to the findings of Miller et al (62), we saw no predisposition for the periventricubar
white
matter.
These
investigators
used a cohort that did graphic or biopsy-proved
The
cause
vascubilis was
systemic
of the in their
not
have angiovasculitis.
presumptive
cerebral
patient
collagen
population vascular
dis-
b. Figure
4. Patient 4. PACNS presenting as a solitary parenchymal hemorrhage. (a) SE axial 600/20 MR image reveals a large focus of high signal intensity in the right parietal lobe, indicative of a subacute hematoma. (b) Early arteriogram in the anteroposterior projection obtained during injection of the left vertebral artery shows multiple foci of narrowing (arrows) involving branches of the posterior cerebral arteries bilaterally, compatible with vasculitis.
ease. It seems possible that the ventricular lesions they described might in fact be the sequelae of vasculitic vascubopathy known cur in patients with this group eases,
particularly
SLE
pena nonto ocof dis-
(41).
January
1992
involves both rior circulations
latume.
The
the
anterior and of the cerebral
characteristic
postevascu-
findings
at
MR imaging are multiple, bilateral gray and white matter lesions that in this study were confined to the supratentoriab space. There appears to be no predisposition for the periventricubar region, although deep white matter lesions were common. PACNS can present somewhat atypically as primary parenchymal hemorrhage or simulate a primary brain tumor. Although MR images were positive in !4::i
all our
-
Figure 5. Patient 6. PACNS simulating a low-grade glioma. SE 3,000/80 axial MR image shows a moderate-sized focus of high signal intensity in the deep white matter of the left occipital lobe. The lesion exerts slight mass effect on the occipital horn of the left lateral ventricle. Biopsy of this lesion revealed granulomatous angiitis.
tion with angiographicabby proved vasculitis, however, no lesion in the corresponding vascular territory could be identified at MR imaging. Although MR images were positive in all patients with PACNS, extrapolation from these results suggests that cases of MR-negative PACNS are entimely possible. It is interesting that no infratentomial lesions were depicted with MR imaging despite cerebral angiogmaphy revealing evidence of vasculitis involving the posterior circulation in all patients. Our data suggest that positive findings on MR images might be used to tailor cerebral angiography in cases of “suspected vascubitis.” In summary, PACNS is a mare disease
that
diffusely
JMiimo1R9#{149}T%Jimhor1
and
segmentally
on the basis
of extrapola-
tion from our correlative angiographic data, it is possible that larger number of patients with rare disease are studied, there cases with normal MR images.
as a this may be
literature.
Arthritis
Rheum
1982;
25:
342-345. 18.
Valvanis A, Friede R, Schubiger 0, Hayek J. Cerebral granulomatous angiitis simulating brain tumor. J Comput Assist Tomogr 1979;
19.
Yankner BA, Skolnik PR, Shoukimas GM, Gabuzda DH, Sobel RA, Ho D. Cerebral granulomatous angiitis associated with isolation of human T-lymphotropic virus type III from the central nervous system. Ann Neurol 1986; 20:362-364. Koo EH, Massey EN. Granulomatous angiitis of the central nervous system: protean manifestations and response to treatment. J Neurol Neurosurg Psychiatry 1988;
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22.
#{149}
Johnson M, Maciunas R, Dutt P. Clinton ME, Collins R. Granulomatous angiitis masquerading as a mass lesion. Surg Neurol 1989; 31 :49-53. Scully R. Massachusetts General Hospital Case Records, case 8-1989: weekly clinicopathological exercises. N Engl J Med 1989; 320:514-524.
23.
References 1. 2.
Three separate hemorrhagic lesions were seen in three patients in our study. One of these lesions was a hemorrhagic cortical infarct associated with other nonhemorrhagic cortical infarcts, which most likely bled as a result of a reperfusional event. Another was a hemorrhage of indeterminate cause in the head of the caudate. The consequence of vessel wall rupture almost certainly was a large hemorrhage in the deep white matter of the might panietal lobe in the third patient. This case was extremely interesting in that this 14-year-old patient was first seen with a primary parenchymal hemorrhage as a single lesion. For every lesion identified at MR imaging, findings of cerebral vasculitis were positive at angiography in the corresponding vascular distribution. In 12 of 33 cases of vascular distnibu-
cases,
of the
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Vilchez-Pc1,edon I, Ruiz A. CNS varicella-zoster vasculitis (letter). Arch Neurol 1982; 39:785. Blue MC, Rosenblum WI. Granulomatous angiitis of the brain with herpes zoster and varicella encephalitis. Arch Pathol Lab Med 1983; 107:126-128. Rosenblum WI, Hadfield MG, Young HF. Granulomatous angiitis with preceding varicella zoster. Ann Neurol 1978; 3:374375.
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Reyes MG, Fresco R, Chokroverty 5, Salud EQ. Virus-like particles in granulomatous angiitis of the central nervous system. Neurology 1976; 26:797-799. Arthur G, Margolis G. Mycoplasma-like structures in granulomatous angiitis of the central nervous system. Arch Pathol Lab Med 1977; 101 :385-387. Thomas L, Davidson M, McCluskey RT. The studies of PPLO infection. I. The production of cerebral polyarteritis by Mycoplasma gallisepticum in turkeys: the neurotoxic property of the mycoplasma. J Exp Med 1966; 123:897-912. Davis DO, Dilenye D, Schlaepfer W. Arterial dilatation in purulent meningitis: case report. J Neurosurg 1970; 32:112-115. Sole-Llenas J, Mercader JM, Mirosa F. Cerebral arteritis: angiographic and pathologic study. Angiology 1978; 29:713-718. Cowen RL, Siqueira EB, George E. Angiographic demonstration of a glioma involving the wall of the anterior cerebral artery: report of a case. Radiology 1970; 97:577578.
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Igarashi Wilburn
M, Gilmartin F, JabbourJT.
