GRANULOMATOUS AUGUTIS/Burger et al.
detrimental (61.1% to 62.5%) than beneficial (38.9% or
37.5%).13
Correlations between the type of response (beneficial or
detrimental) and various factors have been searched for in
order to predict the type of response in other patients. Only
angiographical findings gave a positive correlation. As
shown in table 2, the probability for the occurrence of a
decreased perfusion is higher when the arteriogram shows an
intracranial thrombosis. It is indeed conceivable that a cerebral region deprived of its main arterial supply but with all
its patent arteries already maximally dilated can by no
means benefit by the action of a vasodilator.
References
1. de Rood M, Capon A, Mouawad E, et al: Effects of halothane on
regional cerebral blood flow. Acta Anaesth (Belg) 25: 82-99, 1974
2. Capon A, Fruhling J, Verbist A, et al: Exploration fonctionnelle du debit
cerebral local a la gamma-camera. XVe coll. Med. Nucl. Langue Franc,
Louvain, pp 134-137, 1973
3. Verbist A, Capon A, Fruhling J: A rapid method for evaluation of
regional cerebral blood flow after intra-arterial injection of ""Xe. J Nucl
Med 16: 264-266, 1975

29

4. Heiss WD, Prosenz P, Roszuczky A, et al: A quantitative gamma camera
technique. Scand J Clin Lab Invest 22 (Suppl 102): XI:L, 1968
5. Heiss WD, Prosenz P, Roszuczky A: Technical considerations in the use
of a gamma camera 1600 channel analyzer for the measurement of
regional cerebral blood flow. J Nucl Med 13: 534-543, 1972
6. Ingvar DH, Lassen NA (eds): Brain Work. Copenhagen, Munksgaard,
1975
7. Fruhling J, Verbist A, de Rood M, et al: Etude du debit sanguin cerebral
regional par analyse des courbes d'elimination du Xenon"3. Analyse de la
reproductibilite des mesures. J Belg Radiol 56: 550-551, 1973
8. Wilkinson IMS, Bull JWD, du Boulay GH, et al: Regional blood flow in
the normal cerebral hemisphere. J Neurol Neurosurg Psychiat 32:
367-378, 1969
9. Capon A, Hildebrand J: Unpublished observations
10. McHenry LC Jr, Goldberg HI, Jafle ME, et al: Regional cerebral blood
flow. Response to carbon dioxide inhalation in cerebrovascular disease.
Arch Neurol (Chicago) 27: 403-412, 1972
11. Charlet JP, Marc-Vergnes JP: Quelques pieges methodologiques dans la
mesure du debit sanguin cerebral. In Geraud J, Lazorthes G, Bes A (eds):
L'ischemie Cerebrale dans le Territoire Carotidien. Toulouse, pp
207-210, 1972
12. Paulson OB: Regional cerebral blood flow at rest and during functional
tests in occlusive and non-occlusive cerebrovascular disease. In Brock M,
Fieschi C, Ingvar DH, et al (eds): Cerebral Blood Flow. Berlin, Springer,
pp 111-114, 1969
13. Olesen J: Cerebral blood flow, methods for measurement, regulation.
Effect of drugs and changes in disease. Acta Neurol Scand 50 (Suppl 57)
1974

Granulomatous Angiitis
An Unusual Etiology of Stroke
PETER C. BURGER, M.D.,

J. GORDON BURCH, M.D.,

AND F. STEPHEN VOGEL,

M.D.

SUMMARY A 43-year-old man, who died five months after
the onset of left-sided sensory deficit, had angiographical and
pathological evidence of an angiitis confined largely to the distribution of the right middle cerebral artery. Histological examination identified this process to be intracranial noninfectious granulomatous angiitis. Although certain clinical and pathological features
of this disorder overlap with other vasculitides which affect the cen-

tral nervous system, the disease nevertheless retains sufficient
individuality to warrant status as an entity, and should be considered in the differential diagnosis in adults with lesions which
produce focal neurological deficits and signs of increased intracranial pressure.
The definitive answer regarding an infectious etiology will come
only from detailed culture studies of the affected vessels.

