Original Papers
Childs Brain 10: 1-15 (1983)

Treatment of Moyamoya Disease by
Temporal Muscle Graft ‘Encephalo-Myo-Synangiosis’
Shigekazu Takeuchi, Tadashi Tsuchida, Keishi Kobayashi,
Mitsunori Fukuda, Ryoji Ishii, Ryuichi Tanaka, Jusuke ho
Department of Neurosurgery, Brain Research Institute, and Department of Radiology,
School of Dentistry, Niigata University, Niigata, Japan

Key Words. Moyamoya disease • Cerebral ischemia • Surgical treatment •
Temporal muscle graft • Encephalo-myo-synangiosis • Cerebral blood llow
Abstract. 10 young patients from 7 to 16 years of age with moyamoya disease were
treated by temporal muscle graft (encephalo-myo-synangiosis: EMS) for the purpose of
increasing extracranial and intracranial collateral circulation. Ischemic cerebrovascular
symptoms and signs were encountered in all of them without intracranial hemorrhage.
The patients were observed for 6-26 months after the first operation. Transient ischemic
attacks disappeared in 4 out of 7 patients, and improved in frequency and duration in the
remaining 3. Paresis of the limbs disappeared in I patient and improved in 2. In 2 patients,
facial palsy disappeared and dysarthria improved as well. Ataxia disappeared in 2 patients,
and involuntary movement ofthe extremities disappeared in 2. There were no ineffective cases
clinically. In postoperative carotid angiography, the middle cerebral arteries were visualized
clearly to the peripheral portion mainly via the thickened deep temporal arteries, and the
abnormal vascular networks in the region of the basal ganglia reduced in size in most of
patients. Improvements in intelligence quotient, electroencephalogram and cerebral blood
flow were also obtained in several patients, respectively.
EMS seemed to be an effective surgical treatment in young patients with moyamoya
disease who suffered from cerebral ischemic symptoms.

Introduction

© 1983 S. Karger AG. Basel
0302-2803/83/0101-0001 S2.7.VO

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Moyamoya disease is an occlusive cerebrovascular disease with
unknown etiology. The young patients usually show cerebral ischemic

Takeuchi/Tsuchida/Kobayashi/Fukuda/lshü/Tanaka/Ito

7

symptoms and signs but little tendency to hemorrhagic manifestations.
The study of pediatric patients reveals that this disease shows a progressive
course with repeated ischemic episodes frequently resulting in permanent
deficits [13. 14]. To minimize the deficits in its progressive ischemic phase,
surgical treatment to increase cerebral blood flow has been undertaken
[1, 6-9, 15]. The temporal muscle graft was first reported in 1942 by
Kreclel [10], and was called ‘Encephalo-Myo-Synangiose’ (EMS) in
1950, according to Henschen [5]. The authors performed EMS on the
young patients with moyamoya disease for the purpose of increasing extra­
cranial and intracranial collateral circulation, and obtained satisfactory
results.

Materials and Methods

Patients
For about 2 years, we operated on 10 patients of from 7 to 16 years of age with cerebral
ischemia. 5 were males and 5 were females. The clinical manifestations were various, such as
transient and recurrent or prolonged paresis of the limbs, facial palsy, dysarthria, headache,
mental retardation, convulsion, ataxia or involuntary movement of the extremities, with
frequent alternation ofthe affected side. No intracranial hemorrhage had been encountered
in their courses (table I). A diagnosis was established in all patients with bilateral carotid and
vertebral angiographies. Preoperative computed tomography showed low-density lesions
which, in all patients, varied in size except I (case 5). EMS was performed at 15 operations
in 8 patients, and both EMS and superficial temporal-middle cerebral artery (STA-MC'A)
anastomosis was performed simultaneously at three operations in 2 patients (table II). Cere­
bral blood flow (CBF) was measured pre- and postoperalively by the ' ” Xe inhalation method
and was evaluated by using an initial slope index [19].

