682

J Neurol Neurosurg Psychiatry 2001;70:682–684

SHORT REPORT

Cerebral aneurysms associated with Behçet’s
disease: a case report
S Nakasu, M Kaneko, M Matsuda

Abstract
Cerebral aneurysms in Behçet’s disease
are very rare. The role of vasculitis in the
aetiology of these aneurysms has not been
clarified. A 57 year old man with Behçet’s
disease is described, who had a subarachnoid hemorrhage due to rupture of a
peripheral middle cerebral artery aneurysm. He underwent a successful aneurysmal clipping. Three years later he had
seizures and was found to have a new
aneurysm on the contralateral peripheral

middle cerebral artery as well as some
radiological features of vasculitis. After 3
months of steroid therapy, the aneurysm
disappeared. Although surgical treatment
is the first choice for ruptured aneurysms,
steroid therapy may be eVective for unruptured small aneurysms in patients
with Behçet’s disease.
(J Neurol Neurosurg Psychiatry 2001;70:682–684)
Keywords: Behçet’s disease; cerebral aneurysm; vasculitis

Department of
Neurosurgery, Shiga
University of Medical
Science, Seta, Ohtsu,
Shiga 520–2192, Japan
S Nakasu
M Kaneko
M Matsuda
Correspondence to:
Dr S Nakasu
snakasu@belle.shiga-med.ac.jp
Received 10 August 2000
and in revised form
6 December 2000
Accepted 13 December 2000

Digital subtraction angiography. (A) Left carotid angiography (CAG) on the first admission showing no aneurysm. (B)
Anteroposterior view and (C) lateral view left CAG on the second admission shows a small aneurysm on the peripheral
middle cerebral artery (arrows). The peripheral branch shows stenosis and filling delay. (D) Follow up angiography taken
during the steroid therapy showing disappearance of the aneurysm.

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683

Cerebral aneurysms associated with Behçet’s disease
Table 1

Reported cases of Behçet’s disease associated with cerebral aneurysms

Authors

Age, sex

Location of aneurysm

Katoh et al8
Buge et al9
Kerr1 et al10
Tuji et al11
Hassen-Khodja et al12
Ildan et al13
El Abbadi et al14
Present case

29 M
43 M
12 M
62 F
43 M
28 M
44 M
57 M

MCA
Multiple: ACA, ICA, MCA
Fusiform aneurysms
Multiple: A com, MCA
Multiple, bilateral MCA, ICA, A com
A com
Combined with AVM
A com
Multiple, bilateral MCA
Multiple, bilateral peripheral MCA
Vasculitis

Remarks

MCA=Middle cerebral artery; ICA=internal carotid artery; A com=anterior communicating
artery; ACA=anteior cerebral artery; AVM=arteriovenous malformation.

Cerebral vasculitis is a rare cause of cerebral
aneurysms. Most patients with this combination have an associated systemic lupus erythematosus.1 2 Very few patients reportedly
have isolated CNS angiitis.3 4 Cerebral aneurysms in patients with Behçet’s disease are also
extremely rare. The role of vasculitis in the
aetiology of these aneurysms has not been
clarified. We describe a patient with Behçet’s
disease associated with an unruptured peripheral middle cerebral artery aneurysm that
disappeared after steroid therapy.
Case report
A 57 year old man was admitted to our hospital with a sudden onset of severe headache. He
had a history of Behçet’s disease since he was
38 years old. At that time, he had exhibited
recurrent aphthous stomatitis, skin erythema,
and genital ulcers without eye manifestations.
He had an aneurysm of the right femoral artery
and underwent a replacement of the artery
with an artificial vessel. Since then, he had
experienced several recurrent episodes, but had
been asymptomatic for several years. On
admission, he was stuporous without apparent
paresis. Brain CT showed a dense subarachnoid haemorrhage in the right sylvian fissure
and the insular cistern. Digital subtraction
angiography showed a right peripheral middle
cerebral artery aneurysm. There were no other
aneurysms detected (figure 1 A). The aneurysm was clipped successfully. The parent
artery looked normal at surgery. During the
course of therapy, he transiently exhibited a
genital ulcer, but without other signs of
Behçet’s disease. He underwent a left ventriculoperitoneal shunt for hydrocephalus. He was
discharged with moderate memory disturbance. Three years later, he had a generalised
tonic seizure and was readmitted to our hospital. Brain CT showed a new low density lesion
in the left temporal lobe white matter, which
was thought to represent neuroBehçet’s disease. Electroencephalography detected spikes
in the right frontotemporal region. Digital subtraction angiography showed a small aneurysm
on the left peripheral middle cerebral artery
(figure 1 B, C). Irregular stenotic lesions were
also seen in the bilateral peripheral middle cerebral arteries. Laboratory examination showed
an increase in erythrocyte sedimentation rate.
The concentrations of C-reactive protein, IgA,
and C4 were also increased. An antineutrophic
cytoplasmic antibody and lupus erythematosus
test were negative. He remained stuporous for
days, but gradually recovered. At that time, he