Introduction

characterized by numbness of the left foot, staggering gait,
difficulty with speech, and decreased use of the left arm and
leg. Within a week the numbness extended to the entire left
side of the body, at which time there was urinary urgency
with precipitate micturation, anorexia, and a six-pound
weight loss. The patient denied seizure activity, headache,
visual disturbance, or loss of consciousness. The past medical history contained no relevant illnesses. Specifically,
there was no history of asthma, allergies, sinusitis, or lesions
of the skin.
The patient was well developed and cooperative, and did
not appear ill. The blood pressure was normal. Decreased
sensation to pinprick and light touch was noted over the left
lower face involving the lip and cheek and extending almost
to the midline. Examination of the remaining cranial nerves
was negative. The left arm drifted on outstretched posture
and there was decreased grip in the left hand associated with
clumsiness in succession and fine finger movements. The left
leg was not weak but heel and foot tapping was slow. The
deep tendon reflexes and plantar responses were normal. A
Hoffmann response was present on the left. There was

GRANULOMATOUS ANGIITIS is an inflammatory disorder of unknown etiology whose curious preference for
vessels of the central nervous system may be expressed
clinically as stroke. This report describes the clinical and
pathological manifestations of this disorder and considers its
nosological position among the other vasculitides which may
affect the central nervous system.
Report of a Case
Clinical History
The patient was a 43-year-old black man who was first admitted to Duke University Medical Center on November 9,
1970, for evaluation of repetitive attacks of numbness of the
left side of the body which had begun two months
previously. The initial attack of 20 minutes' duration was
From the Department of Pathology (Drs. Burger and Vogel) and the Division of Neurology (Dr. Burch), Duke University Medical Center, Durham,
North Carolina 27710.

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

30

STROKE

decreased sensation to pinprick and touch over the left hand
and forearm to the elbow and in the left leg from the knee
distally. Position sense was impaired in the left great toe and
vibratory sensation was diminished in the left hand. The patient walked with a very slight limp of the left leg.
Normal routine laboratory data included complete blood
count with WBC differential, urinalysis, serologic test for
syphilis, and serum chemical examinations including blood
urea nitrogen. A brain scan revealed increased tracer activity in the right posterior parietal area. The ECG, EEG, and
roentgenographic examinations of the chest and skull were
normal. On the fourth hospital day, a right carotid arteriogram revealed a fusiform dilation of the intracavernous portion of the right carotid artery in the absence of other
arterial abnormalities. The venous phase of this examination
disclosed a 4-cm avascular area in the right parietal region.
A neoplasm producing focal signs and sensory seizures was
suspected, but the patient refused pneumoencephalography
and rejected the suggestion of surgery. Therefore, he was
discharged from the hospital with prescriptions for Dilantin
and phenobarbital.
On December 1, 1970, the patient returned to the clinic
with the complaint of occipital headache, but appeared well
otherwise. Increased weakness of the left arm was apparent
to the examiner.
On February 11, 1971, the patient was readmitted to the
hospital because of headache, persistent vomiting, confusion, and left hemiplegia of sudden onset four hours earlier.
Somnolence, confusion, and disorientation characterized his
general state. The pulses were full and there were no cranial
or cervical bruits. The right pupil was slightly greater than
the left, but both reacted to light. The left corneal reflex was
decreased. Left central facial weakness and left hemiplegia
were noted. There were bilateral papilledema and retinal
hemorrhages. There were no motor responses to pinprick on
the entire left side.

