Brain & Development xxx (2016) xxx–xxx
www.elsevier.com/locate/braindev

Case Report

Reversible cerebral vasoconstriction syndrome manifesting as
focal seizures without a thunderclap headache: A pediatric case report
Shuji Kuga a,b,⇑, Hironori Goto a,b, Kazuo Okanari a, Tomoki Maeda a, Kenji Ihara a
a

Department of Pediatrics, Oita University Faculty of Medicine, Yufu-City, Oita, Japan
b
Department of Pediatrics, Nishida Hospital, Saiki-City, Oita, Japan

Received 29 January 2016; received in revised form 30 March 2016; accepted 12 April 2016

Abstract
We report a pediatric case of reversible cerebral vasoconstriction syndrome with focal seizures without a thunderclap headache.
A 7-year-old girl had a mild acute headache with nausea after swimming. She subsequently developed hemi-convulsions followed by
right hemiplegia. Brain magnetic resonance angiography revealed generalized vasoconstriction of the main cerebral peripheral arteries. Her hemiplegia was spontaneously resolved within 6 h. Over the next 24 h she suffered from recurrent and transient headaches,
which recurred on days 3 and 5. Follow-up magnetic resonance angiography on day 3 documented the multifocal narrowing of the
main cerebral arteries, which was observed to have diminished at 12 weeks after her initial presentation. She did not have any headaches or neurological deficits after day 5. This case indicates that reversible cerebral vasoconstriction syndrome should be considered
in children with focal seizures even when they do not present with thunderclap headaches. The timely and appropriate evaluation by
magnetic resonance angiography and imaging is essential for diagnosing reversible cerebral vasoconstriction syndrome.
Ó 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Keywords: Reversible cerebral vasoconstriction syndrome; Focal seizure; Hemiplegia

1. Introduction
Reversible cerebral vasoconstriction syndrome
(RCVS) is a clinical and radiographic syndrome that is
characterized by thunderclap headache due to reversible
cerebral vasoconstriction. In adulthood, the prevalence
of RCVS increases with aging; the mean age of
the RCVS patients in the literature is 42–43 years
(range: 4 months–65 years) [1–4]. The syndrome rarely
affects infants or children [5–11] (Table 1). RCVS is
diagnosed based on the observation of multifocal
⇑ Corresponding author at: Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka, Hsama, Yufu 879-5593,
Japan. Tel.: +81 97 586 5833; fax: +81 97 586 5839.
E-mail address: kugashuji@gmail.com (S. Kuga).

narrowing of the cerebral arteries on cerebrovascular
imaging; this usually diminishes within 12 weeks after
the onset of symptoms. We herein report a pediatric case
of RCVS in a patient with focal seizures without
thunderclap headache, which was probably triggered
by the physical stress of swimming.
2. Case report
A 7-year-old girl suffered an acute mild headache and
nausea after undergoing extensive physical stress from
2-h of swimming. She subsequently experienced a
hemi-convulsion which spontaneously resolved within
5 min. She was transferred to our hospital after experiencing another hemi-convulsion followed by rightsided hemiplegia, which spontaneously resolved within

http://dx.doi.org/10.1016/j.braindev.2016.04.006
0387-7604/Ó 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Please cite this article in press as: Kuga S et al. Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report. Brain Dev (2016), http://dx.doi.org/10.1016/j.braindev.2016.04.006

M, male; F, female; TCH, thunderclap headache; BP, blood pressure; N.D., not described; SAH, subarachnoid hemorrhage; IPH, intraparenchymal hemorrhage; LDS, Loeys–Dietz Syndrome.

NO/YES
NO/YES
NO/N.D.
NO/N.D.
NO/N.D.
NO/YES
NO/N.D.
YES/NO
N.D./N.D.
NO/YES
N.D.
130/92
152/87
Normal
210/140
N.D.
150/100
N.D.
154/101
Normal
Deep diving
Nasal spray
Defecation
Head banging
Hypertension
Eletriptan
Cyclosporine
Swimming Sumatriptan
Subclavian artery dissection
Swimming
13 M [5]
10 M [6]
16 M [6]
10 M [6]
15 M [7]
12 M [8]
7 F [9]
13 M [10]
9 M [11]
7 F (this case)

YES
YES
YES
YES
YES
YES
YES
YES
YES
NO

Vomiting Photophobia
Vomiting
None
Phonophobia
Photophobia Phonophobia
Paralysis of lower extremities
Blurred lower right vision
None
Vomiting/seizure visual disturbance
Vomiting

None
None
None
None
None
None
Aplastic anemia
None
LDS
None

Migraine patient/family
Underlying disease
BP
Clinical symptom
TCH
Triggers
Age sex

Table 1
Clinical and radiological features of children with reversible cerebral vasoconstriction syndrome in the published literature.

