Neuro-radiology

Neuroradiology (1993) 35:352-354

9 Springer-Verlag 1993

Spontaneous dissection of the extracranial vertebral artery with
spinal subarachnoid haemorrhage in a patient with Beh et's disease
S. Bahar, O. ~oban, i.H. Giirvit, G. Akman-Demir, A. G6kyi~it
Department of Neurology, Istanbul Medical Faculty, ~apa, Istanbul, Turkey
Received: 15 March 1992/Received in revised form: 20 August 1992

Abstract. A 4 0 - y e a r - o l d m a n with k n o w n d e f i n i t e Beh~et's
d i s e a s e ( B D ) was a d m i t t e d with c o n f u s i o n a l state w h i c h
h a d s t a r t e d 4 days b e f o r e a d m i s s i o n w i t h an a c u t e h e a d ache a n d vomiting. N e u r o l o g i c a l e x a m i n a t i o n r e v e a l e d
confusion, stiff neck, right facial w e a k n e s s , left h e m i paresis, d y s a r t r i a a n d t r u n c a l ataxia. C S F was h a e m o r rhagic a n d x a n t h o c h r o m i c . C r a n i a l C T scans w e r e n e g a tive, b u t M R I s h o w e d a right p o n t i n e h y p e r i n t e n s e lesion
on T 2 - w e i g h t e d images. B i l a t e r a l c a r o t i d a n g i o g r a m s
w e r e n o r m a l . R i g h t v e r t e b r a l a n g i o g r a m s h o w e d findings
c o n s i s t e n t with a d i s s e c t i o n at t h e V2 s e g m e n t of t h e artery. A t t h e level of t h e fifth cervical v e r t e b r a , a r a d i c u l o m e d u l l a r y b r a n c h o f the v e r t e b r a l a r t e r y with an a n e u r y s m a l d i l a t a t i o n in its i n t r a d u r a l p o r t i o n was n o t a b l e .
This case shows that, in B D , a n e u r y s m f o r m a t i o n can also
o c c u r in a spinal a r t e r y a n d s p o n t a n e o u s v e r t e b r a l a r t e r y
d i s s e c t i o n can b e seen.

Key words: S p o n t a n e o u s a r t e r i a l dissections - S u b a r a c h n o i d h a e m o r r h a g e - S p i n a l a n e u r y s m - A r t e r i a l dissect i o n - Beh~et's d i s e a s e

Beh~et's disease, first d e s c r i b e d b y t h e Turkish d e r m a tologist H u l u s i B e h ~ e t in 1937, is a s y s t e m i c d i s e a s e of unk n o w n aetiology. I n v o l v e m e n t o f v a r i o u s o r g a n systems is
d u e to a vasculitis affecting m a i n l y veins, venules, a n d t h e
capillaries. T h e less f r e q u e n t arteritis, w h i c h o f t e n w o r s e n s
the c o u r s e of t h e disease, u s u a l l y affects large a r t e r i e s [1].

Case report
A 40-year-old right-handed man presented with acute, severe head
and backache, vomiting, confusion, diplopia, dysarthria and dysphagia. The head and backache had come on 4 days prior to admission,
diplopia, dysarthria and dysphagia the next day, and subsequently a
confusional state with hallucinations developed. He had a 10-year

Correspondence to: S.Bahar, Department of Neurology, Istanbul
Medical Faculty, 34390 ~apa, Turkey

history of recurrent oral and genital ulcers with occasional erythema
nodosum-like lesions. Seven years previously he had thrombophlebitis in the right leg and an undefined episode of blurred vision.
The same year the skin pathergy test was positive and Behqet's disease was diagnosed. During the next 7 years the disease followed a
silent course except for recurrent oral ulcers.
The patient had abdominal distention and tenderness due to
chronic oesophagitis, gastroduodenitis, and proctitis. He had no active mucocutaneous or ocular signs other than evidence of old glaucoma. He was confused and drowsy with occasional visual hallucinations and had a stiff neck with signs of meningeal irritation. Right
abducens and facial palsies together with dysarthria were noted. He
also had severe truncal ataxia with right cerebellar signs and a slight
left hemiparesis.
The sedimentation rate was 60 mm/h. Other than 20,000 white
blood cells/ram3 his complete blood count, blood chemistry and
coagulation studies were normal. CSF was grossly bloody with
marked xantochromia.
CT was normal. A week after admission, four-vessel angiography
revealed no intracranial abnormality. There was irregular stenosis of
the right vertebral artery (VA), from the fifth to the second cervical
vertebral levels, at the lower end of which was an aneurysmal dilatation of the anterolateral wall. A vertical linear filling defect was seen
between the arterial wall and the aneurysm. An anterior radiculomedullary artery arising from the right VA at C5-C6 had a
3 x 2 mm aneurysm approximately 3 mm before it reached the anterior spinal artery (Fig. i a, b). A month later, the right VA was unchanged, but the radiculomedullary artery at C5-C6 was not seen
(Fig. 1 c).
Cranial MRI on the 25th day showed a hyperintense lesion on
proton density and T2-weighted images on the right of the pons extending to the medulla oblongata (Fig. 2). Coronal Tl-weighted images revealed a narrow, irregular right VA at C2-C5 with a narrowlinear hyperintensity along its lateral wall at C4-C5 (Fig. 3 a). MRI a
year later showed a completely normal right VA (Fig. 3 b).
High-dose methyl-prednisolone therapy was commenced on the
day of admission. The patient showed steady improvement and was
discharged I month later with only slight truncal ataxia.

