J. Obstet. Gynaecol. Res. Vol. 35, No. 5: 974–977, October 2009

doi:10.1111/j.1447-0756.2009.01031.x

Moyamoya-disease-related ischemic stroke in the
postpartum period
Kei Miyakoshi1,3, Misugi Matsuoka1, Daisuke Yasutomi2, Mamoru Tanaka3,
Kazumi Yakubo1, Tatsuro Fukuiya1 and Yasunori Yoshimura3
Departments of 1Obstetrics and Gynecology and 2Internal Medicine, Saitama City Hospital, Midori-ku, Saitama City, Saitama,
and 3Department of Obstetrics and Gynecology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan

Abstract
Stroke during pregnancy or the puerperium is an extremely rare yet serious cause of perinatal morbidity and
mortality. Moyamoya disease, a cerebrovascular occlusive pathology with a female predominance, may become
symptomatic for the first time in association with pregnancy. A 36-year-old woman with postpartum preeclampsia suddenly developed hemiparesis in the left arm with dysarthria after the initiation of antihypertensive measures. Cranial magnetic resonance imaging and angiography revealed acute ischemic lesions in the
right hemisphere along with the steno-occlusive lesions of bilateral terminal portions of the internal carotid
artery, indicating Moyamoya disease. With anti-platelet medication, the patient recovered gradually and was
diagnosed as having Moyamoya disease using conventional angiography 3 months postpartum. In this case,
the fluctuations in blood pressure in association with pre-eclampsia appear to have exacerbated the clinical
symptoms of Moyamoya disease. As such, Moyamoya disease should be considered as an underlying disease
of ischemic stroke associated with pregnancy.
Key words: ischemic stroke, Moyamoya disease, pre-eclampsia, pregnancy.
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Introduction

Case Report

Moyamoya disease is a rare cerebrovascular occlusive
disease with a female predominance. Because of fluctuations in blood pressure associated with pregnancy,
Moyamoya disease may become symptomatic for the
first time during pregnancy and the puerperium.1
Intracranial hemorrhage of sudden onset predominates in patients diagnosed with Moyamoya disease
for the first time in association with pregnancy. To the
best of our knowledge, there are few case reports
regarding the diagnosis of Moyamoya disease following an ischemic attack during pregnancy and the
puerperium.2–4 We herein present a case of postpartum
ischemic stroke in a pre-eclamptic woman, who was
subsequently diagnosed as having Moyamoya disease.

A 36-year-old second parous Japanese woman presented at 31 weeks’ gestation with complaints of
decreased fetal movement, epigastric pain with nausea
and edema for 2 days. Mild elevated blood pressures of
140–150/70–80 mmHg were apparent after attending
our prenatal clinic. Upon arrival, her blood pressure
was 180–190/100–110 mmHg with proteinuria of
100 mg/dl and the ultrasound evaluation revealed an
appropriate-for-date fetus without movement. Because
of the non-reassuring fetal heart rate pattern and severe
pre-eclampsia, emergency cesarean was performed
and placental abruption was found. A depressed
female baby weighing 1436 g was resuscitated and
treated at the neonatal intensive care unit. The woman’s

Received: April 16 2008.
Accepted: December 19 2008.
Reprint request to: Dr Kei Miyakoshi, Department of Obstetrics and Gynecology, Keio University School of Medicine,
35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. Email: kei@sc.itc.keio.ac.jp

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© 2009 The Authors
Journal compilation © 2009 Japan Society of Obstetrics and Gynecology

Postpartum stroke from Moyamoya disease

Anti-platelet medication
Antihypertensive
Magnesium
sulfate

Magnesium
sulfate

Blood Pressure

(mmHg)
200
160
120
80

1

2

3

4

5

Postpartum Day
Figure 1 Changes in blood pressure during the postpartum period. On postpartum day 3, blood pressure was
controlled, however, hemiparesis in the left arm with
dysarthria developed.

medical history was unremarkable and her previous
pregnancy was uncomplicated except for cesarean
delivery because of cephalopelvic disproportion.
Postpartum, the patient received 48 h of intravenous
magnesium sulfate as seizure prophylaxis (Fig. 1). The
immediate postoperative course was uneventful with
blood pressures of 150–160/70–80 mmHg. On postpartum day 2, her blood pressure again elevated to 170–
180/90–100 mmHg and antihypertensive treatment
using long-acting nifedipine was initiated. The following day, her blood pressure was controlled, ranging
from 120–140/60–80 mmHg. However, hemiparesis in
the left arm with dysarthria suddenly developed and
magnesium sulfate was restarted for possible seizure.
Intracranial hemorrhage was not found by immediate
computed tomography. Cranial magnetic resonance
imaging (MRI) illustrated acute ischemic lesions in
the right hemisphere (Fig. 2). Cerebral edema suggesting posterior reversible encephalopathy syndrome
was not demonstrated on MRI. Additionally, magnetic
resonance angiography (MRA) revealed the stenoocclusive lesions of bilateral terminal portions of the
internal carotid artery, indicating Moyamoya disease
(Fig. 3a). Magnesium sulfate and an antihypertensive
were discontinued, and anti-platelet medication with
aspirin was given. On postpartum day 4, her blood
pressure showed a transient elevation to 170–180/90–
100 mmHg but she manifested no additional neurological symptoms. Thereafter, her blood pressure