RC, Gerald Cerebral
B, arteritis
and bacterial meningitis. Arch Neurol 1984; 41:531-535. Sole-Llenas J, Pons-Tortella E. Cerebral angiitis. Neuroradiology 1978; 15:1-li. Ford RG, Siekert RG. Central nervous system manifestations of periarteritis nodosa. Neurology 1965; 15:115-122. Ellis SG, Verity MA. Central nervous systern involvement in systemic lupus erythernatosus: a review of neuropathologic findings in 57 cases, 1955-1977. Semin Arthritis Rheum 1979; 8:212-221. Sanders EACM, Hogenhuis LAH. Cerebral vasculitis as presenting symptom of systemic lupus erythernatosus. Acta Neurol Scand 1986; 74:75-77. Trevor RP, Sondheirner FK, Fessel WJ, Wolpert SM. Angiographic dernonstralion of major cerebral vessel occlusion in sytemic lupus erythematosus. Neuroradiology 1972; 4:202-207. Estey E, Lieberman A, Pinto R, Meltzer M, Pansohoff J. Cerebral arteritis in scleroderma. Stroke 1979; 10:595-597. Ramos M, Mandybur TI. Cerebral vasculitis in rheumatoid arthritis. Arch Neurol 1975; 32:271-275.
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MeyerJS,
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nervous
Sarcoidosis
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of the 1962;
DP. Clinical neuropaDis Nerv Syst 1975;
Lawrence WP, El Gammal T, Pool WH, Apter L. Radiological manifestations of neurosarcoidosis: report of three cases and review
of the
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Citron BP, Halpern Necrotizing angiitis
M, McCarron M, et al. associated with drug abuse. N EnglJ Med 1970; 283:1003-1011. Rumbaugh CL, Bergeron RT, Fang HCH, McCormick R. Cerebral angiographic changes in the drug abuse patient. Radiology 1971; 101:335-344. Margolis MT. Newton TH. Methamphetamine ‘speed’ arteritis. Neuroradiology 1971; 2:179-182. Matick H, Anderson D, BrumlikJ. Cerebral vasculitis associated with oral amphet-
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7:432-441. Aronson SM, Perl thology conference. 34:392-395.
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Campagna-Pinto D. angiitis of the meninges in Neurol Psychiatry 1953; 69:
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FoleyJM,
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Kaye BR. Fainstat M. Cerebral vasculitis associated with cocaine abuse. JAMA 1987; 258:2104-2106. Krendel DA, Ditter SM, Frankel MR. Ross WK. Biopsy-proven cerebral vascultitis associated with cocaine abuse. Neurology 1990; 40:1092-1094. Hughes JT, Brownell B. Granulomatous giant-celled angiitis of the central nervous system. Neurology 1966; 16:293-298. Younger DS, Hays AP, BrustJCM, Rowland LP. Granulomatous angiitis of the brain: an inflammatory reaction of diverse etiology. Arch Neurol 1988; 45:514-518. Clifford-Jones RE, Love 5, Gurusinghe N. Granulomatous angiitis of the central nervous system: a case with recurrent intracerebral hemorrhage. J Neurol Neurosurg Psychiatry 1985; 48:1054-1056. Kolodny EH, Rebeiz JJ, Caniness VS. Richardson EP. Granulomatous angiitis of the central nervous system. Arch Neurol 1968; 19:510-524. Miller DH, Ormerod IEC, Gibson A, duBoulay EPGH, Rudge P. McDonald WI. MR brain scanning in patients with vasculitis: differentiation from multiple sclerosis. Neuroradiology 1987; 29:226-231.
Amphetamine-induced
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1981;
12:
Cerebral arabuse. Med
January
1992
Timothy
MD2
#{149} Robert
Cerebral Vasculitis: and Angiographic Cerebral vasculitis is an unusual disorder with numerous causes. One such entity, noninfectious granulomatous angiitis of the nervous system (CANS), is an extremely rare disease with a predilection for leptomeningeal and parenchymal arteries and veins. Isolated involvement of the central
nervous
system
Radiology
I
From
Philadelphia. requested 2 Current RSNA,
1992;
the
an-
182:65-72
Department
of Radiology,
Hospital
#{149} Herbert
I. Goldberg,
MD
MR Imaging Correlation’
C
vasculitis
EREBRAL
is a mare
One
ulomatous
angiitis
of the
In GCA, usually the temporal artery alone is involved (12). GCA is a granulomatous panamteritis predominantly
disor-
der with numerous causes such disease, noninfectious
(1). gran-
nervous
sys-
tern (CANS), was first described as a distinct entity by Cravioto and Feign in 1959 (2). CANS is an extremely rare disorder
of pamenchymal
meningeal
is characteris-
tic of GANS, which has also been referred to as primary angiitis of the central nervous system (PACNS). The results of magnetic resonance (MR) imaging and angiography in seven patients with presumed PACNS were retrospectively analyzed and correlated. MR images were positive in every case. Charactenstically, lesions were multiple, bilateral, and supratentonal. Both gray- and white-matter infarcts were identified in four of seven patients; infarcts were most common in the deep white matter. PACNS can also appear as primary parenchymal hemorrhage or simulate low-grade glioma. All lesions identified on MR images were associated with positive angiographic findings of cerebral vasculitis in the corresponding vascubar distribution. However, for 12 of 33 vascular distributions with angiographic evidence of cerebral vasculitis, no lesions were identified on MR images. These correlative observations suggest that some patients with proved PACNS may have normal MR imaging results.
Index terms: Arteritis, 13.2581 #{149} Cerebral giography. 17.124 #{149} Cerebral blood vessels, MR. 17.1214 #{149} Vasculitis, 13.2581
MD
I. Grossman,
affect
arteries
vessels
and
of any
predilection teriobes Histologic
and
(200-500 findings
pm
wall,
which
the vessel lymphocytes,
of similar
It can
(4).
nervous
cell arteritis (8,9).
