Clinical Neurology and Neurosurgery 101 (1999) 62 – 67

Case report

Vertebrobasilar artery territory infarction as an initial manifestation of
systemic lupus erythematosus
Sun Uck Kwon, Jae Young Koh, Jong Sung Kim *
Department of Neurology, Uni6ersity of Ulsan, Asan Medical Center, Song-pa, P.O. Box 145, Seoul 138 -600, South Korea
Received 28 October 1998; received in revised form 11 January 1999; accepted 11 January 1999

Abstract
Cerebral infarction is a well-documented complication of systemic lupus erythematosus (SLE), that usually occurs several years
after the diagnosis of SLE. To our knowledge, however, strokes associated with vertebrobasilar artery involvement were not
reported to present as an initial manifestation of SLE. We report two patients, who presented with vertebrobasilar territory
infarction as an initial manifestation of SLE. Patient 1 was a 16-year-old girl, who developed dysarthria and ataxia. MRI showed
multiple infarcts in the pons, cerebellum and thalamus. Four-vessel cerebral angiography showed multifocal stenoses in the
vertebral and basilar arteries with beaded appearance. Patient 2 was a 26-year-old woman, who developed headache associated
with dysarthria, dizziness and ataxia. MRI showed multiple infarcts in the cerebellum, medulla, pons, midbrain and thalamus.
Cerebral angiography revealed occlusion of both vertebral arteries at the first cervical vertebral level with non-visualization of the
basilar artery. Both patients were diagnosed as having SLE supported by laboratory results. Although rare, posterior circulation
stroke can present as an initial manifestation of SLE, which may be attributed to vasculitis or dissection in the vertebral/basilar
artery. © 1999 Elsevier Science B.V. All rights reserved.
Keywords: Systemic lupus erythematosus; Cerebrovascular disease; Vertebral artery; Basilar artery; Vasculitis; Dissection

1. Introduction

2. Case reports

Stroke is a well known complication of systemic
lupus erythematosus (SLE) occurring in 3 – 20% of the
patients, usually several years after the initial diagnosis
of SLE [1–6]. It most often develops secondary to
cardiogenic embolism or antibody-associated hypercoagulable state [1]. Large cerebral vessel disease is uncommon in SLE and rarely presents as an initial
manifestation [7]. To the best of our knowledge, vertebrobasilar artery stenosis/occlusion has not been reported to present as an initial manifestation of SLE, which
prompted us to report the following two patients.

2.1. Patient 1

* Corresponding author. Tel.: + 82-2-224-3442; fax: + 82-2-4744691.

A 16-year-old previously healthy girl, experienced
several episodes of dizziness and dull headache. Three
months later, she suddenly developed dysarthria and
ataxia and became drowsy in a few days. She was a
high-school student and had no history of alcohol or
tobacco use. Recent history of immunization, infection
and exposure to toxins, heavy metals, illicit drugs or
oral contraceptives was denied. Family history of
stroke, thrombosis and miscarriages was also denied.
She had not experienced arthralgia, but her family
stated that the patient’s skin often turned reddish after
sunlight exposure during the past several years.

0303-8467/99/$ - see front matter © 1999 Elsevier Science B.V. All rights reserved.
PII: S 0 3 0 3 - 8 4 6 7 ( 9 9 ) 0 0 0 0 9 - 8

S.U. Kwon et al. / Clinical Neurology and Neurosurgery 101 (1999) 62–67

On admission, her temperature was 36.5°C, pulse
100, rate of respiration 20 and blood pressure was
170/110 mmHg. An examination, the heart, lungs, abdomen and the extremities were normal, but malar
rashes were noted on both cheeks. On neurological
examination, the patient was drowsy and severely
dysarthric. On forward gaze, horizontal right-beating
nystagmus was observed. She exhibited severe truncal
ataxia and action tremor in both arms with ataxia.
Laboratory tests showed leukocytosis (WBC, 11 300/
mm3), but the hemoglobin and platelet count were
within normal limits. Serum protein, albumin, crea-

