Surgical Neurology 67 (2007) 303 – 307
www.surgicalneurology-online.com

Vascular

Sudden cortical blindness in an adult with moyamoya disease
Dal-Soo Kim, MD, Seok-Gu Kang, MD4, Do-Sung Yoo, MD, Pil-Woo Huh, MD,
Kyoung-Suok Cho, MD, Moon-Chan Kim, MD
Department of Neurosurgery, The Catholic University of Korea College of Medicine, Uijeongbu St. Mary’s Hospital,
Uijeongbu, Gyeonggi 480-130, Republic of Korea
Received 5 September 2005; accepted 20 March 2006

Abstract

Backgound: We report a case of an adult with MMD who presented initially with left visual field
defect that had suddenly progressed to bilateral cortical blindness 1 year and 9 months later.
Case Description: A 33-year-old male presented with visual blurring and mild right hemiparesis that
developed suddenly. He was regarded as having MMD based on an imaging study. An inverted
encephalodurogaleosynangiosis using the left occipital artery and inverted encephaloduroarteriogaleosynangiosis with the parietal branch of the superficial temporal artery were done. The
postoperative course was uneventful and without any complication. Several months later, he visited
again because of sudden bilateral blindness preceded by repeated transient blindness in both eyes for
4 days. The right posterior temporal artery, which was well visualized at the time of the first
postoperative follow-up angiography, was no longer seen on repeated 4-vessel angiography.
Biochemical analysis including prothrombin and thrombin time, activated partial thromboplastin
time, fibrinogen, d-dimer, plasminogen, antithrombin, platelet, protein C and S, lupus anticoagulant,
and cardiolipin antibodies; VDRL was within the reference range.
Conclusion: The authors suggest abnormal thromboembolism as a presumed mechanism of the
pathogenesis of MMD in this patient.
D 2007 Elsevier Inc. All rights reserved.

Keywords:

Adult moyamoya disease; Cortical blindness; Sudden progression; Thromboembolism

1. Introduction

2. Case report

Moyamoya disease is a progressive occlusive cerebrovascular disease characterized by stenosis or occlusion at the
distal internal carotid arteries and the proximal anterior
cerebral and/or the middle cerebral artery and the compensatory development of abnormal moyamoya vessels at the
base of the brain. It has been indicated from previous studies
concerning the progression of MMD that the disease tends to
be progressive and dynamic in childhood, whereas it
becomes stable in adulthood [6,11,17].
However, our case indicates that MMD can be suddenly
progressive even in adulthood, of which the incidence is
considerably low compared with pediatric MMD.

A 33-year-old male with visual blurring and mild right
hemiparesis that developed suddenly on April 20, 2003, was
referred to our hospital. This patient visited other hospitals
with the above-mentioned chief complaints, where brain
MRI taken on April 30, 2003, revealed a small localized
area of bright signal in the white matter of the right frontal
lobe adjacent to the frontal horn of the lateral ventricle and a
large cortical bright signal involving the left occipital lobe in
a FLAIR image and T2-weighted image, which were
suggestive of cerebral infarct (Fig. 1).
Four-vessel angiography demonstrated Suzuki and
Takaku’s angiographic stage III and stage IV in the right
and the left side, respectively, and the posterior circulation
showed severe steno-occlusive changes of bilateral posterior cerebral arteries in their quadrigeminal segments with
no opacification of cortical branches (Fig. 2).
Neurologic examination showed right homonymous
hemianopsia and mild right hemiparesis (G4+/G4 ). Brain

Abbreviations: CT, computed tomography; FLAIR, fluid attenuated
inversion recovery; MMD, moyamoya disease; MRI, magnetic resonance
imaging; SPECT, single photon emission computerized tomography.
4 Corresponding author. Tel.: +82 31 820 3638; fax: +82 31 847 2369.
E-mail address: seokgu9@kornet.net (S.-G. Kang).
0090-3019/$ – see front matter D 2007 Elsevier Inc. All rights reserved.
doi:10.1016/j.surneu.2006.03.043

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Fig. 1. Preoperative axial FLAIR (A) and T2-weighted image (B) of MRI scans showing a small infarct at the right frontal white matter and a cortical large
infarct at the left occipital lobe.

Fig. 2. Four-vessel angiogram showing Suzuki and Takaku’s angiographic stage III on the right side (A and B), stage IV on the left side (C and D), and poor
visualization of distal branches of both posterior cerebral arteries (E and F).

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305

Fig. 3. 99mTc-HMPAO brain SPECT showing a perfusion defect at the left
occipital lobe and a decreased perfusion at the left temporoparietal region.