Even
(GCA)
shown rarely to poral artery and (10,11), it seems and GCA do in tinct tends larger
of the
PA 19104. From the 1990 RSNA scientific assembly. Received June 3; revision received July 30; accepted August 12. Address address: Washington Imaging Center, Chevy Chase, Md. 1992
of
is infiltrated
by
large
(7). by 5evof giant-
(temporal GCA
arteritis)
has
been
that
PACNS
fact represent
two
3400 17, 1991; requests
Spruce revision to RIG.
can
giogram
ing,
dis-
classic
only
on
vas-
the
Segmental
basis
narrow-
angiographic
feature
of vasculitis, is nonspecific and is observed in numerous conditions, including PACNS, bacterial or viral am-
teritis,
the systemic
sarcoidosis,
vasculitides,
intravenous
drug
abuse,
atherosclerosis, neoplasm, and arachnoid hemorrhage. To our knowledge, no series tients with PACNS has scribed in the radiobogic and only scattered case
subof pa-
been deliterature, reports, pre-
dominantly in the nonmadiobogic literature, have documented the cornputed tomogmaphic (CT) findings in patients with proved PACNS (5-7,1319). The descriptions of these findings are vague and range from normal to single
or multiple,
bland three
small
or hemrnorhagic case reports have resonance
to barge,
infarcts. Only documented
(MR)
findings
in
patients with PACNS (20-22). We report a retrospective study the MR imaging and angiographic findings
bral
St,
be made
(5-7,13).
the
magnetic
involve both the ternintracranial vessels
April reprint
of cerebral
of positive brain biopsy results; a highly presumptive diagnosis of cemebral vascubitis, however, may be made on the basis of a positive cerebral an-
(PACNS)
of Pennsylvania,
diagnosis
(3).
nosobogic entities (2,3). GCA to involve systemic vessels than those affected by PACNS.
University
Definitive culitis
Characteristically,
likely
nably, GCA characteristically affects persons older than does PACNS and has a more self-limited course (2).
a
been considered to be a variant though
wall is (2). Fi-
am-
cells,
system”
vasorum,
and
Isolated involvement of the central nervous system vascubatume is chamactemistic of GANS. Hence, subsequent to its original decription, GANS has also been referred to in the literature as “ isolated angiilis of the central nervous system” (3,5-6) and more recently as “primary angiitis of the cenPACNS has erab authors
media
of the vessel in PACNS
arteries
these vasculitic lesions result in multiple foci of ischemic or hemorrhagic infarction secondary to vessel wall proliferation with resultant luminal obliteration, thrombolic occlusion, or rupture of necrotic vessel walls.
tral
tunica
shows
cells, and giant cells (4). is usually, but not always, by granubomas composed cells
the
but
in diameter) include necrosis
plasma
mononuclear The vessel surrounded
veins.
size,
for small
bepto-
involving
while this layer relatively spared
in seven
vasculitis.
patients
One
patient
with
had
of
ceme-
bi-
Abbreviations: CSF = cerebrospinal fluid, GANS = granulomatous angiitis of the nervous system. GCA = giant-cell arteritis, HIV = human immunodeficiency virus, PACNS = primary angiitis of the central nervous system, SE = spin echo, SLE = systemic lupus erythematosus, TE = echo time, TR = repetition time.
65
opsy-proved PACNS. patients, clinical and failed to confirm the giographically verified Hence, by exclusion, diagnosis of PACNS
SUBJECTS
AND
In the 68-month
interval 1990, patients
of the University
of Pennsylvania,
and The
Children’s subjects
necessary,
between
April
of PACNS
Phila-
Hospital (five male
of Philaand two
was
made
only
after
exhaustive clinical and laboratory investigation failed to define the cause of the neurologic dysfunction or the angiographic abnormality. Patient evaluation included history, physical examination, chest radiography, complete blood cell count, routine blood chemistry analysis, urinalysis,
rheumatoid
panel
of tests,
mea-
surement
of serum angiotensin-converting enzyme, Lyme titer, lumbar puncture with microbiologic studies, head CT, MR imaging,
and
cerebral
angiography.
Conventional performed
(patient studied nique.
biplane
in every
angiography
case.
In one
was
patient
7), the left vertebral artery with a digital angiographic Three vessels were injected
was techin five
patients, and two vessels were injected in two patients (Table 2). Positive findings on angiograms were defined as a focal or diffuse, smooth or irregular, abnormal narrowing of a vessel, or an abnormal focal dilatation of a vessel (1). In each case, the extent of the vasculitic process was assessed
by recording
the
number
of vascu-
bar distributions involved (Table 2). All patients underwent MR imaging examination, six with a 1.5-1 superconducting magnet (GE Medical Systems, Mibwaukee) and one with a 1.0-1 unit (Siemens, Erlangen, Germany). Spin-echo (SE) sagittal 600/20 (repetition time [TR] msec/echo time [TE] msec) and axial 3,000/30, 80 images were obtained in each case. Gadopentetate dimeglumine, 0.2 mL/kg (Magnevist; Berlex Laboratories, Wayne, NJ) was
administered
postcontrast
in
two
images in the (600/27-30).
patients, axial
and plane
were obtained Positive findings on MR images were defined as high-signal-intensity foci on long-TR images, presumed to represent infarcts
or regions
of ischemia.
Lesions
were of greater magnitude than the cornmonly encountered tiny foci of high signal intensity
often
seen
long-TR images resent sequelae rotic disease in our patient
66
#{149} Radiology
in the white
matter
on
and were believed to repof small-vessel atheroscle-
(which would be uncommon cohort) or foci of demyelina-
tion. In one patient,
arbitrator.