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Operative Technique
Following a skin incision in the frontotemporal region, the temporal muscle was
detached from the skull. After craniotomy, the dura mater was opened carefully to avoid
cutting the main branches of the meningeal artery. The arachnoid membrane was neither
opened nor stripped from the cerebral convexity of each gyrus. In several cases, however, the
arachnoid membrane was opened carefully and cerebrospinal fluid was aspirated to obtain dry
field, for serial-fluorescein angiography to investigate the cpiccrcbral microcirculation. A part
of the periosteum and fascia was stripped from the inner surface of the muscle. The temporal
muscle was simply laid over the arachnoid membrane of the cortex and sutured to the adjacent
dura. After rongeuring the lower part ofthe bone flap to prevent compression and constriction
of the pedicle of the temporal muscle, the bone flap was replaced and fastened to the cranium
and the scalp was closed in two layers. Since the muscle was thick enough in a few cases, only
the deep layer ofthe muscle was used to avoid compression ofthe brain. This modified method
was revealed to show less cosmetic disadvantage and similar effect to the method using the
whole layers ofthe muscle.

Table 1. Clinical summary ofthe cases
Name

Sex

Age on
admissioni

Age at
onset

Clinical course

Signs and symptoms on first admission

1
2
3

Ml
HM
TN

M
M
M

7y 2m
8y 2m
8y 2m

4y 9m
3 y 5m
6y 1m

left hemiparesis
no abnormal findings
ataxia, dysarthria, dysphagia, right facial palsy

4

HK

M

8y 8 m

5y 7m

CS(R)->TIA(R>-»RIND(L)
TIA(R-»L)
TIA(R)-»CS(L)-»TIA(R&L)
->CS(R) [—>TIA(L)]
RIND(R)-»CS(R)->CS(U
->RIND(R&L)

5
6
7
8

NT
SN
KS
MK

F
F
M
F

9y 6m
1Oy 11 m
I2y Ont
I3y 4nt

9y Ont
5y ?m
lOy Ont
2 y ?m

T1A(R) [—»-T1A(R&L)]
TIA(l.)-»CS(Lf-*TIA(R)
TIA(R-*L)
TIA(L-»R)—>CS(R)-»TIA(R)

9
10

RT
MT

F
F

I3y 4m
I6y 6m

11 y 4m
4y ?m

T1A(L) [—>T1A(R&L)]
TIA(R—*L)—>CS(L)

ataxia, dysarthria, right facial palsy, involuntary
movement
no abnormal findings
weakness ollcft hand
no abnormal findings
weakness of right arm. aphasia, mental
retardation
no abnormal findings
involuntary movement, sensory disturbance of
left arm and upper half of trunk, mental
retardation

Treatment of Moyamoya Disease

Case
No.

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TIA = Transient ischemic attack: RIND = reversible ischemic neurological deficit: CS = completed stroke: R&L = alTccted side ofthe limbs:
[ ) = after first operation.

Table II. Postoperative results in clinical landings and angiography

2
3

4

5
6
7
8
9
10

R
L
R
L
L

EMS
EMS
EMS
EMS
EMS

Follow-up Results
periods.
months

Clinical status at present

16
12
14
4
26

excellent

hemiparesis (-)

excellent

TIA(-)

good

10m
16m
9m
1m 3w
7m

good

ataxia(-). dysarthria!.
dysphagia(-), facial palsy(-).
TIA(-)
ataxia(-). dysarthria!.
facial palsy(-).
involuntary movement(-)
t ia !

good

TIA(-), weakness!

13m

excellent
good

TIA(-)
TIA!, weakness!, aphasia(-),
mental ability!
t ia !

R EMS
9
R EMS. ST-MC 25
L EMS. ST-MC 23
L
R
R
L
R
L

EMS
6
2
EMS
EMS
15
2
EMS
EMS. ST-MC 24
EMS
26

R EMS
L EMS
R EMS

21
16
21

L EMS

20

good

good
good

involuntary movcment(-), sensory
disturbance!-), mental ability!

Postoperative angiography
periods
after operation

visualization
basal moyaof MCA via EMS moya vessels

3m
7m
9m

marked
marked
marked
not performed
marked
marked
faint
marked1
marked1

reduced
unchanged
unchanged
unchanged
unchanged
unchanged
reduced
unchanged

7m
4m

not performed
not performed
marked
not performed
faint2
faint

reduced
reduced

4m
4m
2w
5m
9m

marked
marked
faint
marked
marked

reduced
reduced
unchanged
reduced
reduced

unchanged

EMS = Encephalo-myo-synangiosis; ST-MC = superficial temporal-middle cerebral artery anastomosis: TIA = transient ischemic attack;
(-) = disappeared; f and I = improved.
1No patency of ST-MC. 2Good patency of ST-MC.

