European Journal of

Eur J Pediatr (1984)142 : 44-50

Pediatrics
9 Springer-Verlag 1984

Cerebrovascular Moyamoya disease
Y. Yamashiro, H. Takahashi, and K. Takahashi
Department of Pediatrics, Juntendo University School of Medicine, 3-1-3, Hongo, Bunkyo-Ku, 113, Tokyo, Japan

Abstract. Clinical features of ten Japanese children with
cerebrovascular Moyamoya disease are reported and nonJapanese paediatric cases of the disease are reviewed from the
world literature. The most common initial manifestations
were headache in four cases (40%), motor deficit and convulsion in three cases (30%). As their recurrent and/or residual
symptoms, eight children (80%) developed motor deficit consisting of hemiplegia in five cases and paresis or weakness of
the extremities in three cases, and four (40%) had headaches.
The mode of presentation in our cases was similar to that of
non-Japanese cases, in addition to a female preponderance.
Electroencephalographic findings of prominent high voltage
delta bursts following hyperventilation and slowness of returning to the normal pattern, seen in all hyperventilated cases, is
one of the features. Of 15 carotid arteries visualised in ten
patients, sites of occlusion or stenosis were seen between the
bifurcation of the posterior communicating artery and that of
the anterior cerebral artery or the middle cerebral artery in 13
arteries, with a vascular network in the basal ganglia. This
study suggests that if hyperventilation procedures produce
prominent high voltage delta bursts during electroencephalography in children with headache and/or motor deficit,
cerebrovascular disease, especially Moyamoya disease, should
be suspected.
Key words: Moyamoya disease - Occlusion

Subjects studied and clinical findings

Sex and ages. Ten cases, six girls and four boys, all Japanese,
were diagnosed during the period from 1974 to 1981. Clinical
features are presented in Table 1. At the time of diagnosis,
the ages ranged from 2 years 5 months to 12 years 2 months
and the onset of symptoms occurred before 5 years of age in
four patients (400/0). Six children were diagnosed as having
Moyamoya disease before the age of 10 years (600/0) and four
after 10 years. The mean interval between the time of onset of
symptoms and the time of diagnosis was approximately 2 years
9 months.

Symptoms. The most common initial manifestations were
headache (four out of ten cases, 40%); motor deficit and
convulsions (three out of ten cases, 30%); nausea and vomiting were the least common (two out of ten cases, 20%).
Subarachnoid haemorrhage as the initial feature occurred in
one patient only, a 6-year-old girl (Table 2).
With regard to recurrent and/or residual symptoms, motor
deficit was seen in eight out of ten cases (80%), this number
consisted of five cases with hemiplegia and three with paresis
or weakness of the extremities. Headache was found in four
cases (40%) and convulsion, nausea and vomiting, involuntary
movement, speech disturbance, dysphagia and intellectual
deficit were present in one case each out of ten (Table 3).

Laboratory investigations. None of the cases showed abnormalities on routine haematology, liver function tests or routine
urinalysis.

Introduction

Electroencephalographic findings.
Moyamoya disease, characterised by peculiar cerebral angiographic features consisting of an abnormal network at the base
of the brain associated with stenosis or occlusion of both internal carotid arteries at the level of the siphon, was first observed
among the Japanese [25, 31]. This appearance of a fine vascular
network is described by the Japanese expression "Moyamoya",
which means something hazy like a puff of cigarette smoke
drifting in the air [41].
Although childhood Moyamoya disease presenting with
acute hemiparesis as the initial symptom is common, there are
obviously various other manifestations in children with the
disease [24].
The purpose of this paper is to summarise the clinical findings of the disease as seen in ten children and to review
paediatric cases from the world literature.

Electroencephalographic
(EEG) findings were important in that generalised high voltage delta bursts were easily activated by hyperventilation in
all but one who was too young to manage hyperventilation
(case 1). These high voltage delta bursts continued for more
than 60 s after hyperventilation had been stopped (Table 1 and
Fig. 1).