Cerebral angiography (Table 2) in the seven patients demonstrated angiographic evidence of cerebral vascubilis in 33 separate vascular distributions. If only the five patients in whom bilateral carotid and a vertebral injection were performed are considered, then 27 vascular distributions were involved (median = 6, range = 1-8). The supratentoriab circubalion was involved in all seven palients, and angiographic evidence of disease was observed in the infratentoriab vasculature in two palients. Table 3 details the results of MR imaging, which were positive in all seven patients. A total of 36 lesions were identified. Multiple lesions were found in six patients. All lesions were
PACNS was diat the Hospital
the sole abnormal
supratentorial.
Bilateral
disease
was
present in six patients. Four patients had lesions involving both gray and white matter (Fig 1). In five patients, 14 lesions were in the deep white matter. Seven of these deep whitematter lesions in three patients were found in the penventricular white matter. Two of these three patients had additional lesions in gray matter incompatible
with
a diagnosis
of mul-
liple sclerosis. The third patient had a deep white matter lesion in the occipital lobe interpreted as a bow-grade glioma. This patient had another tiny white matter lesion adjacent to the atrium of the right lateral ventricle. Nevertheless, these findings were certainly not typical of multiple sclerosis. Thirteen cortical infarcts were present in three patients, and five deep gray matter lesions were idenlifled in four patients (Fig 2). Three infarcts in two patients involved cortex and subcorticab white matter, but only one isolated subcortical white matter infarct was seen. Hemorrhagic lesions, seen in three patients, included a caudate hemormhage (Fig 3), a hemorrhagic cortical infarct (Fig 2), and an isolated barge parenchymab hemorrhage (Fig 4). Gadopentetate dimeglumine, administered in two patients, revealed enhancement in five of eight lesions in one patient, all of which were cortical infarcts. In the second patient, one of three infarcts was enhanced. Thirty-two of 36 lesions identified
Data and Biopsy
Patient/Age (y)/Sex
RESULTS
female) ranged in age from 3 to 44 years (median, 27 years) (Table I). All patients were normotensive. Three patients underwent brain biopsy. In those patients who did not undergo brain biopsy, or in whom the results of brain biopsy were negative, a presumptive diagnosis
an independent
Table 1 Demographic
METHODS
1985 and December agnosed in seven debphia, delphia.
finding on MR images consisted of a large parenchymal hemorrhage. The median time elapsed between performing MR imaging and angiography was 5 days (range, 0-10 days). All studies were analyzed by two readers and, when
In the other six laboratory data cause of the anvasculitis. a presumptive was made.
Biopsy
Results Results
1/27/F
Negative
2/37/M 3/3/F
Negative Not performed
4/14/M 5/44/M 6/23/M 7/27/M
Not
performed
Not performed Positive Not
performed
on MR images could be correlated with positive angiographic findings in the corresponding vascular distribution. The apparent lack of correlation of four lesions resulted from the angiographic data not being available, as none of the patients in our cohort underwent raphy.
four-vessel Specifically,
cerebral angiogin five patients,
three vessels underwent injection, and in two patients, two vessels underwent injection. It should be emphasized that in 12 of 33 cases of vascubar distribution with angiographic evidence of vascubitis, no identifiable lesions
in the
were
corresponding
visualized
Of the seven graphic, clinical, ings diagnostic
territory
on MR images. patients with angioand laboratory findof PACNS who were
included in this study, three underwent brain biopsy. In two patients (patients 1 and 2), the right frontal lobe and its beptomeninges underwent biopsy with negative results. MR examination in a third patient (patient 6) revealed a moderate-sized lesion confined to the deep white matter of the left occipital lobe, which was
interpreted
as a possible
grade glioma (Fig of this lesion were bomatous angiitis.
low-
5). Results of biopsy positive for granu-
DISCUSSION Prompt diagnosis sential. If untreated,
of PACNS is esthis disease is
rapidly
and
progressive
frequently
fatal. Patients are usually middleaged, and there is no sex-related predominance (7). Headache is the most frequent complaint, and signs of both global and focal neurobogic dysfunction are usually identified (7). Symptoms
such
as fever,
myalgia,
arthral-
gia, and arthritis, which occur frequently in other vasculitic syndromes, are conspicuously absent in PACNS (4). The erythrocyte sedimentation rate is elevated in two-thirds of patients (7). Cerebmospinal fluid (CSF) analysis
characteristically
shows January
a 1992
Table 2 Angiograph
ic Evidence
of Arte
Involvement
ritic
ACA
ICA Vessel Injected
Patient
MCA
R
L
R
L
R
SCA
Vert
PCA
L
R
L
R
L
R
L
LCCA,LV
-
-
-
+
-
-
-
-
+
+
+
+
-
+ +
-
+
+ +
-
BCCA,LV
-
-
-
-
3 4 5 6
BCCA,LV BCCA,LV BCCA,LV BCCA,LV LCCA,LV
-
-
+
+
+
+
-
+
-
-
-
-
-
-
+
+
+
+
-
-
+
-
-
5 6
-
-
+ +
+
+
+
+
+
-
-
+
+
8
-
-
-
-
-
-
-
+
-
-
-
-
-
-
-
+
-
+
-
+
-
-
-
-
Note.-ACA = anterior cerebral artery, BCCA = bilateral common carotid arteries, ICA = supraclinoid mon carotid artery, L = left, LV = left vertebral artery, MCA = middle cerebral artery, = no evidence evidence of involvement, R = right, SCA = superior cerebellar artery, vert = vertebral.
of Lesions
Patient
.
