Case Report

The Great Imitator—Still Today! A Case of Meningovascular

Syphilis Affecting the Posterior Circulation

Jochen B€auerle, MD,* Anna Zitzmann, MD,* Karl Egger, MD,† Stephan Meckel, MD,†
Cornelius Weiller, MD,* and Andreas Harloff, MD*

Cerebral ischemia due to meningovascular syphilis is rare and more frequently af-
fects the anterior circulation than the posterior circulation. We describe clinical fea-
tures and imaging studies of a 50-year-old patient with Parinaud syndrome and a
syphilitic dorsal midbrain infarction. Brain magnetic resonance imaging indicated
vasculitis of the posterior circulation. The diagnosis of meningovascular syphilis
was established by serum and cerebrospinal fluid examinations. Although rare,
because of the high impact on treatment, clinicians should always be aware of me-
ningovascular syphilis in the differential diagnosis of stroke, particularly in young
and male patients with cryptogenic stroke. Key Words: Meningovascular
syphilis—vasculitis—MRI—stroke.
Ó 2015 by National Stroke Association

Case Report (Parinaud syndrome). Magnetic resonance (MR) imaging

showed subacute infarctions of the right medial cerebral
A 50-year-old man was referred to our hospital because
and cerebellar peduncle. Contrast-enhanced MR angio-
of acute onset of double vision 3 days before admission.
graphy demonstrated multiple stenoses restricted to the
He had no other symptoms before and negated headache
vertebrobasilar circulation (Fig 1). Color-coded duplex
or dizziness. His medical history included hypertension
sonography confirmed a severe stenosis of the proximal
and nicotine consumption measuring 40 pack years.
basilar artery and small plaques of both internal carotid
Twelve years ago he had suffered from a nephrotic syn-
arteries. Transthoracic echocardiography and Holter elec-
drome due to membranous glomerulonephritis of unclear
trocardiogram were normal. Fasting low-density lipopro-
etiology, which had resolved spontaneously.
tein cholesterol was 140 mg/dL, and fasting glucose was
Neurologic examination revealed an isolated dorsal
98 mg/dL. Routine laboratory tests including erythrocyte
midbrain syndrome with supranuclear upgaze palsy,
sedimentation rate and C-reactive protein, antinuclear
convergence retraction nystagmus, and lid retraction
antibodies, and antineutrophil cytoplasmic antibody
screening were normal. After these tests, large artery
atherosclerosis was considered as stroke etiology.
From the *Department of Neurology, University Hospital Freiburg,
Despite a low level of suspicion, the younger age and
Freiburg; and †Department of Neuroradiology, University Hospital
Freiburg, Freiburg, Germany.
multiple stenoses of the vertebrobasilar arteries led to
Received February 27, 2014; revision received June 1, 2014; accepted the examination of cerebrospinal fluid (CSF) to exclude
July 27, 2014. vasculitis. CSF revealed 192 cells/mL (90% lymphocytes
Address correspondence to Jochen B€auerle, MD, Department of and 10% monocytes), total protein of 1.16 g/L, and lactate
Neurology, University Hospital Freiburg, Breisacher Str 64, 79106
concentration of 2.21 mmol/L. Laboratory tests for
Freiburg, Germany. E-mail: jochen.baeuerle@uniklinik-freiburg.de.
1052-3057/$ - see front matter
bacteria and fungi, herpes simplex, Varicella zoster virus,
Ó 2015 by National Stroke Association tuberculosis, Lyme disease, listeriosis, and human immu-
http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.07.046 nodeficiency virus (HIV) were negative. However,

Journal of Stroke and Cerebrovascular Diseases, Vol. 24, No. 1 (January), 2015: pp e1-e3 e1
e2 €
J. BAUERLE ET AL.

Figure 1. Brain magnetic resonance imaging

(MRI) examinations at 3 T. (A) Diffusion-
weighted MRI revealed a paramedian dorsal
midbrain infarction (yellow arrow). (B) Multiple
consecutive stenoses of the right vertebral and
basilar artery can be appreciated on contrast-
enhanced magnetic resonance angiography
image (yellow arrows) whereas all other brain ar-
teries appear normal. (C and D) High resolution
black-blood imaging T1-FLAIR MRI. Focal
concentric contrast enhancement of the arterial
wall at level of vertebrobasilar junction is shown
(D, yellow arrow) on postcontrast image
compared with precontrast image (C, yellow
arrow). (Color illustration of figure appears on-
line.)