NO
NO
NO
NO
NO
YES SAH
YES IPH
NO
YES SAH
NO

S. Kuga et al. / Brain & Development xxx (2016) xxx–xxx

Brain bleeding

2

6 h. On admission, a blood analysis (complete-bloodcount, electrolytes, liver and renal function tests) and
cerebrospinal fluid examination revealed no abnormalities, and immunological analysis in serum were all
negative such as rheumatoid factor, or anti-nuclear,
anti-cardiolipin and anti-neutrophil-cytoplasmic antibodies. Electroencephalography demonstrated low
amplitude of background activity in the left hemisphere
without apparent paroxysmal discharges. Brain magnetic resonance imaging (MRI) revealed several trivial
ischemic lesions, none of those seemed to be responsible
for the patient’s right-sided hemiplegia. The abnormal
findings by DWI were almost correlated to FLAIR
signals and showed a relationship with the clinical symptoms. The constant modality of MRI applied was 1.5T
every time in the serial studies. Brain magnetic resonance angiography (MRA) revealed generalized vasoconstriction of the left cerebral arteries (Fig. 1). She
had recurrent headaches that spontaneously resolved
three times within the 5 days after the first attack. An
MRA study on day 3 revealed multifocal and tapered
narrowing of the main cerebral arteries and abnormal
dilated segments of the peripheral arteries. The generalized vasoconstriction of the left cerebral arteries was not
apparent at this time (Fig. 2A). On day 6, she was neurologically intact and was discharged from our hospital.
An MRA study at 12 weeks after this episode showed no
cerebral vasoconstriction or ischemic changes in the
brain (Fig. 2B), and the abnormal signals in FLAIR
almost disappeared at 12 weeks after the onset. She
was doing well without any neurologic deficits or
seizures.
3. Discussion
The present case highlights two important clinical
issues. First, children with RCVS might present with
focal seizures without a thunderclap headache. Second,
an MRA study is especially important for making a
proper diagnosis of RCVS.
According to the recent review articles [1–4], thunderclap headache occurs in almost all patients with RCVS.
For this reason, it is considered to be a key symptom for
the diagnosis of RCVS. RCVS is usually triggered by
specific conditions or events, including pregnancy,
orgasm, acute stressful or emotional situations, bathing,
physical exertion, or the intake of vasoactive drugs.
Generalized convulsions are less common at the time
of presentation [1–4]; it is estimated that convulsion is
caused by ischemia in the motor cortex due to decreased
blood flow, which is caused by a spasm of the cerebral
arteries. Although the present case did not present with
a thunderclap headache, serial MRI and MRA studies
demonstrated findings characteristic to RCVS. About
30–70% of patients with RCVS initially showed no
abnormal findings by brain imaging soon after the

Please cite this article in press as: Kuga S et al. Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report. Brain Dev (2016), http://dx.doi.org/10.1016/j.braindev.2016.04.006

S. Kuga et al. / Brain & Development xxx (2016) xxx–xxx

3

Fig. 1. Magnetic resonance angiography (MRA) of the brain on day 1 showing left predominantly generalized vasoconstriction of the cerebral
arteries (A). A fluid-attenuated inversion recovery (FLAIR) image of the brain on day 1 reveals a restricted high signal area (arrow) of in the left
parietal cortex and hyper-intense regions (arrow heads) in both of the frontal–occipital lobes (B).