SC
DWM
0 0 0 0 0 1 0
6 2 0
I 1* 1
j*
0
3 2 0
2 0 0
3 8
1
14
5
36
1 0 5*
4 5 6
0 0 0
7
7
0 0 0 0 2 0 1
13
3
Total
Total
C/S
1 2 3
weakening
Note-C = cortex, C/S = cortical/subcortical white matter, SC = subcortical white matter. *One lesion was hemorrhagic.
white
matter,
DGM
0GM
deep
=
gray
(35). The angiographic rebral vascubitis are
8 3 6 1
can be seen
7
matter,
DWM
with
an
elevated
(7). Prednisone for
treatment
for
drug
PACNS.
of For
of corticosteroid-resistant
or progressive PACNS, cyclophospharnide can be added to the therapy
The known,
exact cause although
of PACNS numerous
(5).
is unreports
suggest a possible viral cause. The association of herpes zoster ophthalmicus and delayed contralateral hemiparesis is well known (23,24).
Cerebral tients culitis
angiogmaphy shows most
findings cornrnonly
in these
the Ml and A2 segments dle and anterior cerebral
phy
and
logic
history High antibody
vasto
In most of there is also a
of a patient
and used clear
electron microscopy has been to identify herpeslike intranuviral particles in another patient
182
#{149} Number
PACNS
virus from the
CSF
Volume
with
1
enter
into
the
diagnosis
radio-
of cerebral
(29),
to be eccentric
and
involve
segments (36). That
orrhage
produces
who
Willis. Subarachnoid hemorrhage rarely occurs as a consequence of muptume of an arteritic vessel (1). Physical
was
semonegative
munodeficiency virus yet HIV was isolated and CSF (19). Electron
for human
wall
and
within
granubomatous
a patient with ing is supported
im-
(HIV) infection, from the brain microscopy has
mycoplasmabike
in the
traburninab
of the midarteries,
of lymphorna. levels of anti-varicella have been isolated
hence
differential
shorter lesions
tures
respectively (25,26). Histopathobogically, these patients have a granubornatous vasculitis identical to that of PACNS (27,28). Diffuse granubornatous vascubitis has also been described in patients with recent herpes zoster
infection (14-16,27-31). these cases, however,
of noninflarn-
with PACNS (32). The association of PACNS with other viral or viruslike illnesses has also been seen. Specificabby, granubornatous angiitis has mecently been described in a patient
demonstrated
pa-
of cerebral localized
in a variety
of ceand
vasculitis. Atherosclerosis is the most common cause of arterial narrowing seen at angiography. The stenotic lesions of atherosclerosis, however,
protein
is the
therapy
findings nonspecific
to
hemorrhage, and neoplasm may mimic cerebral vasculitis at angiogradeep
=
vessel
secondary process
matory and inflammatory disorders. Noninflammatory vascubopathies such as atherosclerosis, subamachnoid
tend pleocytosis
of a damaged
wall (1) or vasoparabysis an adjacent inflammatory
on MR Images
C
1 3
segment of internal carotid artery, LCCA = left cornof involvement, PCA = posterior cerebral artery, + =
from
Table 3 Distribution
level
3 7
I 2
7
choice
No. of Arteries Involved
structhe
in
PACNS (33). This findby the work of Tho-
mas et al (34), who injected turkey poults with a neurotropic strain of Mycoplasrna gallisepticutn and pro-
duced
a fatal
necmotizing
cerebral
vas-
tions.
When
alternate
with the focal dilatations, is a “ string-of-beads” appearThe vascular narrowings in ceangiitis may be due to spasm,
theme ance. rebrab
edema,
cellular
vascular
infiltration
narrowings
or prolifem-
of the vessel wall, or compression by surrounding thickened meninges from exudate or fibrosis (i). Similarly, focal dilatations may result ation
usual
of an aneurysm
examination,
is well
cause
is rup-
of the circle
CT appearance,
of
and
CSF
in identifyhemor-
rhage. Neoplasms can also produce vascular narrowings and irregularities that resemble arteritis at arteriography.
cubitis. The characteristic angiographic features of cerebral vasculitis consist of vascular narmowings and focal dilata-
the
ture
vasospasm
The
studies should be useful ing cases of subarachnoid
in-
reaction
established.
than do vasculitic subarachnoid hem-
These
findings
may
(meningioma
metastasis
(37) or diffuse or meningeal
(lymphoma, carcinomatosis).
be focal
or glioma) leukemia, CSF
evaluation and MR examination with administration of contrast material will usually indicate the presence of neoplastic disease. Cerebral vasculitis can be of infectious or noninfectious cause. Unfortunately,
cannot other
these
numerous
be distinguished solely on the basis
graphic cerebral nantly
criteria. vasculitis bacterial
Infectious include and viral
rulent
bacterial
arteritis
a complication
of severe
conditions
from one of angiocauses pmedomidisease.
is most
anof Pu-
often
bacterial
Radiology
#{149} 67
Figure
1. Patient
(a) SE 3,000/80
1.
axial
MR
and
image
arteriographic
reveals
images
numerous
obtained
foci of high
in a 27-year-old
signal
patient
intensity
compatible
with
PACNS
with
infarcts
and
multiple
gray
and
white
in the left perisylvian
cortex,
matter
infarcts.
left putamen,
left posterior parietal white matter, and right external capsule (arrows). (b) SE 3,000/80 axial image at a level slightly superior to that in a shows foci of high signal intensity indicative of infarction adjacent to the body of the left lateral ventricle and in the white matter of the left parietal lobe. There is a tiny extraaxial collection in the right frontal region compatible with recent brain biopsy. (c) Early lateral view from an arteriogram of the left common carotid artery shows focal segments of narrowing (arrows) involving the pericallosal and callosomarginal branches of the anterior cerebral artery, which is compatible with vasculitis. In addition, there is nonfilling of multiple middle cerebral branches secondary to extensive vasculitic involvement.
due to Haeinoinfection (38). The most common finding is involvement of the arteries at the base of the brain, as this is the predominant location of purulent arachnoiditis (1,39). More peripheral branches may show vary-
litis, and drug-abuse vasculitis. Periarteritis nodosa is the most common of the systemic necrotizing vascubitides associated with cerebral vascubitis (3).