Angiographicfindings. Fifteen carotid angiographies, consisting of unilateral angiography in five cases and bilateral angiography in five cases, were performed on the ten patients; therefore 15 carotid arteries were visualised.
The sites of occlusion or stenosis are shown in Table 4.
These were determined and classified according to Handa [12]
as follows: Occlusion or stenosis of the internal carotid artery
between the bifurcations of (1) the ophthalmic artery and the

45
Table 1. Clinical features of Moyamoya disease in ten children

Case

Sex

Age
(years)

Age
at onset

Initial symptoms

Recurrent and/or
residual symptoms

EEG
Background
activity

Hyperventilation

l

F

2 5/12

17/t2

Hemiparesis
(rt limb)

Hemiparesis (rt),
dysphagia,
intellectual deficit

Asymmetry
of slow waves

Not tested

2

F

5 1/12

43/12

Headache,
abdominal pain,
vomiting

Headache,
abdominal pain,
vomiting

Normal

Diffuse high
voltage delta
bursts

3

M

6 1/12

53/12

Headache,
nausea,
vomiting

Headache,
nausea,
vomiting,
weakness of limb

Asymmetry of
high voltage
slow waves
(rt>)

High voltage
delta bursts
(lt>)

4

F

6 2/12

2

Headache,
abdominal pain

Headache,
weakness of
extremities

Normal

High voltage
delta bursts

5

F

6 8/12

68/12

Subarachnoid
haemorrhage

Headache,
bemiparesis (rt)

Slow wave
dysrhythmia

Diffuse high
voltage delta
bursts

6

M

911/12

97/12

Headache,
hemiparesis (lt)

Hemiparesis (lt)

Normal

High voltage
delta bursts
(rt>)

7

M

10 3/12

7

Hemiparesis ( I t )

Hemiparesis (lt)

Dysrhythmia,
asymmetry of
slow waves
(rt>)

Diffuse high
voltage delta
bursts

8

F

10 5/12

82/12

Tonic hemiconvulsion (rt)

Hemibatlestic
dyskinesias

Dysrhythmia,
high voltage
slow bursts
(rt >)

High voltage
delta bursts
(rt >)

9

M

11 l~

16/12

Grand mal

Grand real,
hemiweakness (lt)

Normal

High voltage
delta bursts
(rt>)

10

F

12 2/12

64/12

Grand mal

Weakness
of extremities

Voltage
asymmetry of
alpha rhythm

Regional
asymmetry of
high voltage
delta bursts
followed by
grand mal

(lt>)

rt = right, It = left, rt > = dominant at the right hemisphere, It > = dominant at the left hemisphere

posterior communicating artery, (2) the posterior communicating artery and the anterior choroidal arteries, (3) the
anterior choroidal arteries and the origin of the anterior and
middle cerebral artery, and (4) occlusion or stenosis of the
initial segment of the anterior and middle cerebral artery (A1,
M1) with integrity of the carotid siphon.
In most cases, proximal portions of the anterior and the
middle cerebral arteries were affected.
Six out of 15 arteries were affected proximal to the anterior
choroidal artery, and the artery was occluded in 6, stenotic in 9.
In the cases in whom bilateral internal carotid arteries were
visualised, both internal carotid arteries were affected to the
same level in three cases; in the remaining two cases stenotic
or occlusive changes were seen at a different levels in the four
carotid arteries, i.e. at points distal to the posterior communi-

cating artery, distal to the anterior choroidal artery or both in
one case each. Thus we confirmed that the most prominent
feature that characterises this disease is the angiographical
appearance of stenotic or occlusive changes of both internal
carotid arteries with a distinctly pathological large network of
fine vessels in the region of the basal ganglia, and extraordinarily developed transdural extra-internal carotid anastomotic
pathways.

Case histories

Two cases are described, case 4with a typical history, and case 5
with a less typical history in children.

46
Table 2. Initial symptoms of Moyamoya disease in children
Symptoms

Patients in
present study
(10 cases)
(F:M=6:4)

Non-Japanese
children
(62 cases)
( F : M = 41:21)

Motor deficit

3

45

56

15

Headache

4

14

20

34

Convulsion

3

14

13

4

Nausea, vomiting

2

1

5

11

Unconsciousness

1

4

10

29

Speech disturbance

0

9

8

7

Visual disturbance

0

2

5

7

Involuntary movement

0

2

5

4

Sensory disturbance

0

6

4

4

Intellectual deficit

0

4

4

1

Subarachnoid haemorrhage

1

1

1

24

Kudo's patients (Japanese)
Children
(89 cases)
(F:M = 54:35)

Adults
(57 cases)
(F:M = 31:26)

Table 3. Recurrent and/or residual symptoms of Moyamoya disease in children
Symptoms

Patients in
present study
(10 cases)

Non-Japanese
children
(56 cases)

Kudo's patients (Japanese)
Children
(89 cases)

Adults
(57 cases)

