Brain Infarction Caused
by Syphilitic Aortic Aneurysm
A Case Report
Hiroshi Nakane, M.D.
†
†
Yasushi Okada, M.D.
Setsuro Ibayashi, M.D.
†
Seizo Sadoshima, M.D., F.A.C.A.
†

and Masatoshi Fujishima, M.D.
†
FUKUOKA CITY, JAPAN

- ABSTRACT
Neurosyphilis often develops brain infarction. Recently, the authors experienced a case of
brain infarction caused by syphilitic aneurysm on the thoracic aorta, and its clinical significance is reported. A seventy-eight-year-old woman with strongly positive serologic tests
for syphilis suddenly developed global aphasia and right hemiplegia. Brain computed
tomography (CT) showed a large brain infarction in the left middle cerebral artery
territory. A huge aneurysm of the ascending aorta eroded the sternum and the second
and third ribs. The left common carotid artery was completely compressed by the
aneurysm, and blood flow was not demonstrated on enhanced CT. A cerebrospinal fluid
examination revealed normal cell counts, and the titer of a syphilis test was very low. She
did not have any arrhythmia, ischemic heart disease, or valvular heart disease as an
embolic source. Brain infarction may be developed by pressure of an aortic aneurysm on
the left common carotid artery. They recommend consideration of syphilis as an etiology
of brain infarction when luetic patients are seen in old age.

From the *Cerebrovascular Disease Clinic and Division of Clinical Research National Kyushu Medical
Center Hospital, and the †
Second Department of Internal Medicine, Faculty of Medicine, Kyushu

University, Fukuoka City, Japan.

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Introduction

oped right hemiparesis and severe dysarthria. She
admitted to the hospital on the same day
with the diagnosis of brain infarction. Six days
after the first ictus, February 28 during hospitalization, she suddenly fell into coma. Although her
consciousness gradually recovered in a few days,
right hemiplegia and global aphasia were unchanged. On July 16, she transferred to our hospital for further evaluation of the neurologic disorders and tumor on the right chest.
On physical examination, blood pressure was
144/100 mmHg and pulse rate was 80/minute
and regular on admission. There were no abnormalities in the respiratory and heart sounds.
A round and pulsatile mass was palpated on the
right edge of the sternum just below right clavicle. The surface was smooth and the margin was
sharp. The size of the tumor was 4 x 5 cm in diameter and 1 cm in height (Figure 1). Her consciousness was clear with global aphasia. She
was right hemiplegic and had hypesthesia on the
right. The pupils were round and isocoric, and
was

Recent chemotherapy has extremely reduced the

incidence of syphilis, and we rarely see patients
with neurosyphilis or syphilitic aortic aneurysm.
Neurosyphilis is known to accompany brain infarction due to stenosis or occlusion of intracranial arteries, ie, syphilitic arteritis, but a case with
brain infarction by syphilitic aneurysm is very
rare. We herein reported a case of huge syphilitic
aortic aneurysm that caused brain infarction.
Case Report
In the spring of 1991, a seventy-eight-year-old
woman first noticed a

small, elastic, hard tumor,
that had pulsation without pain on the right
upper chest, but she did not have any medical examinations. During the next three years, the
tumor increased in size, but no pain or erosion of
the skin were observed. On February 22, 1994, at
6 AM, her daughter found that the patient devel-

A huge pulsatile mass protruded from the right upper chest.
The surface was smooth, 4 x 5 cm in diameter, and 1 cm in height.

Figure 1.

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light reflex was prompt. Deep tendon hyperreflex and Babinski’s sign were positive on her
right side.
Laboratory data of peripheral blood cell
counts and serum chemistry demonstrated no abnormalities. Serologic test for syphilis was positive (Rapid Plasma Reagin Card Test [RPR] 128x,
Treponema Pallidum Hemagglutination Test
[TPHA] 40,960x: TPHA IgM negative, IgG 128x
and Fluorescent Treponemal Antibody Absorption
Test [FTA-ABS] positive). The examination of
cerebrospinal fluid (CSF) revealed normal cell
counts and protein content. The CSF test for
syphilis was positive, but the titer was low (RPR
negative, TPHA 4x, TPH IgM negative, IgG 4x,
FTA-ABS positive).
A chest x-ray film demonstrated a marked en-

largement of the upper mediastinum to the right
(Figure 2). The chest computed tomography (CT)
with contrast enhancement revealed a huge saccular aortic aneurysm in the ascending aorta extending to the innominate artery with occluding

thrombus in the lumen (Figure 3). This aneurysm
had grown forward and destroyed the upper sternum and the second and third ribs. Furthermore,
the aneurysm involved the aortic arch and compressed the left common carotid artery (CCA).
The contrast medium was not visible in the left
CCA from its origin. The lumen of the left CCA
was packed by soft-density material the same as
that of the aortic aneurysm. This suggested that
the left CCA was filled with organizing thrombus
and occluded from the origin. A brain CT showed
a widespread low-density area in the territory of
the left middle cerebral artery (Figure 4).
Magnetic resonance angiography (MRA) did not
demonstrate any stenosis of segmental construction or occlusion of any other intracranial arteries
except for the left internal carotid artery (ICA)
(Figure 5). The cervical pulsed Doppler echo
demonstrated that the left CCA was packed with
thrombus and there was no flow in it.
Atherosclerotic change of the left ICA and external carotid artery was not evident.