63

tinine, glucose, cholesterol and triglyceride levels were
within normal limits. Total amount of protein in 24-h
urine was 0.56 g. Fluorescent antinuclear antibody
(FANA) test was positive at 1:40 dilution with homogenous pattern. Anti-ds-DNA antibody was 21.7 Iu/ml
(normal range: B 7.0 Iu/ml) and direct Coombs’ test
was positive. Lupus anticoagulant was also positive.
The cerebrospinal fluid examination showed normal
findings. Chest X-ray, EKG and echocardiography revealed no abnormalities. Brain MRI showed multiple
small high signal lesions in the left lateral pons, bilateral superior cerebellar peduncles, cerebellar hemi-

Fig. 1. Brain MRI of patient 1. There are multiple small high-signal intensity lesions in the pons, bilateral cerebellar peduncles, cerebellar
hemisphere (A), midbrain (B) and right medial thalamus (C) on T2-weighted image.

64

S.U. Kwon et al. / Clinical Neurology and Neurosurgery 101 (1999) 62–67

Fig. 2. Cerebral angiogram of patient 1. There was alternating segments of narrowing and ectasia involving the left vertebral, basilar artery and
right posterior cerebral artery (A). In addition, the left ICA angiogram shows diffuse narrowing of M1 segment of middle cerebral artery (B,
arrow).

sphere, midbrain and the right paramedian thalamus on
T2-weighted image (Fig. 1). Four-vessel cerebral angiography showed diffuse narrowing of the proximal
portion of the left middle cerebral artery (Fig. 2A) and
multifocal, irregular stenoses/narrowings of the left distal vertebral, basilar and the proximal portion of the
right posterior cerebral artery with occasional beaded
appearance (Fig. 2B).
The patient’s blood pressure was normalized without
any treatment. Oral anticoagulation and monthly administration of intravenous cyclophosphamide were
tried and she gradually improved. Six months later she
was able to walk by herself, although she remained
ataxic and dysarthric. She did not experience a recurrent stroke until 3 years later when a follow-up brain
MRI was taken that revealed no additional lesions.

2.2. Patient 2
A 26-year-old previously healthy woman suddenly
developed occipital headache followed by dizziness and
slurred speech. The symptoms had progressed until
several days later, when she was admitted to our hospital. She was married and had one child. She had not
experienced arthralgia, skin lesion, or spontaneous
abortion, previously. She was not a cigarette smoker
and rarely drank alcohol. There was no history of illicit
drug use, oral pill ingestion, head trauma or recent
immunization. Family history of stroke, thrombosis
and miscarriage was denied.
On admission, her temperature was 36.8°C, pulse
104, rate of respiration 18 and blood pressure was

200/120 mmHg. Physical examination of the heart,
lungs, abdomen and the extremities was normal. There
was no skin rash or lymphadenopathy. On neurological
examination, she was drowsy, dysarthric and dysphagic. There was a horizontal, right beating nystagmus, mild left facial weakness and left hemiparesis
(IV/V). The sensation was preserved in all limbs. The
deep tendon reflexes were normal, but Babinski sign
was present on both sides.
Laboratory tests revealed anemia (hemoglobin, 7.8
g/dl) and mild leukocytosis (9000/mm3). The platelet
count was 322 000/mm3 and the ESR was 80 mm/h.
Serum creatinine, glucose, cholesterol and triglyceride
levels were within normal limits. Total amount of
protein in 24-h urine was 0.94 g. Anti-ds DNA antibody was 7.6 Iu/ml (normal range: B 7.0 Iu/ml). Fluorescent antinuclear antibody (FANA) test was positive
at 1:640 dilution with homogenous pattern and direct
Coombs’ test was also positive. Lupus anticoagulant,
anticardiolipin antibodies and serum protein C and S
levels were all within normal limits. Cerebrospinal fluid
examination was normal. Chest X-ray, EKG and
echocardiography revealed no abnormalities. Brain
MRI showed multiple high signal lesions in the left
lateral medulla, bilateral cerebellum, pons, midbrain
and the right thalamus on T2 weighted image (Fig. 3).
Four-vessel cerebral angiography revealed occlusion of
both vertebral arteries at the first cervical vertebral level
with nonvisualization of the basilar artery (Fig. 4). The
remaining extra- and intracranial vessels were normal.
She gradually improved on treatment with antihypertensives, warfarin and prednisolone. At 9 months of

S.U. Kwon et al. / Clinical Neurology and Neurosurgery 101 (1999) 62–67

follow-up, she was able to stand and walk with
assistance.