Motor deficit

8

48

58

22

Headache

4

4

0

2

Convulsion

1

18

0

0

Nausea, vomiting

1

1

0

0

Unconsciousness

0

1

2

4
1t

Speech disturbance

1

9

17

Visual disturbance

0

3

8

9

Involuntary movement

I

1

5

2

Sensory disturbance

0

2

2

4

Dysphagia

1

1

2

1
13

Intellectual deficit

1

7

40

Subarachnoid haemorrhage

0

3

0

0

No symptom

0

6

0

0

Case 4. The patient, a 6-year-old girl, was admitted on account
of headache and weakness of the bilateral extremities. She
had been well until the age of 2 years; from this time she
complained of abdominal pain and/or headache following
emotional upsets. From the age of 3 years the headaches were
associated occasionally with weakness of the bilateral extremities particularly when excited or angry. At the age of 4 years
she was suspected of having epilepsy from an abnormal EEG
and her symptoms seemed to improve with the use of anticonvulsants. However, the symptoms recurred when she
played the harmonica or performed physical exercises at
school at the age of 6 years. These ischaemic attacks were
clearly triggered by hyperventilation.
On examination, there were no abnormal physical or
neurological signs. The fundi were normal. EEG showed a
characteristic pattern illustrated in Fig. 1 and the waves continued for 6 min.

Carotid arteriography (Fig. 2) demonstrated typical findings of Moyamoya disease. Accentuated appearance of the perforators was observed only on the anterior substance. These
passed through the basal ganglia and reached the subcortex.
On the right side there were no major branches of the middle
cerebral artery but a vast network of fine vessels was apparent
at the cranial base.
A diagnosis of Moyamoya disease was made from this evidence and she was discharged. Subsequently she has been
doing well at school on the anticonvulsants and vasodilators
although she tends to have bouts of headache during winter,
but seldom with weakness of the extremities.
Case 5. A 6-year-old girl was semi-comatose when admitted to
hospital as an emergency. Her past medical history was unremarkable. On the day of admission, she suddenly complained of headache associated with vomiting and epistaxis.

47

Fig. 1. EEG showed 2-3 c/s high
voltage delta bursts 2 min after the
end of hyperventilation, which were
dominant on the left hemisphere at
Fp and right hemisphere at P.O. The
electrodes were applied according to
the international 10-20 system

Table 4. Sites of occlusion or stenosis of internal carotid arteries
Site of occlusion
or stenosis of internal
carotid artery

Our cases
(10 children)

Handa's cases
(16 children)

1) Above Oph.A.
Occlusion
Stenosis

0}
2

2

5}
1

6

2) Above PCo.A.
Occlusion
Stenosis

0}
4

4

3}
1

4

Occlusion
Stenosis

4}
3

7

4}
7

11

4) A1, M1, or both
Occlusion
Stenosis

2}
0

2

1}
2

3

5) Not determined

0

0

Total

15

24

3) Above ACh.A.

Oph.A. = ophthalmic artery, PCo.A. = posterior communicating
artery, ACh.A. = anterior choroidal artery, A.C.A. = anterior cerebral
artery, M.C.A. = middle cerebral artery, A1 = initial segment of anterior cerebral artery, M1 = initial segment of middle cerebral artery

Fig. 2. Left carotid arteriography revealed extreme narrowing of the
middle cerebral arteries (1), and the anterior cerebral artery did not fill
with contrast material on the left side. A dense vascular network was
found in the region of basal ganglia (~t)

Soon after she fell down and almost immediately lost consciousness. She was taken to hospital where clinical examination revealed a semicomatose state, pallor pulse rate 84/min;
respiratory rate 30/rain and regular; blood pressure 110/60
m m H g ; temperature 37.3 ~ C. Neurological findings included
n u c h a l rigidity, bilateral Kernig and Brudzinski signs. The subsequent neurological examination did n o t show a focal neurological deficit but the right ocular funds showed a blurred
pupilla. Routine laboratory data were within n o r m a l ranges
b u t a l u m b a r p u n c t u r e revealed blood-stained fluid with a pressure of 250 mm. A diagnosis of subarachnoid haemorrhage was

made and cerebral angiography was performed. Right carotid
angiography was a b n o r m a l as shown in Fig. 3. The background
activity of E E G taken on admission showed slow wave dysrhythmia (Fig. 4). Hyperventilation, after recovering from the
unconsciousness, produced diffuse high voltage delta bursts
which c o n t i n u e d for more t h a n 5 rain (Fig. 5). She remained
semicomatose for 3 days. This state was followed by a right
sided hemiparesis. No special treatment was instituted, b u t
subarachnoid haemorrhage and the right sided hemiparesis
subsided spontaneously.