of SLE developed months later. Central nervous ment in sarcoidosis
The
3%-5%
ing degrees of diffuse Acute bacterial arteritis
sis, sis of
meningitis,
philus
usually
influenzae
narrowing (1). may result in
formation of mycotic aneurysm (39). The arteritis associated with tubercubus meningitis also has a marked tendency to involve arteries at the base of the brain, particularly the supraclinoid portion of the internal carotid artery and the horizontal portion of
the
middle
quently branches.
cerebral also involves The proclivity
artery the
(1), but convexity of syphilis
invade the vasculature is quite dent in tertiary meningovascular
freto
evidis-
ease.
other
bitides, yasu
by necrotizing
and
is usually
the disease tients with
inflammation
ported patients
by Ford and with central
symptoms
tremely
a late
manifestation
(3). In the periarteritis
had
topathobogically,
of
disease,
from
PACNS (3). Cerebral vasculitis has been described to be associated with several of the collagen vascular diseases, including systemic lupus erythematosus
first
(50).
uncommon.
In their
findings
cases of SLE, Ellis and Verity found evidence of cerebral in only 7% . In SLE, as with collagen vascular diseases, vasculitis generally occurs
with disease genhuis
previously
diagnosed
review
in 57 (41) vasculitis
the
other
cerebral in patients
systemic
(41,43-45). Sanders and Ho(42) reported a case of cere-
bral
vasculitis
tom
of SLE.
as the
Systemic
presenting
manifestations
system involveoccurs in only (3). Cerebral angiin association is exceedingly et al (49) mefindings in a vasculitis. His-
the
symp-
described
evidence occlusion of
in
In 1971,
leagues
(SLE) (41-43), scberoderma (44), and rheumatoid arthritis (45), but is exthe neumopathobogic
16
form
of
granubo-
angiitis seen in sarcoid is very to PACNS (4). The two diseases are best distinguished by the presence of systemic samcoidosis and leptomeningitis, which have been seen in all reported cases of angiitis neurosarcoid (3,46-49). CSF analysis and MR examination with contrast material administration will usually demonstrate the presence of leptomeningeal disease as the cause of an arteritis. Illegal drug-abuse vasculitis was
Siekert (40), all nervous system
distinction
patients
patient
matous similar
series of 114 panodosa re-
systemic
enables
of
itis has been described with sarcoidosis but rare (46-48). Lawrence ported angiographic patient with samcoid
primarily of muscular arteries (1). Central nervous system involvement in periarteritis nodosa has been meported to occur in 46% of cases (40)
analysis of CSF will indicate the bactenab cause of the arteritis. A large number of viruses display neurotropism (39). With the exception of herpes
#{149} Radiology
vascu-
however,
acterized
which
68
necrotizing
include GCA, Takaarteritis, Wegener granubomatoand bymphomatoid granulomato(3). Pemiarteritis nodosa is a disease multiple system involvement char-
In syphilitic angiitis, both arteries and veins are affected and there is a predilection for branches of the middle cerebral artery (39). Luetic artemiab lesions are generally diffuse and affect all leptomeningeab vessels, causing difficulty with collateral circubation (39). Results of microbiologic
zoster, however, viruses generally affect vessels of microscopic cabiber(1). The noninfectious vascubitides are numerous. In addition to PACNS are the systemic necrotizing vasculitides, vasculitis associated with collagen vascular disease, neurosarcoid vascu-
systemic
in their
(51)
1970
Rumbaugh reported
by
Citron
and
et
ab
cob-
angiographic
of cerebral vasculitis in 19 addicts who
and/or mostly
used methamphetarnine. Methamphetamine or “speed” arteritis has subsequently been described by numerous
other
investigators
(52-54).
Cerebral vasculitis has also been meported to be associated with heroin (55) and, more recently, cocaine abuse (56,57). The exact pathogenesis of drug-abuse vasculitis is unknown, but a hypersensitivity reaction to the drug and/or to impurities has been postulated
(55). January
1992
tious cause of the vasculitis patient. Physical examination, sis of CSF, and MR and CT
revealed titers history
‘
c-.
were .
C..
#{149}.::
.. CA
...; 1,
Si#{149}
::
a.
patient zostem
b.
Figure
2. Patient 3. Multiple gray matter infarcts in a 3-year-old patient with PACNS. (a) SE 3,000/80 axial image reveals high signal intensity compatible with infarction in the left thalamus and bilateral anterosuperior insular cortex. (b) SE 3,000/80 axial image reveals abnormal high signal intensity in the medial aspect of the left frontoparietal cortex, which is indicative of infarction. A focus of low signal intensity within the infarct represents hemorrhage.
A
Lyme had oph-
In addition, there of systemic disease Results of rheumatoid
unremarkable. and
enzyme #{149}54
,...
disease.
graphs
%Jk0::
-0’
no meningeal were negative. No of recent herpes
thalmicus. evidence patient. #{149}
in any analystudies
Chest
a
was no in any panels
radio-
angiotensin-converting
levels
were
normal.
No pa-
tient had a history of intravenous drug abuse. Although by these exclusionary criteria, PACNS does seem the plausible diagnosis, it is certainly
most con-
ceivable that the observed vascubitic changes could be the initial presentation of one of the systemic vascubitides, such as periarteritis nodosa, or one of the collagen vascular diseases. As previously discussed, a single case of SLE appearing as cerebral vasculitis in the absence of antecedent or synchronous
systemic
disease
reported (42). All patients hort responded favorably with prednisone and/or phamide. At this writing,
were
in remission
has
been
in our coto therapy cycbophosall patients
and
no patient
had
developed any manifestation of a systemic disease. Finally, it is highly unlikely that sarcoidosis was the cause of vascubitis in any of our patients because angiitic neurosarcoid has been reported only in patients with systemic sarcoidosis, which we were able to successfully exclude in our cohort
(2,46-49).
a.
b.