Figure 2 Brain axial diffusion-weighted magnetic resonance image showing acute ischemic lesions in the
right hemisphere (arrowheads). R, right; L, left.

lowered gradually and remained mildly elevated but
stable at 140–150/80–90 mmHg without antihypertensive measures (Fig. 1). She recovered gradually and the
neurological status was normal 5 days after the onset
of symptoms. Three months postpartum, Moyamoya
disease was confirmed by conventional angiography
(Fig. 3b,c).

Discussion
Moyamoya disease is characterized by stenosis or
occlusion of the terminal portion of internal carotid
arteries, along with abnormal capillary networks,
known as Moyamoya vessels.5 Although a variety of
genetic, environmental, and infectious causes have

© 2009 The Authors
Journal compilation © 2009 Japan Society of Obstetrics and Gynecology

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K. Miyakoshi et al.

Figure 3 Radiological findings of steno-occlusive lesions
of the terminal portion of bilateral internal carotid
arteries and Moyamoya vessels. (a) Antero-posterior
view of magnetic resonance angiogram. Frontal angiogram of right common (b) and left internal (c) carotid
artery. Note that the distal internal carotid artery is
occluded and the middle cerebral artery is not visualized in the right hemisphere. The left distal internal
carotid artery is seen as stenotic. Abnormal capillary
networks known as Moyamoya vessels are clearly
demonstrated by conventional angiography (arrowheads). R, right; L, left.

been postulated, the etiology of Moyamoya disease
remains unclear.4,5 The diagnosis of Moyamoya disease
is made based on angiographic features from MRI and
MRA as well as conventional angiography.6 Its occurrence has been reported worldwide, although it was
originally reported in Japan. Among the Japanese, it
depicts a bimodal age distribution with a higher peak
in 10–14 year olds and a lower peak in 30–40 year olds.7

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Common manifestations in Japanese patients are transient ischemic attacks in children and cerebral hemorrhage in adults. Of interest, epidemiological studies
from the USA have shown that ischemic attack is
the predominant initial symptom in both adults and
children, indicating racial differences in its clinical
presentation.8
As the diagnosis of Moyamoya disease is based on
its angiographic features, case detection may hinge on
the performance of angiography. In this regard, the
use of conventional angiography in the evaluation
of ischemic stroke varies widely between individual
health care providers. In our case, MRI revealed acute
ischemic lesions without cerebral edema in the right
hemisphere. The patient had no history of a cardioembolic source (i.e. cardiac structural abnormalities or
arrhythmias). Although pre-eclampsia may be associated with the development of cerebral ischemia, the
MRI results warranted further evaluation of underlying cerebrovascular disease, and then the use of MRA
revealed vascular lesions indicative of Moyamoya
disease.
Our patient showed ischemic symptoms when her
blood pressure was lowest after the initiation of antihypertensive measures. Hemodynamic change during
the early puerperium may place undue stress on the
cerebral vessels.1 Komiyama et al. reported that the
development of pre-eclampsia may cause deterioration
of clinical symptoms of Moyamoya disease.1 Preeclampsia, a pregnancy-specific multi-organ disorder,
is characterized by endothelial dysfunction.9 The
development of neurological disorders in preeclampsia is thought to be related to vasoconstriction associated with endothelial damage, as well as
impaired autoregulation of the cerebral vessels.9 Therefore, fluctuation of blood pressure and cerebral
pathologies associated with pre-eclampsia could make
patients with Moyamoya disease become symptomatic
of this disease. In contrast to the previous pregnancy,
our patient developed severe pre-eclampsia in the
index pregnancy. Ischemic lesions were found in the
right hemisphere which showed marked vascular
changes associated with Moyamoya disease. The combination of acute reduction in blood pressure with an
antihypertensive, pre-eclampsia, and underlying
Moyamoya disease appeared to be associated with
ischemic stroke in this case.
Stroke during pregnancy or the puerperium is an
extremely rare yet serious cause of perinatal morbidity
and mortality.10 Hypertension and ruptured cerebral
aneurysm are considered as major causes of cerebral

© 2009 The Authors
Journal compilation © 2009 Japan Society of Obstetrics and Gynecology

Postpartum stroke from Moyamoya disease

stroke during pregnancy. Although Moyamoya disease
is a rare cerebrovascular disorder, it should be considered as an underlying disease of ischemic stroke associated with pregnancy.

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© 2009 The Authors
Journal compilation © 2009 Japan Society of Obstetrics and Gynecology

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