3. Discussion
Our two patients initially presented with acute infarction in the cerebellum and the brain stem due to
vertebral/basilar artery disease. The possible causes of
arterial steno-occlusion in young adults include embolism, dissection, premature atherosclerosis, coagulopathy and other angiopathies. We think the possibility
of atherosclerosis and cardiogenic embolism was rea-

65

sonably excluded in our cases by the patients’ age and
echocardiographic findings. The angiographic findings
were not compatible with moya-moya disease, Takayasu’s arteritis, fibromuscular dysplasia, mitochondrial
diseases and strokes associated with hypercoagulable
state. Sneddon syndrome and isolated angiitis of the
central nervous system (CNS) were excluded, respectively, by the absence of livedo reticulitis and the following pieces of evidence supporting the diagnosis of
SLE: malar rash, photosensitivity, proteinuria and positive results for anti-ds-DNA antibody and lupus anticoagulant in patient 1 and the presence of anemia with
positive direct Coomb’s test, proteinuria, positive anti-

Fig. 3. Brain MRI of patient 2. There are multiple high-signal intensity lesions in the left medulla, bilateral cerebellar hemisphere (A), right
midbrain (B) and right thalamus (C) on T2-weighted image.

66

S.U. Kwon et al. / Clinical Neurology and Neurosurgery 101 (1999) 62–67

Fig. 4. Cerebral angiogram of patient 2. The left (A) and right (B) vertebral arteries were occluded (arrow) at the first cervical vertebra level. There
was luminal irregularity (curved arrow) just proximal to the occluded right vertebral artery (B).

ds-DNA antibody and FANA in patient 2. Thus, both
our patients satisfied the criteria for SLE by Tan et al.
[8] and developed stroke in the active state of SLE.
Although cerebral infarction often complicates SLE,
posterior circulation stroke associated with vertebrobasilar artery stenosis/occlusion has not been described as an initial manifestation of SLE.
Large cerebral vessel disease is an uncommon manifestation of SLE. Mitsias and Levine [7] previously
reviewed 30 patients (including 24 cases from literature) with SLE associated with symptomatic large
cerebral arterial occlusive disease and stated that the
pathogenic mechanisms may include coagulopathy,
cardiogenic embolism, large cerebral vessel vasculitis/
occlusive vasculopathy, cervical arterial dissection and
premature atherosclerosis. In our patients, histopathological information was not available and actual vascular pathology remains unknown. However, the
angiographic findings of patient 1 — multifocal
stenoses with beaded appearance — were most consistent with vasculitis. In spite of a few reports about
vasculitis of large cerebral artery in SLE [9 – 11], vasculitis in a strict sense was considered a rare cause of
stroke [1,2]. This rarity may be explained by the fact
that inflammatory vasculopathy in this condition predominantly affects the small vessels of the kidney,
skin, heart, lungs, gastrointestinal tract and the central
nervous system [12].
In patient 2, there was an occlusion of both vertebral arteries. There was no atherosclerotic changes or