VOL 8, No

1, JANUARY-FEBRUARY

1977

A right carotid arteriogram again revealed an avascular
area in the right parietal region, and in addition, for the first
time, also demonstrated irregular beaded luminal profiles
with focal occlusions in the parietal branches of the middle
cerebral artery (fig. 1). Retrograde pial collaterals extended
toward the region of avascularity. The internal cerebral vein
and anterior cerebral arteries were shifted to the left and
there was downward displacement of the middle cerebral
artery at the sylvian point. Steroid therapy was begun.
On the second hospital day, a left ventricular tap revealed
clear cerebrospinal fluid at an opening pressure of 600 mm
H2O. Ventriculography demonstrated a 1-cm shift of the
right lateral ventricle to the left. Because of the deteriorating neurological status indicative of uncal herniation, an exploratory right parietal craniotomy was performed. The
dura was extremely tense and when incised in the anteroinferior parietal region the brain ruptured spontaneously
through the pia. The inferior portion of the right parietal
lobe was yellow and avascular. A ventricular needle was introduced in an attempt to locate an intracerebral hematoma,
but none was encountered and the operative procedure was
terminated. The patient died shortly thereafter.
Pathological Findings

The contours of the 1,450-gm brain were distorted by a
large, soft, recent infarct in the right frontoparietal area.
The expansile character of this lesion had induced subfalcine
and transtentorial herniations and had produced a groove in
the ipsilateral third cranial nerve. Although confined largely
to the parietal and posterior frontal lobes, the infarct extended into the anterior portion of the occipital lobe and also
involved the superior temporal gyrus. Within this recent lesion, there were scattered older (2 to 3 cm) cystic infarcts.
Petechiae were dispersed throughout both cerebral
hemispheres, but were most prominent on the right.
The large vessels at the base of the brain were unremark-

FIGURE 1. The arterial phase of a carotid angiogram performed on the day before death reveals beaded
luminal profiles in the posterior parietal branches of the right middle cerebral artery (arrows).

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

31

GRANULOMATOUS ANGIITIS/Burger et al.
able and essentially free of atherosclerosis. The peripheral
branches of the middle cerebral arteries over the cerebral
convexities, especially on the right, showed segmental
nodular thickenings associated with marked reductions in
luminal diameters.
The recent cerebral infarcts were characterized
microscopically by myelin pallor, neuronal eosinophilia and
karyolysis, and marked perivascular and perineuronal
vacuolation. The older lesions were either cystic or filled
with gitter cells, and sometimes surrounded by hemosiderinladen macrophages and mineralized neurons.
Throughout both cerebral hemispheres, but to a much
greater extent on the right in the distribution of the middle
cerebral artery, there was a striking, severe granulomatous
inflammation of arteries, arterioles, and, to a lesser extent,
veins and venules (figs. 2 to 7). The angiitis was much more
prominent in the pial vessels, but also affected those within
the parenchyma. The basic lesions were aggregates of
epithelioid cells containing occasional multinucleated forms.
These aggregates involved either the entire vascular circumference (fig. 2) or just a sector (fig. 3) and were
noteworthy for their segmental distributions (figs. 4 and 5).
Small clusters of these epithelioid cells sometimes appeared
independent of vessels so as to simulate intraparenchymal
tubercles, but adjacent sections usually demonstrated a
vascular association, or a reticulin stain disclosed the outlines of a vessel in the midst of the epithelioid response.
The epithelioid aggregates in arteries were usually
positioned within the outer media and adventitia (figs. 2, 3,
and 4). Although the intima sometimes shared in the

epithelioid response, more often it showed only thickening
by fibrous tissue, foam cells, lymphocytes, and recent and
old thrombi (figs. 2 and 4). The exact position of the
epithelioid cells in veins was more difficult to define and their
infiltration was more often transmural (figs. 5 and 6).
Distinguishing arterial or venous landmarks were sometimes
obliterated by the severity of involvement. Conspicuous
lymphocytic aggregates containing a rare eosinophil cuffed
both the affected vessels and smaller vessels throughout the
parenchyma.
Although the vast majority of the granulomatous lesions
were noteworthy for the absence of necrosis, in two vessels,
deeply eosinophilic granular necrotic areas were surrounded
by elongated epithelioid cells.
In addition to the scattered multinucleated forms that
were randomly dispersed within the epithelioid aggregates,
other larger multinucleated cells were aligned along the inner media where they cradled or surrounded amorphous
basophilic material (fig. 7). These masses were stained
positively for elastic tissue or, less commonly, for iron, and
were sometimes noted along the intima without the
epithelioid response. These were not consistent findings,
however, and the media of many vessels were overrun by
epithelioid cells in the absence of any apparent abnormalities in the internal elastic lamellae. One artery contained
multiple giant cells that surrounded lucent, birefringent
fibers similar to those presumed to be cotton fibers
sometimes noted after cerebral arteriography. 1
In addition to the active lesions described above, many
vessels, especially the larger pial branches of the right mid-