Takeuchi/Tsuchida/Kobayashi/Fukuda Ishii/Tanaka Ito

1

Operation
• 1st, 2nd

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Case
No.

Treatment of Moyamoya Disease

5

Results

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Report o f a Representative Case
A 7-year-old right-handed boy (case 1) had been well until
November 23, 1977, the age of 4 years and 9 months, when he suddenly
complained of right hemiparesisand aphasia which lasted for 1 month. He
was admitted to the Department of Pediatrics of the Niigata University
Hospital on the day following the attack. A diagnosis of moyamoya disease
was established, by using cerebral angiography. Although the patient
showed a gradual recovery, he experienced transient weakness of the right
limbs and aphasia on January 28, 1978. The patient was discharged
without any neurological deficits on February 11, 1978, after medical
treatment.
On May 14, 1979, he suddenly suffered from hemiparesis on the left
side which was cleared up in a few days. He was then admitted to the
Department of Neurosurgery of the Niigata University Hospital on March
25. 1980. Neurological examination disclosed slight hemiparesis on the
left side. The carotid angiography on the right side showed typical findings
of moyamoya disease (fig. 1). On April 10, 1980, EMS was performed on
the right side. The immediate postoperative course was uneventful, and he
was discharged 9 days later. The patient showed gradual improvement of
left hemiparesis from 1 month after the operation. In the postoperative
carotid angiography on the right side performed 3 months after EMS.
almost all branches of the middle cerebral artery were visualized clearly to
the peripheral portion through the thickened deep temporal, the middle
meningeal, the superficial temporal and the posterior auricular arteries
(fig. 2a, b). The abnormal vascular network in the region of the basal
ganglia (basal moyamoya vessels) reduced in size as well (fig. 3). Then he
began to notice weakness in the right arm. He was readmitted to our
department for the left-sided operation on July 27, 1980. On neurological
examination, slight weakness of the right arm, and slight dysmetria on the
left side were shown. Left-sided EMS was performed on August 7, 1980.
Postoperative course was uneventful, and he was discharged 9 days after
the operation.
During the next 2-3 months he showed improvement in all of the
neurological impairments. He went to school vigorously without any
neurological deficits during the time of follow-up, 16 months after the first
operation. The postoperative external carotid angiography on the left side
performed 7 months after EMS showed similar findings to that on the right

Takeuchi/Tsuchida Kobayashi Fukuda lshii/Tanaka/Ito

6

Fig. 1. Anteroposterior (left) and lateral (right) projections of the right internal carotid
angiogram of case 1 showing occlusion of the carotid bifurcation with the extensive abnormal
vascular network in the region ofthe basal ganglia (basal moyamoya vessels). The anterior and
middle cerebral arteries are attenuated but reconstituted through the basal moyamoya vessels.
An enlarged ophthalmic artery and a tentorial branch ofthe internal carotid artery provide
transdural anastomotic pathways.

Results in ¡0 cases
The patients were observed in clinical findings, angiogram, IQ, EEG,
and CBF, for from 6 to 26 months (table II).
Transient ischemic attacks (TIA’s) which had occurred frequently
before the operation, disappeared in 4 out of 7 patients, and improved in
frequency and duration in the remaining 3. Paresis of the limbs disap­
peared in I patient and improved in 2. In 2 patients, facial palsy disap­
peared and dysarthria improved as well. Ataxia disappeared in 2 patients.

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side. Postoperative examination by using the Wechsler intelligence scale
for children. 10 months after the first operation, revealed slight improve­
ment in full-scale intelligence quotient (IQ) and remarkable improvement
in performance IQ, but slight deterioration in verbal IQ. An awake elec­
troencephalogram (EEG) at rest performed 10 months after the first EMS,
showed an improvement such as a decrease of slow waves and an increase
of alpha activity (fig. 4). Mean hemispheric value of CBF gradually
increased in the postoperative course (fig. 5).

Fig. 2. a Lateral projection, in early arterial phase (left), and late arterial phase (right),
of the right external carotid angiogram of case I, 3 months after EMS, showing almost all
branches of the middle cerebral artery via the thickened deep temporal (DTA). the middle
meningeal (MMA), the superficial temporal (STA). and the posterior auricular (PAuA)
arteries, b Anteroposterior projection of the same angiogram as figure 2a showing the bifur­
cation of the middle cerebral artery in retrograde fashion.