evidence of vasculitis in other vessels. Bilateral occlusion of the vertebral arteries could be caused by simultaneous embolic migration from unknown source.
However, the luminal irregularity and stenosis just
proximal to the occluded right vertebral artery appear
to be more consistent with arterial dissection than
embolic occlusion. Furthermore, the initial clinical presentation of this patient was a progressive one—an
unlikely feature for the embolic events. These features,
along with the presence of preceding occipital
headache, suggest that arterial dissection may have
produced the vertebral artery occlusion in this patient.
Unfortunately, we failed to find features suggestive of
dissection on axial MRI images. Vertebral arterial dissection in patients with SLE have been reported [7,13],
but the mechanism for the dissection remains unclear.
Considering the pathological findings of the previously
reported dissecting aortic aneurysm in SLE patients—
active arteritis or obliterative endarteritis of vasa vasorum resulting in multiple infarcts and disruption of the
vessel wall [14,15]—an underlying arteritis or arteriopathy may predispose the vessels to be easily dissected.
In summary, our patients demonstrated that posterior circulation stroke can occur as an initial manifestation of SLE, which may be attributed to vasculitis or
dissection of the vertebral artery and/or basilar artery.
The possibility of SLE should therefore be raised and
appropriate laboratory tests be performed when young
women presenting with posterior circulation stroke are
encountered.

S.U. Kwon et al. / Clinical Neurology and Neurosurgery 101 (1999) 62–67

References
[1] Futrell N, Millikan C. Frequency, etiology and prevention of
stroke in patients with systemic lupus erythematosus. Stroke
1989;20:583 – 91.
[2] Devinsky O, Petito CK, Alonso DR. Clinical and neuropathological findings in systemic lupus erythematosus: The role of
vasculitis, heart emboli and thrombotic thrombocytopenic purpura. Ann Neurol 1988;23:380–4.
[3] Kaell AT, Shetty M, Lee BCP, Lockshin MD. The diversity of
neurologic events in systemic lupus erythematosus: Prospective
clinical and computed tomographic classification of 82 events in
71 patients. Arch Neurol 1986;43:273–6.
[4] Sibley JT, Olszynski WP, Decoteau WE, Sundaram MB. The
incidence and prognosis of central nervous system diseases in
systemic lupus erythematosus. J Rheumatol 1992;19:47–52.
[5] Futrell N, Schultz LR, Millikan C. Central nervous system
disease in patients with systemic lupus erythematosus. Neurology
1992;42:1649 – 57.
[6] Gonzalez-Grespo MR, Blanco FJ, Ramos A, Ciruelo E, Mateo
I, Lopez Pino MA, Gomez-Reino JJ. Magentic resonance imaging of the brain in systemic lupus erythematosus. Br J Rheumatol 1995;34:1055 – 60.
[7] Mitsias P, Levine SR. Large cerebral vessel occlusive disease in
systemic lupus erythematosus. Neurology 1994;44:385–93.

.

67

[8] Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield
NF, Schaller JG, Talal N, Winchester RJ. The 1982 revised
criteria for the classification of systemic lupus erythematosus.
Arthritis Rheum 1982;25:1271 – 7.
[9] Suzuki Y, Kitagawa Y, Matusoka Y, Fukuda J, Mizushima Y.
Severe cerebral and systemic necrotizing vasculitis developing
during pregnancy in a case of systemic lupus erythematosus. J
Rheumatol 1990;17:1408 – 11.
[10] Weiner DK, Allen NB. Large vessel vasculitis of the central
nervous system in systemic lupus erythematosus: Report and
review of literature. J Rheumatol 1991;18:748 – 51.
[11] Liem MD, Gzehs DJ, Flanders AE. MRI and angiographic
diagnosis of lupus cerebral vasculitis. Neuroradiology
1996;38:134 – 6.
[12] Belmont HM, Abramson SB, Lie JT. Pathology and pathogenesis of vascular injury in systemic lupus erythematosus. Arthritis
Rheum 1996;39:9 – 22.
[13] Youl BD, Coutellier A, Dubois B, Leger JM, Bousser MG.
Three cases of spontaneous extracranial vertebral artery dissection. Stroke 1990;21:618 – 25.
[14] Guard RW, Gotis-Graham I, Edmonds JP, Thomas AC. Aortitis with dissection complicating systemic lupus erythematosus.
Pathology 1995;27:224 – 8.
[15] Sclair M, Nassar H, Bar-Ziv Y, Putterman C. Dissecting aortic
aneurysm in systemic lupus erythematosus. Lupus 1995;4:71–4.