6**^.
.

' - < ;

:,-,,„,- T
A*i
_j~*i-_ - . . • #„_r
FI
FIGURE
2. n/:.L
With the
exception
of
the exit point
of
a smaller
vessel,i
the entire circumference of this medium-sized pial artery is greatly
thickened and its lumen narrowed by the presence of epithelioid
cells within the media and adventitia, and byfibrosis and chronic inflammation within the intima. Note the cotlagenization and
mononuclear cell infiltration of the pia (H & E, X60).

FIGURE 3. Distinctively, as in this pial artery, granulomatous
angiitis may involve only a sector of a vessel's circumference with
disruption of the media and replacement with epithelioid and mononuclear cells (H & E. XI00).

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

32

STROKE

VOL

8, No

1, JANUARY-FEBRUARY

1977

FIGURE 5. The nodular epithelioid foci along the course of this
serpentine cerebral vein underscore the segmented character of this
process. The adjacent white matter shows astrogliosis and losses of
myelin and axis cylinders (H & E, XI70).
FIGURE 4. Longitudinal sections of the wall of a large cerebral
artery show, in a Masson preparation (right) (XI70), the presence
of epithelioid cells in the media and associated thickening of the underlying inlima. The resultant disorganization of the media is better
appreciated in an adjacent section stained for reliculin by the Wilder
technique (left) (XI70). I = intima, M = media, A = adventitia.

die cerebral artery, showed lesions of greater chronicity as
evidenced by organized thrombi, scant inflammatory infiltrates, and mural and perithelial fibrosis.
PAS and methenamine silver stains on histological sections from multiple areas failed to disclose fungi; similarly
negative were stains for acid fast bacilli, gram negative or
positive organisms, and spirochetes.
The brain stem, cerebellum, and spinal cord were free of
vasculitis. The latter showed Wallerian degeneration of the
left lateral corticospinal tract.
The general autopsy revealed the aorta and carotid
arteries in the neck to be unremarkable. The heart was normal and showed no evidence of valvular disease or mural
thrombus formation. There were multiple small old infarcts
in both kidneys.
Careful examinations of multiple histological sections disclosed only one extracranial focus of angiitis which appeared
as a nodular aggregate of epithelioid cells within the intima
of a lobar vein in the right kidney (fig. 8). One lymph node at
a pulmonary hilum contained a cluster of epithelioid and

giant cells without necrosis. No acid fast bacilli or fungi
were identified within either of these granulomatous foci.
Discussion
The clinical course, the angiographical findings, and the
postmortem examination of the present case document the
presence and effect of an angiitis largely confined to the nervous system and characterized by a vascular infiltration of
epithelioid cells, giant cells, and lymphocytes in the absence
of a demonstrable infectious agent. As such, this case conforms to the "entity" of granulomatous, or giant-cell
granulomatous, angiitis. 29 Similar, and possibly identical,
lesions have been reported under the term giant-cell
arteritis.10- "
Granulomatous angiitis has rather
distinctive
characteristics that appear to define it as a clinicopathological entity. However, its true individuality rests on
the differentiation of this disorder from the other vasculitides which affect the central nervous system. Notable
among the latter are the collagen vascular diseases, sarcoidosis, giant-cell (temporal) arteritis, granulomatous
angiitis associated with Hodgkin's disease, allergic angiitis,
infectious angiitis, Takayasu's disease, and rheumatoid
arthritis. Some of these are readily distinguished from the
granulomatous angiitis in question, others are not.