Treponema pallidum particle agglutination assay, vene- duplex sonography indicating chronic alteration of the
real disease research laboratory test, and fluorescent vertebrobasilar arteries.
treponemal anti-body absorption test were highly posi-
tive in both serum and CSF, and we diagnosed meningo-
Discussion
vascular neurosyphilis. Additionally, we performed high
resolution black-blood imaging (pre- and post-contrast T1 Neurosyphilis occurs at any stage of syphilis, and
fluid attenuated inversion recovery (FLAIR)-weighted) the central nervous system may be involved early in
MR imaging at 3 T. Here, only the vertebrobasilar arteries the course of the disease. Meningovascular syphilis
showed concentric vessel wall enhancement indicating seems to be the predominant manifestation of neurosy-
vasculitis (Fig 1).1 Further medical history analysis philis in HIV-uninfected individuals and appears
regarding risk factors for syphilis revealed homosex- approximately 5-12 years after the initial infection
uality. with T pallidum.2-4 Contrarily, in HIV-infected patients
Three days after admission, we initiated intravenous syphilitic meningitis may be more common than me-
treatment with penicillin G (4 million units every ningovascular syphilis.4 Two types of arteritis have
4 hours). Because CSF revealed an elevated cell count been described: endarteritis obliterans of medium
of 35 cells/mL after 14 days of treatment, penicillin G and large arteries (ie, Heubner arteritis) and Nissl–
was given for 21 days. Moreover, we added clopidogrel Alzheimer arteritis affecting small arterial vessels.
75 mg daily to 100 mg acetylsalicylic acid and atorvas- Predominantly, both variants emerge as acute
tatin 40 mg per day because of the high-grade intracra- stroke whereas the anterior circulation is affected
nial stenosis. Regarding the vasculitis, we prescribed more frequently than the posterior circulation.5,6
prednisone 100 mg, which was tapered over 8 weeks. Meningovascular syphilis is a rare etiology of
During hospital stay, ocular symptoms resolved almost ischemic stroke but screening should be performed in
completely. Four months later, CSF examination patients at increased risk for syphilitic infection.7
confirmed resolution of the pleocytosis. Otherwise, ver- Several previous case reports highlight this differential
tebrobasilar stenosis was still unchanged in color-coded diagnosis of stroke.8,9 Interestingly, in the 19th century,
A CASE OF MENINGOVASCULAR SYPHILIS e3

syphilis was a common cause of stroke. In fact, most References

of the eponymous terms associated with stroke,
1. Swartz RH, Bhuta SS, Farb RI, et al. Intracranial arterial
like Parinaud, Weber, Foville, or Millard–Gubler
wall imaging using high-resolution 3-tesla contrast-
syndrome, and so forth arose as a consequence of enhanced MRI. Neurology 2009;72:627-634.
the relatively small strokes affecting the brain stem 2. Conde-Sendın MA, Amela-Peris R, Aladro-Benito Y, et al.
due to small and medium vessel syphilitic vasculitis Current clinical spectrum of neurosyphilis in immuno-
that are seldom seen today, as the etiology of competent patients. Eur Neurol 2004;52:29-35.
3. Burke JM, Schaberg DR. Neurosyphilis in the antibiotic
stroke is predominantly an atherosclerotic disease.5
era. Neurology 1985;35:1368-1371.
Thus, the presence of such syndromes may warrant 4. Katz DA, Berger JR, Duncan RC. Neurosyphilis. A
at least a venereal disease research laboratory test in comparative study of the effects of infection with hu-
addition to the routine stroke workup. man immunodeficiency virus. Arch Neurol 1993;50:
Because of the existing cardiovascular risk factors 243-249.
5. Merrit HH, Adams RD, Solomon HC. Neurosyphillis.
and mild arteriosclerosis of the internal carotid arteries
New York: Oxford University Press 1946.
in our patient, we had initially assumed intracranial 6. Holmes MD, Brant-Zawadzki MM, Simon RP. Clinical
large artery atherosclerosis as etiology of stroke. Sur- features of meningovascular syphilis. Neurology 1984;
prisingly, CSF analysis was abnormal and revealed 34:553-556.
the final diagnosis of neurosyphilis. Retrospectively, 7. Kelley RE, Bell L, Kelley SE, et al. Syphilis detection in ce-
rebrovascular disease. Stroke 1989;20:230-234.
the patient’s prior membranous glomerulonephritis
8. Flint AC, Liberato BB, Anziska Y, et al. Meningovascular
may have been a syphilitic nephropathy because renal syphilis as a cause of basilar artery stenosis. Neurology
involvement is a well-described finding in secondary 2005;64:391-392.
syphilis.10 Although rare and because of the high 9. Feng W, Caplan M, Matheus MG, et al. Meningovascular
impact on treatment, clinicians should always be syphilis with fatal vertebrobasilar occlusion. Am J Med
Sci 2009;338:169-171.
aware of meningovascular syphilis in the differential
10. Hunte W, al-Ghraoui F, Cohen RJ. Secondary syphilis and
diagnosis of stroke, particularly in young and male pa- the nephrotic syndrome. J Am Soc Nephrol 1993;3:
tients with cryptogenic stroke. 1351-1355.