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and involuntary movement of the extremities disappeared in 2. Clinically,
there were no ineffective cases. Postoperative results were excellent in 3
patients who had neither neurological deficits nor TIA’s, and good in
7 patients having slight neurological deficits or less frequent TIA's. At the
time of follow-up, 8 patients attended regular school, and only 1 patient

Takeuchi Tsuchida Kobayashi/Fukuda/lshii/Tanaka/Ito

8

Fig. 3. Lateral projection of the right internal carotid angiogram of case I. taken before
the operation (left), and at the same lime as figure 2a (right), showing reduction in size of the
basal moyamoya vessels, postoperatively.
L F -C

C -P

P -0

R F -C

C -P
P -0

v/vwyvv^ —wAVvWVv^ ^ a/v^ a°V/v'—"VyVw

L F -a T
a T -p T

p T -0

_aT'/vM w ^ A A v / w X v

a T -p T

'v vAr~\v^ y A

/d^VA/i^v>/yv\AA/v

p T -0

v^ V ^ ^ ^ w vv ' ^ /s/\/V -vw W V ^*^JV ^A /\yW ^

before

J 5 0 pV
1 sec

after E M S ; R (1 0 M l, L (6 M )

Fig. 4. Awake electroencephalogram of case 1 recorded at rest, before (left), and
10 months after the first operation (right), showing a decrease of slow waves as well as an
increase of alpha activity, postoperatively.

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RF

9

Treatment of Moyamoya Disease
IS I

Ml.

7Y

M

70 -

60-

£
before

T

after

T

R -E M S

[3M]

L-EM S

R[7M]
L [3M]

R[11M]
l [7M J

attended a special class for poor learners which she had also attended preoperatively (case 8). The remaining 1 patient had finished lower secondary
class before surgical treatment (case 10). No prolonged neurological defi­
cits were encountered postoperatively in all patients.
Postoperative carotid angiography was performed on 14 sides of
9 patients between 1 month, 3 weeks and 16 months after each operation
(table II). Remarkable filling of the middle cerebral arteries mainly via the
thickened deep temporal arteries was obtained in eleven sides of 7 patients
(fig. 6), but faint filling of the arteries was obtained in three sides of
3 patients. Although external carotid angiography on the left side of
1 patient (case 10) showed only a slightly thickened deep temporal artery
and faint visualization of the middle cerebral arteries 2 weeks after EMS,
repeated angiography of the same patient performed 5 months after EMS
showed marked filling of the middle cerebral arteries. In eight sides of
5 patients, the middle cerebral arteries were filled via not only the deep
temporal artery but also the middle meningeal, the superficial temporal
and posterior auricular arteries (fig. 2a). The basal moyamoya vessels
reduced in size in eight sides of 6 patients (fig. 3). and remained unchanged
in six sides of 5 patients.
Full-scale IQ examined between 3 and 21 months after the first oper­
ation was improved significantly in 2 patients, remained unchanged in 7,
and deteriorated in 1. Performance IQ was improved in 5 patients, and

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Fig. 5. Repeated measurements of cerebral blood flow (ISI: initial slope index) of case I
showing preoperative decrease of mean hemispheric value on the left side, and gradual
increasing on both sides, postoperatively.

10

Takeuchi Tsuchida/Kobayashi/Fukuda/lshii/Tanaka/Ito

R (* M )

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Fig. 6. Lateral projections of the postoperative external carotid angiograms on the right
side of case I (MI), the left side of case 3 (TN). the right side of case 4 (HK). both sides of case 9
(RT) and the right side of case 10 (MT). showing remarkable visualization of the middle cere­
bral arteries, respectively. Each numeral indicates number of months alter the operation.