Fig. 2. (A) MRA of the brain on day 3 shows multifocal stenosis in the proximal internal arteries, middle and posterior cerebral arteries (arrows). (B)
Repeat MRA 12 weeks after the onset of symptoms shows the normalization of the multifocal stenosis.

presence of clinical symptoms. Nevertheless, the MRA
examination would be essentially to detect spasms of
the cerebral arteries, which are specific findings to RCVS
[1–4]. Sporadic signals of small ischemic changes and the
white matter of the cerebral cortex are also characteristic, but are not specific, to RCVS. We therefore assume
that substantial numbers of pediatric RCVS might be
under-recognized. We emphasize the importance of
repeated MRA examinations for suspected cases for
the diagnosis of RCVS.
There is no established therapy for RCVS. Most
patients make a full recovery with time; however, rare
cases may develop a progressive clinical course. It is
therefore important to care for the patients for the
first few days after the onset of symptom. Calcium
channel blockers, brief courses of glucocorticoids,
magnesium sulfate, serotonin antagonists, and dantrolene are indicated to relieve the vasoconstriction in the
acute phase [1–4]. An early diagnosis is therefore crucial to the care of patients, as it allows for the administration of disease-specific treatment and minimizes
the risk of complications and sequelae. Avoiding the
risk factors for attacks is also important for the
patients.

The principal differential diagnosis of RCVS without
thunderclap headache includes 5 types of diseases: epilepsy with hemiparesis (also called Todd’s paralysis),
hemiplegic migraine, reversible posterior leukoencephalopathy syndrome (RPLS), moyamoya disease,
and childhood primary angiitis of the central nervous
system (cPACNS). Todd’s paralysis mimics the clinical
symptom of RCVS with hemiplegia after seizure. We
speculate that transient postictal hypoperfusion due to
a reduction in the cerebral blood flow might underlay
the same pathophysiology in both diseases [12]. Todd’s
paralysis follows a transient course within several hours,
whereas RCVS is typically complicated with recurrent
headaches within a few days. Sequential follow-up
MRA is crucial to detect characteristic abnormalities,
such as narrowing of the cerebral arteries and reversibility of abnormal vascular findings. Second, hemiplegic
migraine is caused by spreading depression of cortical
neurons that results in motor aura, such as unilateral
muscle weakness, and is occasionally accompanied by
seizure. The diagnosis of hemiplegic migraine is based
on the clinical symptoms that are defined in the International Classification of Headache Disorders, 3rd edition
[13]. Taking past medical and family history into

Please cite this article in press as: Kuga S et al. Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report. Brain Dev (2016), http://dx.doi.org/10.1016/j.braindev.2016.04.006

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S. Kuga et al. / Brain & Development xxx (2016) xxx–xxx

consideration and performing follow-up MRA are essential to distinguish hemiplegic migraine from RCVS.
Third, brain MRI of RPLS patients typically shows symmetrical white matter edema in the posterior cerebral
hemispheres, suggesting a similar underlying pathophysiological mechanism to RCVS, whereas RPLS is usually
caused by hypertension [1]. Fourth, moyamoya disease is
one of the most important diseases to be distinguished
from pediatric RCVS with hemiparesis. Vascular imaging (e.g., MRA) of moyamoya disease is highly specific.
Nevertheless, all pediatricians should remember that
avoiding hyperventilation or hyper-exercise is extremely
important to prevent further deterioration. Finally, one
of the angiographic differential diagnoses of RCVS is
childhood PACNS (cPACNS) that have many overlap
clinical symptoms, such as headache, seizures, and focal
deficits. The diagnosis of cPACNS is usually straightforward according to the clinical course and laboratory
data. Patients with cPACNS show typically subacute to
chronic disease, insidious and progressive course, with
pleocytosis and protein elevation in the CSF, and irreversible neurovascular abnormalities, whereas patients
with RCVS demonstrate sudden onset, with normal or
near-normal CSF analysis, and reversible neurovascular
abnormalities. From the histopathological point of view,
elective brain biopsy samples might aid in the definitive
diagnosis, although the clinical indications would be reasonably limited [14].
In summary, we presented the case of a pediatric
patient who was diagnosed with RCVS. At the onset
of symptoms, the patient experienced generalized focal
seizures without a thunderclap headache. The incidence
of RCVS may be under-recognized in children who only
present with generalized focal seizures with transient
palsy. MRA and MRI studies that focus on the signs
of this syndrome are indispensable for its diagnosis,
and early intervention and prophylactic management
are important for the long-term prognosis of pediatric
patients with RCVS.

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Please cite this article in press as: Kuga S et al. Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report. Brain Dev (2016), http://dx.doi.org/10.1016/j.braindev.2016.04.006