SPECT using 99mTc-HMPAO revealed perfusion defect in
the left occipital lobe and decreased perfusion in the left
temporoparietal region (Fig. 3).

Fig. 5. Brain perfusion CT (A) and 99mTc-HMPAO brain SPECT (B)
showing newly developed perfusion defect at the right posterior temporooccipital region in addition to preexistent perfusion defect at the left
occipital lobe.

Fig. 4. Follow-up angiogram showing excellent revascularization at the left
occipital and temporoparietal region (A and B) and good patency of the
right posterior temporal artery (C and D).

An inverted encephalodurogaleosynangiosis using the
left occipital artery and inverted encephaloduroarteriogaleosynangiosis with the parietal branch of the superficial
temporal artery were done on May 14, 2003, and June 17,
2003, respectively.
The postoperative course was uneventful and without
any complication. Follow-up angiography, which was done
on February 4, 2004, revealed excellent revascularization on
the left occipital and temporoparietal region and good
patency of the right posterior temporal artery (Fig. 4).
On February 13, 2004, the patient with suddenly
developed bilateral blindness preceded by repeated transient
blindness in both eyes for 4 days was taken by his spouse to
our outpatient clinic. Brain perfusion CT taken in February
13 revealed another perfusion defect involving the right
posterior temporo-occipital area, which was consistent with

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Fig. 6. Repeated 4-vessel angiogram (A and B) showing nonvisualization of
the right posterior temporal artery that was clearly seen on the postoperative
first follow-up angiogram.

the finding using 99mTc-HMPAO brain SPECT (Fig. 5). The
right posterior temporal artery that was well visualized at
the time of the first postoperative follow-up angiography
on February 4, 2004, was no longer seen on the repeated
4-vessel angiography taken on February 16, 2004 (Fig. 6).
Since then, he had been on clopidogrel (75 mg) and
acetylsalicylic acid (200 mg) for the following 4 weeks
under the assumption of MMD-related thromboembolism.
However, biochemical analysis including prothrombin
and thrombin time, activated partial thromboplastin time,
fibrinogen, d-dimer, plasminogen, antithrombin, platelet,
protein C and S, lupus anticoagulant, cardiolipin antibodies,
and VDRL was within the reference range.
3. Discussion
Unlike pediatric MMD, it is very rare to verify clinical
and/or angiographic progression in adult MMD. Furthermore, adult MMD, which presented with cortical blindness
due to visual field defect, is extremely rare. Only 2 cases
were reported in the literature: one case is a 30-year-old
woman who initially presented with a transient visual field
defect that progressed to blindness 1 month later [5], and the
other, a 31-year-old pregnant woman who presented with
sudden blindness on the right side [19].
The authors suggested that an unstable hemodynamic
state or fewer external carotid artery collaterals in the
posterior circulation in the first case and hypercoagulability
due to pregnancy in the second case are the mechanisms that
caused blindness in these moyamoya patients. The clinical
signs of MMD are usually presumed to be caused by the
chronic hemodynamic compromise known to occur in
steno-occlusive cerebrovascular disease.
The most frequently used treatment modalities for MMD
are direct and/or indirect cerebral revascularization procedures, which are known to reverse chronic hemodynamic

insufficiency by improving cerebral blood flow on the
affected vascular territories [8,9,13,14].
However, even with sufficient neovascularization, transient ischemic attack or stroke, mental retardation, and
psychomotor disturbance may develop [8,10]. Therefore,
the clinical progress of MMD under these conditions
underlines the possibilities of other pathologies causing
arterial ischemic stroke.
According to recent articles [1-4,7,12,15,16,18], it is
attempting to speculate that some hemostatic abnormalities
may contribute to the pathogenesis of pediatric MMD.
Bonduel et al [2] described that prothrombotic disorders
were associated with MMD in up to 40% of pediatric
patients [8].
Our case also suggested that the possibility of abnormal
thromboembolism is most likely to be related with blindness
due to sudden nonvisualization of the posterior temporal
artery on the right side, which was very prominent at the
postoperative follow-up angiography taken 12 days before
in spite of laboratory findings within the reference range.

4. Conclusion
Our case was thought to be a very rare adult MMD,
which presented with suddenly progressed blindness due to
infarction of the occipital lobe.
Although most patients with MMD present with cerebral
infarction or hemorrhage of an anterior circulation, a
posterior circulation symptom or visual impairment could
be an initial manifestation of the MMD. Although it is
mainly pediatric MMD patients who progress rapidly
clinically and/or radiologically, adult patients can suddenly
deteriorate even in the posterior circulation.
Furthermore, it is strongly suggested that other mechanisms, such as thromboembolism, should be elucidated in
addition to the hemodynamic compromise to explain the
clinical or radiological progression in MMD.

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