Figure
3. Patient 2. Images obtained in a 37-year-old patient with PACNS and a caudate hemorrhage. (a) SE 600/20 axial MR image reveals a focus of high intensity in the left caudate nucleus compatible with subacute hemorrhage. In addition, there is abnormal low signal intensity within the splenium of the corpus callosum, indicative of infarction. (b) Anteropostenor image obtained early during arteriography of the left common carotid artery reveals focal areas of narrowing involving the supraclinoid internal carotid artery (arrowhead) and the proximal segments of the anterior cerebral artery (solid arrow) and middle cerebral artery (open arrow), compatible with vasculitis.
Cababrese and Mallek (7) found the results of cerebral angiography to be abnormal in 84% of patients with PACNS, with classic findings of arteritis observed in 65%. False-negative angiograms in patients with PACNS are not uncommon, because this disease primarily affects the precapillamy arterioles (58), which are below the resolving capacity of cerebral angiography. Numerous cases of biopsyproved PACNS associated with nor-
mal
cerebral
One of the patients reported herein had biopsy-proved PACNS. Prior to establishing a presumptive diagnosis of cerebral vasculitis in the remaining
had lymphoproliferative disease or a history of malignancy. Because of the nonspecific nature of the angiographic findings in vascubi-
been
six patients, we excluded nonvasculitic vasculopathies to the best of our diagnostic capabilities. Specifically, our cohort was normotensive, and CSF analysis and MR and CT examination revealed no evidence of sub-
tis, no definite causal diagnosis could be made for these six patients without a brain biopsy. Nevertheless, after thorough clinical and laboratory investigation, PACNS was suggested as the presumptive diagnosis on the basis of exclusion. To be more specific, theme was no evidence of an infec-
infectious
arachnoid or
hemorrhage,
leptorneningeal
Volume
182
brain
disease. #{149} Number
1
tumor,
No
patient
artemiograms
been reported (20-22,59,60). to afflict small
in the Despite vessels,
have literature its predilection PACNS has
histopathobogically
shown
to
involve barge branches of the main cerebral arteries (2,61). In fact, for case 1 in their original description of nongranubomatous
the nervous Feigin tous
system,
(2) stated process
angiitis
Cravioto
“a severe
involved
of
and granuboma-
arteries
and
of varying caliber, from the die cerebral artery to the intraparenchymal vessels.” Cerebral angiographic findings in our six patients veins
Radiology
mid-
#{149} 69
with tient
presumed PACNS and one pawith proved PACNS were corn-
patible
with
those
in previous
reports
in the literature (4,6,13). Angiography revealed segmental, diffuse, bilateral disease rial and
that involved infratentorial
the supratentocirculations
mize
biopsy specimens from the frontal hemisphere
morbidity.
The
MR imaging,
are usulobe of the to mini-
leptomeningeab
which
revealed
a moderate-sized lesion in the deep white matter adjacent to the occipital horn of the left lateral ventricle (Fig 5). The lesion was not enhanced with contrast material on CT scans. Results of angiography at the time of MR irnaging were interpreted as negative. In retrospect, subtle regions of focal narmowing were detected in the distribution of the left posterior cerebral amtemy. Hence, this constellation of findings at the lime was suggestive of a bow-grade glioma. Brain biopsy of this lesion was then performed. Tissue analysis of the specimen revealed granubomatous angiilis of the cerebral vessels. 70
#{149} Radiology
by Valvanis case
case of tumor was
et ab (18).
mass
lesion
This
of granulomatous was seen on a CT
scan as a homogeneously and
biopsy is important in that cerebral vascubitis often has a predilection for the beptomeningeal vasculature. Although PACNS is a diffuse disease, it is segmental; hence, brain biopsy mesults can be negative. Three patients in the present study underwent brain biopsy, the results of which confirmed PACNS in only one. The false-negative results in two patients can be considered a sampling error. Both of these patients underwent right frontal lobe and leptomeningeal biopsy. It is interesting that MR examination in one of these patients (patient 2) revealed disease limited to the left hemisphere. Angiography in this patient did reveal vascubitis on the right side that was limited to the supraclinoid segment of the internal carotid artery and proximal segments of the anterior cerebral artery and middle cerebral artery. In the second patient (patient 1), MR imaging also revealed much more extensive disease on the left side. In this case, however, the right carotid artery was not studied. The patient in our cohort who did have positive brain biopsy results (patient 6) was initially evaluated with
CT and
reported
dramatic a brain
biopsy-proved angiitis originally
vessels of all sizes. Only one patient had involvement in a single vascular distribution. It is our opinion that brain biopsy should be reserved for the group of patients in whom thorough clinical investigation, MR imaging, and angiography fail to explain an aquired neurologic deficit. Brain and leptomeningeab ably taken nondominant
An even more PACNS simulating
enhanced
involving
the
cortex
and
subcomtical white matter of the right temporal-occipital lobe. In addition, Johnson et ab (21) described the MR findings in a case of biopsy-proved PACNS that was seen as bilateral parietooccipitab mass lesions that crossed the splenium of the corpus callosum.
All patients were examined strength case
in the with
MR yielded
present study high-field-
imaging,
which
positive
findings.
in each A me-
dian interval of 5 days elapsed tween performing MR imaging performing
cerebral
five
of seven
was
performed
In no case
beand
angiography.
In
patients,
MR imaging
prior
to angiography.
were
steroids
or cytotoxic
agents administered before cerebral angiography. Steroid therapy was begun in only one case after angiography but before MR examination. It is unlikely that in these five patients the severity of cerebral vascubitis, as assessed at the time of angiography, was greater than at the time of MR imaging (a mean of 5 days earlier). Conversely, in the one untreated pa-
lient who underwent MR tion subsequent to cerebral
examinaangiogra-
phy, it is also unlikely that the brain lesions changed significantly in the interval. In addition, in this patient,
all of the ages
lesions
were
tamed
evident
present
several
on
days
on MR ima CT
prior
scan
ob-
to cerebral
angiography.