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

GRANULOMATOUS ANGUTIS/Burger et al.

33

The vascular lesions of the collagen-vascular diseases of
polyarteritis nodosa 12 " and systemic lupus erythematosus18
are known for their predominant extracranial positions and,
in the case of lupus, for a predilection for young women. By
contrast, granulomatous angiitis is predominantly intracranial, affects the sexes in approximately equal incidence,
and usually occurs later in life. Furthermore, the acute
necrotizing character of polyarteritis,12"17 and to a lesser extent lupus, the propensity of lupus to involve smaller
vessels,18 and the tendency, although not absolute,17 of polyarteritis to spare veins are distinguishing features. It is of interest that the small aneurysms which characterize the extracranial vessels in polyarteritis may in certain cases also be
visualized roentgenographically in the cerebral vessels.16

FIGURE 6. The dominance of epithelioid cells with scattered multinucleated forms is apparent within this cerebral vein
(H & E, X400).

As evidenced by cases reported as sarcoidosis, but which
could also be interpreted as granulomatous angiitis,19-20 or
vice versa by cases reported as granulomatous angiitis which
might be sarcoidosis,6 these two entities are not always
readily distinguished. Both are or may be systemic diseases
and are characterized by non-caseating granulomatous inflammation. Sarcoidosis, moreover, may feature a prominent vasculitis in other organs.21 The character of sarcoidosis with its epithelioid cells, giant cells, and elastic
membrane degeneration is unquestionably very similar to
that of granulomatous angiitis. Sarcoidosis, however,
usually is positioned at the base of the brain, although disseminated lesions may occur,22 and is noted more for discrete perithelial tubercles rather than the unrestrained
epithelioid cell proliferation of granulomatous angiitis.23
While not a conspicuous part of the latter entity, necrosis is
still more prominent than in most cases of sarcoidosis.
A number of cases of intracranial arteritis similar, if not
identical, to granulomatous angiitis have been reported as

•

'

-

* •

FIGURES 7A AND B. A resemblance to giant-cell (temporal) arteritis is imparted in some vessels by the
presence of multinucleated giant cells of the foreign body type which tend to aggregate about degenerative
material with the tinctorial properties of elastica. The dark-staining intimal and medial plaques in the two
vessels at the right of illustration A stained positively for elastica and iron. (A) H & E, XlOO; (B) H & E. X425.

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

•

.

34

STROKE

FIGURE 8. In contrast to most other vasculitides, granulomatous
angiitis usually eschews involvement of extracranial vessels. In the
present case, this solitary granulomatous nodule was an isolated
finding in a renal vein; its morphology and position resemble, but
are not entirely identical to, the intracranial
vasculitis
(H & E, X250).

"giant-cell arteritis." By either statement or implication, it
has been suggested that these cases bear identity with giantcell arteritis of the temporal arteries.10- "• 24 Certainly, the
presence of giant cells and elastic membrane degeneration is
common to both granulomatous angiitis and temporal
arteritis, the age of incidence is similar, and there are welldocumented examples of concomitant temporal arteritis and
intracranial angiitis wherein veins may also show involvement.25 Elevated erythrocyte sedimentation rates are also
seen frequently in both conditions. However, in general,
temporal arteritis is known for its aversion of the central
nervous system, it eschews venous involvement, and
pathologically it is not characterized by the marked granulomatous response of granulomatous angiitis.26-27 Unfortunately, the temporal arteries were not examined in the
present case.
Rarely, a cerebral vasculitis of granulomatous nature
characterized by epithelioid cells, giant cells and
lymphocytes in small arteries and veins is associated with
Hodgkin's disease.28 Although this angiitis is in many
respects identical to the cerebral granulomatous angiitis in
the present report, its relationship, if any, to the latter entity
remains uncertain.
An entity described as allergic granulomatosis shares the
epithelioid cell response of granulomatous angiitis, but in
the original report it was noted for extracranial disease and a
close association with asthma.29 Although asthma has not
been documented in most cases of granulomatous angiitis, it
was present in one case in which there was extensive extracranial involvement.3 Other allergic vasculitides are known
for systemic distribution and marked necrotizing vascular
changes.30-31
A granulomatous vasculitis is frequently a component of