Treatment of Moyamoya Disease

11

remained unchanged in 5. Verbal IQ was improved in I, remained
unchanged in 7, and deteriorated in 2.
Among 15 hemispheres of 10 patients in which postoperative EEG
was examined between 2 and 24 months after each operation, improve­
ments in EEG such as increase of alpha activity, decrease of slow waves
and disappearance of build up were obtained in ten hemispheres of
7 patients. Postoperative EEG remained unchanged in four hemispheres
of 3 patients and revealed an increase of slow waves in only one hemi­
sphere of 1 patient.
Postoperative CBF was measured in 16 hemispheres of all patients 1-4
times between 2 weeks and 24 months after each operation. The mean
hemispheric value of CBF increased significantly in eight hemispheres of
5 patients, remained unchanged in six hemispheres of 6 patients, and
decreased in two hemispheres of 2 patients. It seemed that postoperative
CBF did not increase in the hemispheres with moderate or large lowdensity lesions in the preoperative computed tomographic scans.
There were complications postoperatively, such as sensory aphasia
lasting for 8 months (case 4), dysphagia and dysarthria lasting for I week
(case 10), motor aphasia lasting for 3 days (case 6), and subcutaneous
abscess (case 2). Sensory aphasia was considered to be caused by unsuc­
cessful establishment of STA-MCA anastomosis on the left side.
Dysphagia and dysarthria seemed to be caused by hypocapnia during the
anesthesia. The cause of the motor aphasia after left EMS might be attrib­
utable to minimal compression of the brain by the grafted muscle which
was observed in the postoperative computed tomographic scans. Although
the verbal IQ deteriorated in the patient complicated by sensory aphasia,
the other 3 patients recovered without any neurological deficits.

Discussion

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Moyamoya disease is a cerebrovascular disease consisting of progres­
sive narrowing or occlusion of the internal carotid arteries starting at the
site of the carotid siphon, bilateral abnormal vascular networks in the
region of the basal ganglia, leptomeningeal anastomoses and multiple
transdural external-internal carotid anastomoses. The disorder may occur
at any age, but more frequently in the juvenile age-group than in adults.
The clinical manifestations in children are cerebral ischemic symptoms
and signs, including hemi- and monoparesis, mental retardation, involun­

Takeuchi/Tsuchida/Kobayashi/Fukuda/Ishii/Tanaka/Ito

12

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tary movement of the limbs, and epileptic seizure and headache. It is well
known that progression of the disease process with time occurs in clinical
[3. 13, 14, 18], angiographic [4, 16, 18,20], electroencephalographic [2,3,
12], and computed tomographic findings [22].
Since the pathogenesis of moyamoya disease is still unknown, no effec­
tive treatment has been found for this disease. Medical treatment with
steroids, vasodilators, and low molecular weight dextran has been disap­
pointing. Superior cervical ganglionectomy and cervical perivascular
sympathectomy have been reported to improve the clinical symptoms
[21], but are unable to prevent the progression of the disease. The most
reasonable approach to the treatment of this chronic multiple-occlusive
cerebrovascular disease appears to be construction of anastomotic chan­
nels to increase blood supply to the brain.
Several surgical attemps have been made in the past for increasing
collateral channels. They are STA-MCA anastomosis [1,7, 9], EMS [6],
encephalo-duro-arterio-synangiosis [15], and omental transplantation [8],
Among these procedures, STA-MCA anastomosis has been performed in
most patients with this disease, and reported to be effective in improve­
ment of the cerebral circulation. However, as stated by Amine et al. [1],
STA-MCA anastomosis is not easily applicable in small children because
of the size of their cerebral and superficial temporal vessels. Besides our
case (case 4), several cases which worsened after STA-MCA anastomosis
in this disease have been reported [17, 24], Furthermore, moyamoya
disease is characterized by not only occlusions of the circle of Willis but
also multiple stenotic and/or occlusive lesions of cortical arteries as
demonstrated by pre- and postoperative angiography [4, 11], For this
reason, the selection of the arterial branch for anastomosis is very impor­
tant, and double or triple anastomoses, if feasible, are recommended.
On the other hand, EMS, which is placing of the temporal muscle on
the surface of the brain, seems to be easier and safer than STA-MCA anas­
tomosis. Moreover, visualization of the middle cerebral arteries in the
postoperative external carotid angiography seems to be shown more
widely by EMS than by single STA-MCA anastomosis, in some cases.
Although there were cases in which it was possible to perform STA-MCA
anastomosis in this series, we performed EMS alone and obtained a satis­
factory effect similar to that of STA-MCA anastomosis. However, the
effects of EMS alone in clinical findings seem to appear 2-3 months after
the operation, more slowly than those of STA-MCA anastomosis.
In three sides of 3 patients (cases 3, 7 and 8), only faint filling of the