MR examination tive in all patients sistent
with
the
of PACNS, ease.
results were in this study, known
a diffuse
Common
pathogenesis
segmental
MR
dis-
findings
in our
patient cohort were numerous dian, six), bilateral, supratentorial farcts involving both gray matter. The exact mechanism farction, such as ischemia
nab encroachment
(mein-
and
white of infrom lumi-
or secondary
thrombotic occlusion, not discernible.
On
posicon-
was
MR images,
infarcts
obviously
were
most
commonly
seen in the deep white matter, including the corpus callosum and capsular tracts. Contrary to the findings of Miller et al (62), we saw no predisposition for the periventricubar
white
matter.
These
investigators
used a cohort that did graphic or biopsy-proved
The
cause
vascubilis was
systemic
of the in their
not
have angiovasculitis.
presumptive
cerebral
patient
collagen
population vascular
dis-
b. Figure
4. Patient 4. PACNS presenting as a solitary parenchymal hemorrhage. (a) SE axial 600/20 MR image reveals a large focus of high signal intensity in the right parietal lobe, indicative of a subacute hematoma. (b) Early arteriogram in the anteroposterior projection obtained during injection of the left vertebral artery shows multiple foci of narrowing (arrows) involving branches of the posterior cerebral arteries bilaterally, compatible with vasculitis.
ease. It seems possible that the ventricular lesions they described might in fact be the sequelae of vasculitic vascubopathy known cur in patients with this group eases,
particularly
SLE
pena nonto ocof dis-
(41).
January
1992
involves both rior circulations
latume.
The
the
anterior and of the cerebral
characteristic
postevascu-
findings
at
MR imaging are multiple, bilateral gray and white matter lesions that in this study were confined to the supratentoriab space. There appears to be no predisposition for the periventricubar region, although deep white matter lesions were common. PACNS can present somewhat atypically as primary parenchymal hemorrhage or simulate a primary brain tumor. Although MR images were positive in !4::i
all our
-
Figure 5. Patient 6. PACNS simulating a low-grade glioma. SE 3,000/80 axial MR image shows a moderate-sized focus of high signal intensity in the deep white matter of the left occipital lobe. The lesion exerts slight mass effect on the occipital horn of the left lateral ventricle. Biopsy of this lesion revealed granulomatous angiitis.
tion with angiographicabby proved vasculitis, however, no lesion in the corresponding vascular territory could be identified at MR imaging. Although MR images were positive in all patients with PACNS, extrapolation from these results suggests that cases of MR-negative PACNS are entimely possible. It is interesting that no infratentomial lesions were depicted with MR imaging despite cerebral angiogmaphy revealing evidence of vasculitis involving the posterior circulation in all patients. Our data suggest that positive findings on MR images might be used to tailor cerebral angiography in cases of “suspected vascubitis.” In summary, PACNS is a mare disease
that
diffusely
JMiimo1R9#{149}T%Jimhor1
and
segmentally
on the basis
of extrapola-
tion from our correlative angiographic data, it is possible that larger number of patients with rare disease are studied, there cases with normal MR images.
as a this may be
literature.
Arthritis
Rheum
1982;
25:
342-345. 18.
Valvanis A, Friede R, Schubiger 0, Hayek J. Cerebral granulomatous angiitis simulating brain tumor. J Comput Assist Tomogr 1979;
19.
Yankner BA, Skolnik PR, Shoukimas GM, Gabuzda DH, Sobel RA, Ho D. Cerebral granulomatous angiitis associated with isolation of human T-lymphotropic virus type III from the central nervous system. Ann Neurol 1986; 20:362-364. Koo EH, Massey EN. Granulomatous angiitis of the central nervous system: protean manifestations and response to treatment. J Neurol Neurosurg Psychiatry 1988;
3:536-538.
20.
51:1126-1133.
21.
22.
#{149}
Johnson M, Maciunas R, Dutt P. Clinton ME, Collins R. Granulomatous angiitis masquerading as a mass lesion. Surg Neurol 1989; 31 :49-53. Scully R. Massachusetts General Hospital Case Records, case 8-1989: weekly clinicopathological exercises. N Engl J Med 1989; 320:514-524.
23.
References 1. 2.
Three separate hemorrhagic lesions were seen in three patients in our study. One of these lesions was a hemorrhagic cortical infarct associated with other nonhemorrhagic cortical infarcts, which most likely bled as a result of a reperfusional event. Another was a hemorrhage of indeterminate cause in the head of the caudate. The consequence of vessel wall rupture almost certainly was a large hemorrhage in the deep white matter of the might panietal lobe in the third patient. This case was extremely interesting in that this 14-year-old patient was first seen with a primary parenchymal hemorrhage as a single lesion. For every lesion identified at MR imaging, findings of cerebral vasculitis were positive at angiography in the corresponding vascular distribution. In 12 of 33 cases of vascular distnibu-
cases,
of the
Ferris EJ, Levine HL. Cerebral arteritis: classification. Radiology 1973; 109:327-341. Cravioto H, Feigin I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology 1959; 9:599-609.
3.
4.
Sigal LH. The neurologic presentation of vasculitic and rheumatologic syndromes. Medicine 1987; 66:157-180. Nurick 5, Blackwood W, Mair WGP. Giant cell granulomatous angiitis of the central nervous system. Brain 1972; 95:133142.
5.
6.
Cupps TR, Moore PM, Fauci AS. Isolated angiitis of the central nervous system. Am Med 1983; 74:97-105. Craven RS, French JK. Isolated angiitis of the central nervous system. Ann Neurol 1985;
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
25.
26.
27.
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