VOL 8, No

1, JANUARY-FEBRUARY

1977

tuberculous meningitis, but is distinguished by the presence
of acid fast bacilli together with necrosis.32 Because of their
ability also to elicit granulomatous responses, other microorganisms such as Listeria monocytogenes and Treponema
pallidum have been evaluated as etiological agents in
granulomatous angiitis.9 Serologic tests for syphilis in patients with the latter disorder have, with only rare exception,
however, been negative, and no organisms have been
cultured from the cerebrospinal fluid or identified in tissues.
Aspergillosis and phycomycosis (mucormycosis) are
noteworthy for vasculitis, but these infections most frequently occur in the immunologically compromised patient
or diabetic, pursue a rapid course, and are caused by the unmistakable large hyphae.33
Takayasu's disease and other diseases of the aortic arch
often produce prominent cerebral symptomatology, but do
so by their stenosing effect on the aorta and its large extracranial branches.34 Aortic arch disease, furthermore, has not
been associated with documented intracranial granulomatous angiitis and the pathological findings, although not
completely characterized and usually evaluated in the
chronic stage, are predominantly those of chronic inflammation and fibrous scarring rather than the exuberant
epithelioid response of granulomatous angiitis.36-36
Changes consistent with cerebral vasculitis have been
documented by angiography in rheumatoid arthritis,37 but
there has been no consistent history of significant joint disease associated with intracranial granulomatous angiitis.
Thus a number of arteritides share clinical and/or
pathological features of granulomatous angiitis. For the
reasons enumerated above, we suggest that intracranial
granulomatous angiitis be viewed as an entity, at least until
more compelling data are at hand to establish its relationship to other forms of extracranial or intracranial angiitis.
As in the present case, this disorder usually presents in
adults in the fifth through eighth decades with mental
changes and localized neurological deficit in accompaniment
with signs and symptoms of increased intracranial pressure
— a combination which may initiate concern about a neoplasm. The cerebrospinal fluid has consistently revealed
lymphocytosis and elevated protein. Cerebral arteriography
in at least one previous case designated as "giant-cell
angiitis" demonstrated changes similar to those of the present case." The disorder ordinarily terminates fatally in from
1 to 12 months, although the course in some patients is more
prolonged.8
Although usually restricted to the central nervous system,
complete autopsies in some of the previous cases have shown
a systemic component by the demonstration of cardiac,4 aortic,4 lymph node,4-6 pulmonary, 4 - 8 - 8 or abdominal visceral4- 8 - 8 granulomatous angiitis. The present case includes the
kidney among these extracranial sites. The nodal focus of
granulomatous inflammation within a mediastinal lymph
node in this case is a common autopsy finding, so that
although it may relate to the granulomas in the kidney and
the brain, it may also be coincidental.
The etiology of granulomatous angiitis is unknown. The
failure to demonstrate stainable organisms within the tissue
or recover infectious agents in the cerebrospinal fluid has encouraged the use of the prefix "noninfectious." However,
like sarcoid, the intense granulomatous response is tantalizing to those accustomed to associate such inflammation with