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developed aphthous stomatitis. We decided to
follow the aneurysm conservatively because it
was small and unruptured. He was discharged
under steroid therapy (60 mg prednisolone
daily). Three months after initiation of steroid
therapy, he was readmitted for an angiographic
study. Digital subtraction angiography showed
no aneurysm (figure 1 D), although a mild
irregularity of the vessels was still present. The
dose of the steroid was gradually tapered over 7
months. After completion of the steroid
therapy, follow up digital subtraction angiography showed no aneurysms. However, readministration of the steroid became necessary 3
months later because of recurrent aphthous
stomatitis and an increase of C-reactive
protein. His psychological manifestations
gradually worsened due to progression of neuroBehçet’s disease.
Discussion
Behçet’s disease is characterised by specific
clinical manifestations including recurrent
aphthous stomatitis, genital ulcers, anterior
uveitis, and vasculitis. NeuroBehçet’s disease
occurs, mostly late in the course of the disease,
in about 10% of patients.5 The neurological
manifestations of the disease are attributed
mainly to involvement of the peripheral small
arteries or veins, and association of the disease
with cerebral aneurysms is very rare. Benamour et al6 and Al-Dalaan et al7 found only
one cerebral aneurysm in 316 cases and 119
cases with Behçet’s disease, respectively,
whereas they did not routinely perform cerebral angiographical studies. Although several
authors have described the combination of
Behçet’s disease with cerebral aneurysms,5–7
only eight cases have been reported in detail,
including our case (table 1).8–14 Most of the
aneurysms originated from the common sites
of the ordinary saccular aneurysms without
radiological features of vasculitis. Five of the
eight cases had multiple aneurysms, although
this high frequency may have some reporting
bias. Buge et al reported multiple fusiform
aneurysms.9 One aneurysm originated from a
feeding artery of an arteriovenous malformation.12 Aneurysm formation in vasculoBehçet’s
disease usually occurs in the systemic major
arteries. The histological findings of aneurysms
include ruptured internal and external elastic
laminae with thickening of the tunica media,
and vasculitis of the vasavasorum with perivascular lymphocytic infiltration.5 However, in
cerebral aneurysms in Behçet’s disease, histological examination in two patients showed no
features of vasculitis.8 11 Therefore, it is diYcult
to exclude the possibility of coincidental
association in most of the cases. However, the
high frequency of multiple aneurysms in these
patients may support the likelihood that
Behçet’s disease has some role in the formation
of cerebral aneurysms in some cases. In our
patient, we were not certain at the first admission whether the aetiology of the aneurysm was
related to Behçet’s disease. However, the
second aneurysm was found in association with
the findings of vasculitis and simultaneously
with the appearance of manifestations of the

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Nakasu, Kaneko, Matsuda

disease. These facts support the hypothesis that
Behçet’s disease is related to the aetiology of
this aneurysm. Interestingly, the aneurysm disappeared after steroid therapy. This is the first
report that shows the eVectiveness of steroids
in treating cerebral aneurysms associated with
Behçet’s disease, although spontaneous resolution of the aneurysm cannot be completely
ruled out. In isolated CNS angiitis, Biller et al
also described the possible resolution of one of
multiple aneurysms after immunosuppressive
therapy.3 However, Kerr et al described a case
of a 12 year old boy with Behçet’s disease, who
had developed multiple aneurysms and subarachnoid haemorrhage in rapid succession
even under steroid therapy.10 Also, several
patients with systemic lupus erythematosus
showed aneurysm rupture during the course of
steroid therapy.2 In these patients, prolonged
steroid therapy had been given before the diagnosis of cerebral aneurysms. Therefore, the
disease might have become resistant to the
steroid therapy. In conclusion, cerebral aneurysms may be associated with Behçet’s disease.
Surgical treatment is the first choice if they
have already ruptured. However, steroid
therapy may also be eVective in patients with
unruptured small aneurysms who have not
received steroid therapy previously.

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angiitis and ruptured cerebral aneurysm in patients with
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studies of three cases of cerebral aneurysms associated with
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