Treatment of Moyamoya Disease

13

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middle cerebral arteries in the postoperative external carotid angiography
was shown (table II). Among the 3 patients, STA-MCA anastomosis had
also been performed on I patient (case 7). In this patient, the middle cere­
bral arteries were visualized markedly via the thickened superficial
temporal artery but faintly through the deep temporal artery. In the other
2 patients, large low-density lesions had been present in the temporo­
parietal region on the preoperative computed tomographic scans.
Furthermore, only a small part of the temporal muscle had been used for
EMS to preserve the spontaneous multiple transdural cortical anasto­
moses in I patient (case 8).
There was no complication which seemed to be attributable to EMS
in this series except for the motor aphasia in 1 patient. Matsushima et al.
[15] described that 1 patient experienced focal seizures after EMS which
necessitated an increase in anticonvulsants. But we experienced no cases
with epileptic seizures postoperatively in this series.
We performed serial fluorescein angiography in several cases, in order
to know the epicocerebral microcirculation of this disease [23]. The time
intervals from carotid injection of fluorescein dye to the first filling of the
cortical arteries were various in each gyrus, and were delayed in most gyri.
Moreover, the vessels of some cortical surfaces were not filled even in the
last frame at 26.9 s after carotid injection of the dye. Arteriovenous transittimes ofthe dye were also prolonged in most of the cases. From these fluor­
escein angiographic findings, the epicerebral microcirculation in
moyamoya disease seemed to be isolated in each gyrus or part, and to be
various and prolonged in circulation time in most cases.
It is necessary to know the natural history of moyamoya disease for
evaluation of the operative results. The study of pediatric patients revealed
that this disease showed a progressive course for about 2-3 years, with
repeated cerebral ischemic episodes frequently resulting in permanent
deficits [13]. M aki et al. [14] reported the results of clinical follow-up
studies of 24 cases with onset below the age of 13, which had average
follow-up periods of 5 years and 9 months. They suggested that the prog­
noses of moyamoya disease were good in about one third, poor in one
third, and somwhere between these two (borderline), in the other third.
Furthermore, they pointed out that the factors causing a poor prognosis for
mental function were early onset, repeated TIA’s followed by residual
neurological deficits, symptoms suggesting bilateral or dominant hemi­
spheric lesions, and angiographically widespread occlusion. In our series,
6 patients had both TIA’s and completed stroke, and 4 patients had TIA's

Takeuchi/Tsuchida/Kobayashi/Fukuda/Ishii/Tanaka/Ito

14

only, involving bilateral hemispheres in the courses of all 10 patients.
2 patients (cases 8 and 10) who had had mental retardation before the
surgical treatment, improved slightly in mental function postoperatively,
but 1 of them attended special classes for poor learners, pre- and postoper­
atively. None of the patients had poor prognosis for motor function.
Although it is not so easy to compare our operative results with natural
history, the surgical treatment of moyamoya disease including EMS seems
to be effective procedure for preventing the progression of clinical findings
caused by cerebral ischemia and increasing CBF.
Because of a small number of cases, and short follow-up periods in this
report, further studies and long-term follow-up are required. On the basis
of the results in this series, including fluorescein angiographic findings, we
believe that EMS is a reasonable and adequate procedure for the purpose
of increasing extracranial and intracranial collateral circulation in chil­
dren with moyamoya disease.

References

2
3

4
5

6

7
8

9
10

Amine, A.R.C.; Moody, R.A.; Meeks, W.: Bilateral temporal-middle cerebral artery
anastomosis for moyamoya syndrome. Surg. Neurol. 8: 3-6 (1977).
Aoki. Y; Hiraga. H.; Ichijo, S.: EEG of moyamoya disease. Electroenceph. clin. Neuro­
physiol. 43: 490 (1977).
Carlson. C.B.: Harvey, F.H.; Loop. J.: Progressive alternating hemiplegia in early child­
hood with basal arterial stenosis and telangiectasia (moyamoya syndrome). Neurology.
Minneap. 23: 734-744 (1973).
Handa, J.; Handa. H.: Progressive cerebral arterial occlusive disease : analysis of
27 cases. Neuroradiology 3: 119-133 (1972).
Henschen, C : Operative Revaskularisation des zirkulatorisch geschädigten Gehirns
durch Auflage gestielter Muskcllappen (Encephalo-Myo-Synangiose). Arch. klin. Chir.
264: 392-401 (1950).
Karasawa, J.: Kikuchi, H.: Furuse. S.; Sakaki. T.: Yoshida, Y.; Ohnishi. H.: Taki. W.:
A surgical treatment of 'moyamoya' disease. 'Encephalo-myo-synangiosis'. Neurol,
med. chir., Tokyo 17: 29-37 (1977).
Karasawa, J.: Kikuchi, H.; Furuse, S.; Kawamura. J.: Sakaki. T.: Treatment of
moyamoya disease with STA-MCA anastomosis. J. Neurosurg. 49: 679-688 (1978).
Karasawa, J.: Kikuchi. H.; Kawamura, J.: Sakaki, T.: Intracranial transplantation of
the omentum for cerebrovascular moyamoya disease: a two-year follow-up study. Surg.
Neurol. 14: 444-449 (1980).
Kraycnbiihl, H.A.: The moyamoya syndrome and the neurosurgeon. Surg. Neurol. 4:
353-360 (1975).
Kredcl, F.E.: Collateral cerebral circulation by muscle graft. Technic of operation with
report of 3 cases. Sth. Surg. II: 235-244 (1942).
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1