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

GRANULOMATOUS ANGIITIS/Burger et al.
infection. Kolodny et al.8 have noted a similarity between
this human disease and a cerebral angiitis associated with
mycoplasma infection in young turkeys,38 but, like many
others, are more inclined to view human granulomatous
angiitis as an autoallergic phenomenon. One case has
responded favorably to steroids.8 Clearly, the definitive
answer regarding an infectious etiology will come only from
detailed culture studies of the affected vessels.
Addendum
Since this manuscript was submitted, two case reports of
granulomatous angiitis have appeared which bear on the
possible infectious etiology of this entity. Reyes et al.
(Neurology 26: 797-799, 1976) identified intranuclear
"virus-like" particles in brain tissue obtained postmortem.
Scully et al. (New EnglJ Med295: 944-950, 1976) discussed
the pathological findings in a cortical biopsy specimen and
reported negative results of culture for tubercle bacilli and
other infectious agents.
References
1. Silberman J, Cravioto H, Feigin I: Foreign body emboli following cerebral angiography. Arch Neurol 3: 711-717, 1960
2. Harbitz F: Unknown forms of arteritis, with special reference to their
relation to syphilitic arteritis and periarteritis nodosa. Am J Med Sci 163:
250-272, 1922
3. Newman W, Wolf A: Noninfectious granulomatous angiitis involving the
central nervous system. Trans Am Neurol Assoc 77: 114-117, 1952
4. Cravioto H, Feigin I: Noninfectious granulomatous angiitis with a
predilection for the nervous system. Neurology 9: 599-609, 1959
5. Budzilovich GN, Feigin I, Siegel H: Granulomatous angiitis of the nervous system. Arch Path 76: 250-256, 1963
6. Peison B, Padleckas R: Granulomatous angiitis of the central nervous
system. Ill Med J 126: 330-334, 1964
7. Hughes JT, Brownell B: Granulomatous giant-celled angiitis of the central nervous system. Neurology 16: 293-298, 1966
8 Kolodny EH, Rebeiz JJ, Caviness VS Jr, et al: Granulomatous angiitis of
the central nervous system. Arch Neurol 19: 510-524, 1968
9. Nurick S, Blackwood W, Mair WGP: Giant-cell granulomatous angiitis
of the central nervous system. Brain 95: 133-142, 1972
10 McCormick HM, Neubuerger KT: Giant-cell arteritis involving small
meningeal and intracerebral vessels. J Neuropath Exp Neurol 17:
471-478, 1958
11. Hinck VC, Carter CC, Rippey JG: Giant-cell (cranial) arteritis. A case
with angiographic abnormalities. Am J Roentgenol Rad Ther Nucl Med
92: 769-775, 1964
12. Kernohan JW, Woltman HW: Periarteritis nodosa. A clinicopathologic
study with special reference to the nervous system. Arch Neurol Psychiat
39: 655-686, 1938
13. Winkelman NW, Moore MT: Disseminated necrotizing panarteritis
(periarteritis nodosa). A clinicopathologic report. J Neuropath Exp
Neurol 9: 60-77, 1950
14. Malamud N: A case of periarteritis nodosa with decerebrate rigidity and