48
The patient was discharged with full capacity for motor
movement on the 50th day after admission. Subsequently she
has been in excellent health apart from occasional complaints
of headache.

Discussion

Moyamoya disease was first thought to be confined to the Japanese race, however subsequent reports have revealed that it
can occur in other races [17].

The present authors have found 62 non-Japanese case of
Moyamoya disease in children in the world literature [1-11,
13-16, 18, 23, 26, 27, 29, 32-40, 42, 43] and have compared their
clinical signs and symptoms to those of the cases in this paper
and to a Japanese national survey by Kudo et al. [24].
Sex distribution has shown a female preponderance in
Japanese children ([24], 54 females : 35 males), the same ratio
in non-Japanese children (28 females : 14 males), and in this
communication (6 females : 4 males).
Though manifestations of Moyamoya disease may present
at any age, the most common time of presentation in children
is from 5 to 10 years. This was so for Japanese children, nonJapanese children and the present cases.
Weakness, paresis or paralysis of the extremities, headache
and convulsive manifestations occur more frequently in
paediatric patients, while subarachnoid haemorrhage is
usually the presenting factor in adults. The onset ofweakaaess
of a limb or limbs is sometimes characteristic at all ages. The
weakness occurs suddenly, often following hyperventilation
procedures, such as playing on a flute or harmonica, blowing
on hot soup to cool it and while undergoing E E G examination.
It often disappears quickly but frequently recurs. These modes
of presentation are presumably symptoms of mild, transitory
ischaemia [30, 44].

Fig. 3. Right carotid arteriography revealed a virtually complete occlusion of the right middle cerebral artery (t), and extreme narrowing of
the anterior cerebral artery. A fine capillary network at the basal ganglia was well visualised (tt)

In this vascular disease, there are three important aspects of
the EEG: (1) slow wave pattern and changing amplitude of
background activity in the resting record (sleeping record in
younger children), (2) localised slow waves and (3) by hyperventilation, prominent "build ups" that is high voltage delta
bursts and slowness in returning to the normal pattern. These
were the findings in all cases except one who was too young to
manage hyperventilation in this report and the present authors
propose that E E G is a useful screening test for Moyamoya
disease, although Nishimoto et al. [31] denied its value.
The angiographic findings in Moyamoya disease seem to be
fairly constant: site of occlusion, vascular network in the basal
ganglia, and well-developed transdural anastomosis. It seems
that the occlusion is usually more extensive and more marked
in the child than in the adult. As described in this study,

Fig.4. Background ~tctivity of EEG
showed slow wave dysrhythmia

49

ca

Fig. 5. Hyperventilation produced a
diffuse high voltage delta burst which
continued for more than 5 rain

the occlusions are usually bilateral in childhood, but may be
unilateral in the adult.
The aetiology of Moyamoya disease is not known and
opinion is generally divided between a congenital versus an
acquired aetiology [12, 25, 31, 32, 42, 45].
Nishimoto and Takeuchi [31] suggested an embryonic malformation of the circle of Willis with persistance ofanastomotic
networks akin to the "rete mirabile" pattern found in the
embryo. Suzuki [41] and many authors, however, proposed that
the disease is an acquired one because the development of a
Moyamoya vascular pattern has been observed following head
trauma, bacterial meningitis, tuberculous meningitis, brain
tumour, phacomatosis, arteriosclerotic vascular disease [15,
16, 42], sickle cell anaemia [38] and Fanconi's anaemia [3]. An
auto-immune reaction [42] and allergic reaction [45] have been
mentioned as aetiological conditions. The prognosis is variable. As far as the paediatric patients are concerned, the
mortality rate is lower than that of adults, but the symptoms
gradually deteriorate after fluctuation and motor deficit,
speech disturbance, convulsion and intellectual deficit will
remain as recurrent and/or residual symptoms (Table 3). If
there is subarachnoid haemorrhage as in case 5, it can be life
threatening.
From our own and other author's experiences [6, 23], anticonvulsants and/or vasodilators may be useful in the management of this disease in a therapeutic and prophylactic sense.
In recent years, surgical treatment, encephalo-myo-synangiosis, STA-MCA anastomosis [19, 20], and intracranial transplantation of the o m e n t u m [22], have been practised successfully.

Conclusion
Moyamoya disease should be suspected when hyperventilation procedures produce the characteristic patterns during
electroencephalography in children with headache and/or
motor deficit.

Acknowledgement. We thank our paediatric colleagues who allowed
us to study their patients.

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Received January 26, 1983 / Accepted August 5, 1983