The chest x-ray film showed the enlargement of the right
mediastinum
and cardiomegaly.
upper

Figure 2.

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The chest computed tomography enhanced with contrast medium showed a huge
saccular aortic aneurysm filled with thrombus. The aneurysm destroyed the sternum and ribs,
compressing the proximal part of the left common carotid artery. The left common carotid artery
was occluded with thrombus, and blood flow was not detected. L CCA, left common carotid artery;
L subclavian A, left subclavian artery.

Figure 3.

Brain computed tomography on admission showed a widespread low-density area in
the left middle cerebral artery territory.

Figure 4.

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Magnetic resonance angiography in axial view demonstrated the occlusion
of the left internal carotid artery. The other intracranial arteries except for left internal
carotid artery did not show segmental occlusion or marked stenosis.

Figure 5.

We speculated that her brain infarction may
have been caused by the left CCA occlusion on
the basis of syphilitic aortitis. We started to administer benzylpenicillin benzathine 2,400,000
units per day orally for thirty days. The titer of
the serum syphilis test did not change after the
treatment. During the hospitalization for ninetyfour days, she did not experience recurrence of
ischemic stroke, and her neurologic status was

Discussion

protruded to the right upper chest. The characteristic of a luetic aneurysm is to expand enough
to reach, compress, and erode the contiguous
sternum and anterior right thoracic cage.’ The
size and structure of the aneurysm of our patient
is compatible with luetic aneurysm. Furthermore, she showed a markedly high titer for the
serum syphilis test. The titer for RPR was 128x,
TPHA was 40,960x and, FTA-ABS was positive
but TPHA IgM was negative. Cardiovascular
complications have been observed in approximately 10% of untreated lues.1 These are uncomplicated aortitis, aortic aneurysm, valvulitis
with aortic regurgitation, and coronary ostial

The patient in this report had a huge aortic
aneurysm on the ascending aorta. The saccular
aneurysm eroded the ribs and the sternum and

stenosis.2 These cardiovascular diseases may
cause cerebrovascular accidents, but brain infarction is very rare as a cardiovascular complications caused by syphilis.

not changed.

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The most common type of syphilitic vasculitis
with brain infarction is neurosyphilis. Less than
10% of patients with seropositive syphilis develop
neurosyphilis.3 Merritt et al4 described the characteristics of neurosyphilis as follows: asymptomatic (31%), meningitis (6%), vascular syphilis
(10%), tabes dorsalis or general paresis (42%),
and miscellaneous (11%). In another study, 8.8%
of 2019 neurosyphilis patients had meningitis,
19% had meningovascular disease and more than
70% had parenchymal brain disease.5

ic infarction. However, she did not have arrhythmia, atrial fibrillation, valvular heart disease, or
ischemic heart disease. So we assume that brain

embolism from heart disease can be ruled out.

Furthermore, the chest CT, cervical pulsed
Doppler echo, and MRA findings did not demonstrate severe atherosclerotic or thrombotic

change

of cervical arteries. Considering these findings,
we assumed that artery-to-artery embolism may
be impossible in this case.

Meningovascular syphilis frequently develops
stroke owing to the occlusion of intracranial
arteries. Two types of syphilitic arteritis are
proposed. Heubner arteritis affects large and
medium-sized arteries and is characterized by
fibroblastic proliferation of the intima, thinning
of the media, and fibrous and inflammatory
changes of the adventitia. The other is Nissl
arteritis, which affects small vessels and is characterized by the proliferation of endothelial and
adventitial cells.6,7 The CT findings in neurosyphilis consist of small infarcts affecting both the gray
and white matter in the multiple arterial distributions.8 In the present case, first, the CSF syphilis
test was positive, but the titer was very low and
the cell counts were within normal limits. Second,
on MRA there were no characteristic neurosyphilitic changes in intracranial arteries, such as
segmental constriction and occlusion.8 Third, the
CT findings were not small infarcts but large and
widespread infarcts in the middle cerebral artery
territory. Thus, we reasonably assume that in our
case, neurosyphilis may not have been a potential
cause of brain infarction.
There have been few reports on a syphilitic
aneurysm developing into brain infarction.
Iemura et al9 reported a case of brain infarction
associated with syphilitic aneurysm, in which they
speculated that atrial fibrillation or ulceration of
aorta may have been possible embolic sources.
On the other hand, in our case, the main mechanism of the brain infarction may have been the
saccular syphilitic aneurysm that markedly enlarged and completely occluded the left CCA. The
chest CT revealed that a huge and saccular aortic
aneurysm compressed the left CCA, and contrast
medium was not visible in its lumen. Thus, blood
flow through the left CCA seemed to be severely
obstructed. The other possible mechanism is embolic infarction. The CT findings in this case were
a widespread low-density area with clear margin.
These findings are rather compatible with embol-

Conclusion
We report a case of brain infarction caused by a
syphilitic aortic aneurysm protruding from the

right upper chest. Brain infarction may have developed by direct aneurysmal compression of the
carotid artery. We should consider vascular
syphilis as the etiology of an ischemic complication when we see the patients with aortic
aneurysm and syphilis.

Acknowledgments
We thank Akira Kawashima, M.D., for his helpful
advice on the radiographic examination.

Hiroshi Nakane, M.D.
Second Department of Internal Medicine
Faculty of Medicine, Kyushu University
Maidashi 3-1-1, Higashi-ku
Fukuoka City, 812 Japan

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