35

extensive encephalomalacia in a five-year-old child. J Neuropath Exp
Neurol 4: 88-92, 1945
15. Foster DB, Malamud N: Periarteritis nodosa. A clinicopathologic report
with special reference to the central nervous system. A preliminary
report. Univ Hosp Bull (Mich) 7: 102-104, 1941
16. Leonhardt ETG, Jakobson H, Ringqvist OTA: Angiographic and
clinicophysiologic investigation of a case of polyarteritis nodosa. Am J
Med 53: 242-256, 1972
17. Moskowitz RW, Baggenstoss AH, Slocumb CH: Histopathologic
classification of periarteritis nodosa: A study of 56 cases confirmed at
necropsy. Proc Staff Meet Mayo Clin 38: 345-357, 1963
18. Johnson RT, Richardson EP: The neurological manifestations of systemic lupus erythematosus. A clinical-pathological study of 24 cases and
review of the literature. Medicine 47: 337-369, 1968
19. Bottcher E: Disseminated sarcoidosis with a marked granulomatous
arteritis. Arch Path 68: 419-423, 1959
20. Meyer JS, Foley JM, Campagna-Pinto D: Granulomatous angiitis of the
meninges in sarcoidosis. Arch Neurol Psychiat 69: 587-600, 1953
21. Michaels L, Brown NJ, Cory-Wright M: Arterial changes in pulmonary
sarcoidosis. Arch Path 69: 741-749, 1960
22. Cares RM, Gordon BS, Kreuger E: Boeck's sarcoid in chronic
meningoencephalitis. Organic psychosis with massive softening due to
Boeck's disease. J Neuropath Exp Neurol 16: 544-554, 1957
23. Jefferson M: Sarcoidosis of the nervous system. Brain 80: 540-556, 1957
24. Hirsch M, Mayersdorf A, Lehmann E: Cranial giant-cell arteritis. Br J
Radiol 47: 503-506, 1974
25. Kjeldsen MH, Reske-Nielsen E: Pathological changes of the central nervous system in giant-cell arteritis. Report of a case. Acta Ophthalmol 46:
49-56, 1968
26. Wilkinson IMS, Russell RWR: Arteries of the head and neck in giantcell arteritis. A pathological study to show the pattern of arterial involvement. Arch Neurol 27: 378-391, 1972
27. Hamilton CR Jr, Shelley WM, Tumulty PA: Giant-cell arteritis: Including temporal arteritis and polymyalgia rheumatica. Medicine 50: 1-27,
1971
28. Rewcastle NB, Tom MI: Noninfectious granulomatous angiitis of the
nervous system associated with Hodgkin's disease. J Neurol Neurosurg
Psychiat 25: 51-58, 1962
29. Churg J, Strauss L: Allergic granulomatosis, allergic angiitis, and
periarteritis nodosa. Am J Path 27: 277-301, 1951
30. Paronetto F: Systemic nonsuppurative necrotizing angiitis. In Miescher
PA, Muller-Eberhard HJ (eds): Textbook of Immunopathology, vol II.
New York, Grune and Stratton, pp 722-732, 1969
31. McCombs RP: Systemic "allergic" vasculitis. Clinical and pathological
relationships. JAMA 194: 1059-1064, 1965
32. Moore MT: Meningitis. In Minckler J (ed): Pathology of the Nervous
System, vol III. New York, McGraw-Hill, pp 2391-2397, 1972
33. Fetter BF, Klintworth GK, Hendry WS: Mycoses of the Central Nervous
System. Baltimore, Williams and Wilkins, pp 25-37, 159-169, 1967
34. Currier RD, Dejong RN, Bole GG: Pulseless disease: Central nervous
system manifestations. Neurology 4: 818-830, 1954
35. Rosen N, Gaton E: Takayasu's arteritis of coronary arteries. Arch Path
94: 225-229, 1972
36. Huson REB: Cardiovascular Pathology, vol I. London, Edward Arnold,
pp 531-538, 1965
37. Sievers K, Nissila M, Sievers U-M: Cerebral vasculitis visualized by
angiography in juvenile rheumatoid arthritis simulating brain tumor.
Acta Rheum Scand 14: 222-232, 1968 •
38. Thomas L, Davidson M, McCluskey TR: Studies of PPLO infection: I.
The production of cerebral polyarteritis by mycoplasma gallisepticum in
turkeys; the neurotoxic property of the mycoplasma. J Exp Med 123:
897-912, 1966

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016

Granulomatous angiitis. An unusual etiology of stroke.
P C Burger, J G Burch and F S Vogel
Stroke. 1977;8:29-35
doi: 10.1161/01.STR.8.1.29
Stroke is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 1977 American Heart Association, Inc. All rights reserved.
Print ISSN: 0039-2499. Online ISSN: 1524-4628

The online version of this article, along with updated information and services, is located on the
World Wide Web at:
http://stroke.ahajournals.org/content/8/1/29

Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in
Stroke can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office.
Once the online version of the published article for which permission is being requested is located, click Request
Permissions in the middle column of the Web page under Services. Further information about this process is
available in the Permissions and Rights Question and Answer document.
Reprints: Information about reprints can be found online at:
http://www.lww.com/reprints
Subscriptions: Information about subscribing to Stroke is online at:
http://stroke.ahajournals.org//subscriptions/

Downloaded from http://stroke.ahajournals.org/ by guest on June 28, 2016