Discussion
D u r i n g t h e c o u r s e o f Behqet's disease, n e u r o l o g i c a l inv o l v e m e n t is v a r i a b l e . I n a p r o s p e c t i v e s t u d y w e f o u n d
n e u r o l o g i c a l i n v o l v e m e n t in 5.3 % of cases [2].
A r t e r i e s a r e a f f e c t e d m u c h less f r e q u e n t l y t h a n veins:
1-6 % , as c o m p a r e d to a b o u t 70 % [3-5]. T h e a r t e r i e s m o s t

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Fig.la, b. Right vertebral angiogram:
a anteroposterior and
b lateral views. Irregular stenosis of the vertebral artery (VA) between C2 and C5 (open
arrows). Small dissecting aneurysm at the
proximal end of the stenosis (curved arrow).
The anterior radiculomedullary artery
(arrowhead) has a small aneurysm (arrow)
on its intraspinal segment just before it
reaches the anterior spinal artery (small arrows), e Angiogram I week later. The anterior radiculomedullary artery does not fill
Fig.2. MRI (2000/38) shows increased signal,
due to infarction, in the right side on the
lower brain stem
Fig.3. a Coronal Tl-weighted MRI shows
segmental narrowing from C2 to C5. There is
high signal along the lateral wall (arrows) of
the right VA
b Normal flow in the right VA (arrows)
1 year later

commonly affected are the aorta and peripheral arteries.
Arterial lesions usually consist of an aneurysm, pseudoaneurysm or obstruction associated with an aneurysm.
Arteritis of vasa vasorum or arteritis of the arterial wall itself are thought to be responsible [6]. T h e r e is one report
of arteritic occlusion of the branches of the middle cerebral artery [7].
Our case fulfilled the diagnostic criteria of the International Study Group for Beh~et's disease [8], and
presented with two frequent distinct constellations of
neurological findings, signs of meningeal irritation and
brain stem involvement. What makes it unique is the
presence of two very uncommon vascular lesions, which
we believe caused the clinical picture: meningeal irritation was due to subarachnoid haemorrhage (SAH) from
a spinal artery aneurysm, and brain stem signs were
caused by VA dissection.
S A H is extremely rare in Beh~et's disease. We found
only 1 case of spinal SAH, a patient who developed spinal
block signs [9]. In our case four-vessel angiography revealed aneurysmal dilatation of a radiculomedullary
branch of the right VA. Although on the anteroposterior
views it appeared fusiform, the possibility of a saccular an-

eurysm could not be excluded. Matsumoto et al. [6] reported four pathologically verified saccular aneurysms
due to vasculitis in Beh~et's disease. In either case, we considered this aneurysmal dilatation to be the cause of the
SAH, and that the aneurysm developed because of
Beh~et's disease vasculitis, although there is no pathological verification.
Isolated spinal artery aneurysms are rare [10-14]; a review of the literature in 1981 revealed six cases in all [15].
In the last 10 years, eight more have been reported, including a posterior spinal artery aneurysm due to vasculitis in a patient with systemic lupus erythematosus, one
associated with fibromuscular dysplasia (FMD) and two
in patients with coarctation of the aorta, one associated
with pseudoxanthoma elasticum [16-18].
In our case brain stem signs developed on the day following the SAH. The angiographic findings, i.e., the irregular narrowing at the Vz segment of the right VA,
together with the aneurysmal dilatation at the beginning
of this segment, explain these signs. The radiological findings are compatible with arterial dissection [19]. Neither
spinal artery aneurysm, nor this type of arterial involvement has been reported in Beh~et's disease.

354
S p o n t a n e o u s dissection of the V A is less frequent than
that of the carotid artery. T h e cause of s p o n t a n e o u s dissection is u n k n o w n , but predisposing factors such as F M D ,
cystic medial necrosis, accumulation of m u c o p o l y s a c carides and reticular fibre deficiency are possibilities. It is
u n c o m m o n l y associated with polyarteritis nodosa, syphilis, and migraine [20]. In a series of 15 cases (20 vessels)
of extracranial V A dissections, Chiras et al. [21] r e p o r t e d
F M D and hypertension in 30 % and 50 % respectively
[21]. We f o u n d no r e p o r t of s p o n t a n e o u s V A dissection in
Beh~et's disease.
In our case the dissection of the V A involved the portion f r o m which the aneurysmal radiculomedullary artery
arose and b e c a m e s y m p t o m a t i c right after the S A H . We
find it difficult to regard the occurrence of these two vascular lesions as pure coincidence, and speculate that both
were manifestations of vasculitis. W h e n patients with systemic diseases k n o w n to cause vasculitis, like Behqet's disease, present with brain stem signs, the possibility of arterial dissection should be considered before the diagnosis
of direct p a r e n c h y m a l involvement.

Acknowledgements. We wish to thank Dr. Meral Konige and Dr.
Hasan Yazacl for their comments and Dr. Gttlay Gtirvit of Roche
Pharmaceuticals for her assistance in the preparation of this report.

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