Treatment ofMoyamoya Disease

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13
14

15
16
17

18
19

20

21
22

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Kobayashi, K.: Takeuchi. S.: Tsuchida. T.: Ito, J.: Encephalo-myo-svnangiosis (EMS)
to moyamoya disease - with special reference to postoperative angiography (Japanese).
Neurol, mod. chir., Tokyo 21: 1229-1238 (1981).
Kodama. N.: Aoki. Y.: Hiraga. El.; Wada. T.: Suzuki. J.: Electroencephalographic find­
ings in children with moyamoya disease. Archs Neurol., Chicago 36: 16-19 (1979).
Kudo, T. : Fukuda. S.: Spontaneous occlusion ofthe circle of Willis: a disease entity, its
pathology and clinical features (Japanese). Adv. ncurol. Sci. 20: 750-757 (1976).
Maki, Y.: Nakada. Y.: Nose, T : Yoshii, Y.: Clinical and radioisotopic follow-up study
of "moyamoya’. Child’s Brain 2: 257-271 (1976).
Matsushima, Y.: Fukai, N.: Tanaka. K.tTsuruoka.S.: Inaba, Y.:Aoyagi. M.;Ohno, K.:
A new surgical treatment of moyamoya disease in children: a preliminary report. Surg.
Neurol. 75: 313-320 (1981).
Murphy, M.J.: Progressive vascular changes in moyamoya syndrome. Stroke II:
656-658 (1980).
Nakagawa. Y.: Shimoyama, M.: Kashiwaba, T ; Suzuki. Y.; Gotoh, S.: Miysaka, K.:
Takei. H.: Gotoh. S.; Ohtsuka. K.: Abc. H.; Tsuru, M.: Reconstructive operation of
moyamoya disease and its problems (Japanese). Neurol. Surg. 9: 305-314 (1981).
Nishimolo. A.: Takeuchi, S.: Abnormal cerebrovascular network related to the internal
carotid arteries. J. Neurosurg. 29: 255-260 (1968).
Risberg. J.; Ali.Z.: Wilson, E.M.: Wills. E.L.: Halsey, J.H.. Jr.: Regional cerebral blood
How by '"Xenon inhalation. Preliminary evaluation of an initial slope index in patients
with unstable llow compartments. Stroke 6: 142-148 (1975).
Suzuki. J.: Takaku, A.: Cerebrovascular "moyamoya' disease. Disease showing
abnormal net-like vessels in base of brain. Archs Neurol.. Chicago 20:288-299 (1969).
Suzuki. J.: Takaku. A.: Kodama. N.: Sato, S.: An attempt to treat cerebrovascular
‘moyamoya’ disease in children. Child’s Brain 1: 19.3-206 (1975).
Takeuchi.S.: Kobayashi. K.:Tsuchida. T.; Imamura, H.;Tanaka. R.: Ito. J.: Computed
tomography in moyamoya disease. J. Comput. assist. Tomogr. 6; 24-32 (1982).
Takeuchi, S.: Kobayashi. K.: Tsuchida. T.: Ishii. R.: Tanaka. R.: Ito. J.:Cerebral hemo­
dynamics in patients with moyamoya disease. A study ofthe epicerebral microcircula­
tion by fluorescein angiography (submitted).
Yonekawa. Y.: Handa. H.: Surgical treatment of occlusive cerebrovascular diseases
(Japanese). Brain Nerve. Tokyo 32: 239-255 (1980).

Shigekazu Takeuchi, MD. Department of Neurosurgery. Brain Research Institute,
Niigata University, I Asahi-machi, Niigata 951 (Japan)
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