Case Report

Management for a patient of moyamoya disease presenting with
ischemic stroke in the first trimester of pregnancy
Masashi Watanabe, MD, Kanehisa Kohno, MD, PhD, Tomoki Shinohara, MD,
Toshimoto Seno, MD, PhD, Satoshi Fujiwara, MD, PhD, Shinji Onoue, MD, PhD,
Shinya Fukumoto, MD, PhD, Haruhisa Ichikawa, MD, PhD, Shinji Iwata, MD, PhD,
Kensho Okamoto, MD, and Shiro Ohue, MD, PhD

We report an extremely rare case of a 27-year-old woman presenting with ischemic
stroke as an initial manifestation of moyamoya disease in the first trimester of pregnancy.
We conducted an artificial abortion when her neurological symptoms rapidly became
refractory to optimal antithrombotic treatments. The progression of neurologic deficits
stopped immediately after abortion, resulting in recovery to independence, with slight
motor aphasia and right hemiparesis due to improved cerebral flow. We highlight rapid
artificial abortion combined with antithrombotic treatment for patients of moyamoya
disease with pregnancy-associated ischemic stroke as an appropriate treatment to correct
hemodynamic instability and suppress the progression of neurological symptoms.
Keywords: Moyamoya disease—Adult-onset—Ischemic stroke—The first
trimester of pregnancy—Artificial abortion
© 2020 Elsevier Inc. All rights reserved.

Introduction
The prevalence of pregnancy-associated stroke is
approximately 0.03%,1,2 of which only 2% are due to
moyamoya disease (MMD).3 The majority of strokes are
hemorrhagic among Japanese women,3 and occur in the
peripartum and early postpartum periods.4,5 Here we
report the management of an extremely rare case presenting with ischemic stroke as an initial manifestation of
MMD in the first trimester of pregnancy.

Case summary
A 27-year-old woman in the seventh week of pregnancy suffered from severe hyperemesis gravidarum
(HG) for nine days. She was hospitalized after
From the Department of Stroke Center, Ehime Prefectural Central
Hospital, 83, Kasugamachi, Matsuyama, Ehime 790-0024, Japan.
Received May 20, 2020; revision received June 8, 2020; accepted
June 14, 2020.
Corresponding author. E-mail: natural.mw.stream@gmail.com.
1052-3057/$ - see front matter
© 2020 Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jstrokecerebrovasdis.2020.105075

developing speech and gait disturbances beginning two
days prior. Neurological examination revealed mild
consciousness disturbance, motor aphasia, right hemiparesis, and right-sided hemispatial neglect. Laboratory
investigations indicated hypovolemia and ketosis with
normal blood coagulation and cerebrospinal fluid.
Brain magnetic resonance imaging on diffusionweighted image showed high intensity lesions in both
watershed territories between the anterior and middle
cerebral arteries (Fig. 1A). Brain magnetic resonance
angiography and arterial spin labeling revealed severe
stenosis and occlusion at the terminal portion of the
bilateral internal carotid artery with cerebral hypoperfusion (Fig. 1B,C), compatible with MMD. We decided
to conduct an artificial abortion on the second day
because her neurological symptoms rapidly became
refractory to optimal antithrombotic treatments. The
progression of neurologic deficits stopped immediately
after abortion, resulting in recovery to independence,
with slight motor aphasia and right hemiparesis due to
improved cerebral flow (Fig. 1D,E). There has been no
recurrence over one year after bilateral revascularization (Fig. 1F).

Journal of Stroke and Cerebrovascular Diseases, Vol. 29, No. 10 (October), 2020: 105075

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M. WATANABE ET AL.

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Fig. 1. Brain magnetic resonance imaging (MRI). (A) Diffusion-weighted images from brain MRI show hyperintense lesions in both watershed territories
between the anterior and middle cerebral arteries with left-sided dominancy. (B, C) Brain magnetic resonance angiography (MRA) reveals severe stenosis and
occlusion at the terminal portion of the bilateral internal carotid artery and the circle of Willis (arrows) presenting with hypoperfusion by arterial spin labeling
(ASL). (D, E) Brain MRA and ASL show improvement of cerebral perfusion after abortion (arrowheads). (F) Brain MRA shows development of transdural anastomoses by bilateral combined revascularization surgery over one year later (arrowheads).

Discussion
Pregnancy is recognized as a risk factor for stroke. It is
not uncommon for MMD to coincide with pregnancy,
since MMD is more common in females, and more prevalent in the second or third decade of life.6 The MMD
patients diagnosed during pregnancy have more hemorrhagic strokes, fewer bypass surgeries, and more unfortunate outcomes for both mothers and babies than MMD
patients diagnosed prior to pregnancy.7 The majority of
patients diagnosed as MMD during pregnancy was at
28.7 § 9.1 weeks of gestational age, with no occurrences
before 19 weeks.7
To the best of our knowledge, there have been no
reported cases of stroke in the first trimester. We did,
however, find two case reports of pregnant woman presenting with chorea in the first trimester before MMD
diagnosis. Unno et al. reported a 16-year-old pregnant
woman who developed chorea without HG, which disappeared after an artificial abortion.8 Kim et al.
reported a 28-year-old pregnant woman whose chorea
gradually and spontaneously improved with the disappearance of HG.9 These cases suggest that changes in
cardiovascular hemodynamics due to pregnancy and

HG exacerbate the risk of ischemic stroke in MMD
patients, even in the first trimester.10 We highlight rapid
artificial abortion combined with antithrombotic treatment for MMD patients with pregnancy-associated
ischemic stroke as an appropriate treatment to correct
hemodynamic instability and suppress the progression
of neurological symptoms.

Grant support
No targeted grant support reported.

Disclosure
The authors report no disclosures relevant to the manuscript.

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