The epidemiological study on 100 cases with Moyamoya disease which have been experienced at Tohoku University from 1963 to 1980 is reported. Although the results of our study are not so different from those of ever reported epidemiological studies, high frequency of inflammation above the neck, especially recurrent chronic tonsillitis, in their past history was the different one. Namely, 74% of child and 46% of adult cases have experienced inflammations above the neck, i. e., chronic tonsillitis, otitis media and sinuitis maxillaris in their past history. Morbidity of this disease is high in young girl. The average age of the onset in children was 4 years and a half, and 35 years and a half in adults. The initial symptoms of children were those due to cerebral ischemia, such as paroxysmal hemiplegia, aphasia and mental handicap. On the contrary, in adults representative initial symptoms were those due to intracranial hemorrhage.

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There have been only few reports with precise description of the prognosis of moyamoya disease. Therefore, we have conducted a long-term follow-up study of this disease especially on its clinical features. Fourty-one cases of moyamoya disease were analized on the onset of illness in detail. Twenty-seven cases were children and fourteen cases were adults.
According to the clinical type of onset, they were classified into 20 cases of ischemic attack, 5 cases of epileptic attack, 1 case of haemorrhagic attack and 1 case of other type in children, and 9 cases of haemorrhagic attack, 3 cases of ischemic attack and 2 cases of other clinical pictures in adults. Out of 41 cases, 36 cases have been followed for 2 years to 32 years. In five of them, various surgical vascular reconstruction was performed. Therefore, the remaining 31 cases were the materials of this follow-up study.
At the time of this follow-up study, there were 9 cases of normal daily activity, 3 cases of mental retardation, 2 cases of some neurological deficits and 6 cases of mental retardation plus some neurological deficits in children, and 6, 0, 4 and 1 cases respectively in adults.
The methods of vascular reconstruction were encephalo-myo synangiosis in 3 cases, encephalo-myo synangiosis plus STA-MCA anastomosis in one case and encephalo-myo synangiosis plus encephalo-duro-arterio synangiosis in one case. The result of surgical treatment is excellent in 3 cases.

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Long term follow up study (3.5-16 years duration) was performed on 26 cases of Moyamoya disease with drug therapy. These cases were divided into two groups, using cerebral vasodilator and “cerebral vasodilator and metabolic activator”. There is no definite difference between two groups regarding clinical and EEG improvement when considered severity of the disease each cases.
Improvement of EEG in resting record and during and after hyperventilation with 5% CO₂ inhalation were obtained. All cases of former group revealed improvement of clinical symptoms, two fourth of later group showed clinical improvement as well.

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Initial pathological finding in Moyamoya disease is stenotic change at the carotid fork due to some unknown causes. Many discussions have been made on the causal genesis of this change at the carotid fork, but there have been no proof on them nor any experimental study. Based on our study of 100 cases of this disease, we have found that many patients experienced chronic inflammation above the neck, i. e. chronic tonsillitis, neck phlegmon, and that pathological changes in autopsy cases are similar to those of angitis due to immunological reaction such as panarteritis nodosa.
From these facts, we presumed that the causal genesis of Moyamoya disease should be certain immunological arteritis resulted from inflammation. In order to prove our presumption, foreign protein of other animals was injected to mongrel dogs repeatedly for certain period, so that hyperimmune state was obtained. Serial arterial specimen of both groups revealed similar arterial changes of this disease, such as exfoliation of the intima, thickening of the intimal wall, rupturing and winding of the lamina elastica interna, muscle layer necrosis of the media and formation of mural thrombus. These arterial changes were limitedly observed around the carotid fork, i.e. the terminal portion of the internal carotid artery, the proximal portion of the anterior and middle cerebral arteries and posterior communicating artery. It is also characteristic that the distal portion of cerebral arteries had no pathological changes. We supposed that the localized appearance of these pathological changes should have some correlation with autonomic nerve system originating from the superior cervical ganglion, which is easily affected by infected tonsil.
According to Reilly's phenomenon, stimulated sympathetic ganglion would give influences to the arteries innervated from the ganglion. In addition to that, anatomical innervation of the autonomic nervous system is very prominent especially around the carotid fork.
Based on these facts, in other group of dogs unilateral superior cervical ganglionectomy was carried out first, then foreign serum was injected to the residual superior cervical ganglion as well as to the whole body for general sensitization. In these dogs, less arterial changes on the side of ganglionectomy were observed. Therefore, it would be possible to have some correlation with cervical ganglion for the occurrence of arterial changes at the carotid fork. From the results of our experiments, our hypothesis on the causal genesis of this disease is as follows. Inflammation causes immune reaction and stimulate the superior cervical ganglion at the same time, so that the arteritis localized at the carotid fork are induced. Changes of arterial wall resulted from arteritis are organized, and then organic stenosis at the carotid fork progresses gradually.

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To assess the genetical and immunological abnormalities in the pathogenesis of Moyamoya disease, we studied 23 patients with angiographically diagnosed Moyamoya disease including 15 child type and 8 adult type cases. A) In HLA-A, -B, -C stereotyping we have found significant association of AW24, BW46 and BW54 with relative risk of 3.83, 6.50 and 3.58 respectively. B) Natural T cell toxic autoantibody (NTA) was detected by FACS analysis in 5 of 23 patients examined. C) Millipore filter assay for double stranded DNA revealed high percent binding in 4 out of 18 patients with the disease. D) Anti-vessel antibody was not detected in any of 23 patients studied. Significant association with certain HLA types in addition to the presence of NTA and anti-double stranded DNA antibody in patients sera support the idea of genetical and immunological disturbances in the pathogenesis of Moyamoya disease.

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Both the Moyamoya Phenomenon and complete or incomplete occlusion of the internal carotid fork are essential neuroradiological findings of the true Moyamoya Disease of unknown etiology. However, the Moyamoya Phenomenon often is observed in occlusive diseases of the internal carotid fork whose etiologies are known, i. e., arteriosclerosis, tuberculous meningitis, sickle cell anemia, etc. Recently the authors observed the rather acute development of unilateral Moyamoya Phenomenon following severe vasospasm of the bilateral anterior cerebral and left middle cerebral arteries due to the rupture of an anterior communicating aneurysm. An autopsy of this case revealed the marked development of anastomotic small arteries and arteriolae in the basal part of the brain, and only on the left side. Such exteraordinarily dilated arteries mainly are branches of the posterior communicating, anterior choroidal and perforating arteries, and well correspond to the Moyamoya Phenomenon observed angiographically. There were no occlusive changes in the main intracranial arteries except for moderate stenosis of the A2 portion of the bilateral anterior cerebral arteries. Histologically, arteriosclerotic change generally was, very slight, and a slight thickening of the intima and edematous change of the media were observed only at the stenotic site. There was no histological change in the markedly dilated Moyamoya vessels. The following 4 factors have been suspected of contributing to development of the Moyamoya Phenomenon : 1. The chronology of arterial occlusion. 2. Location and extent of occlusion. 3. The cause of occlusion. 4. Anatomical and functional disposition of the basal circulation. As regards the chronology, chronic or slowly progressive arterial stenosis has been thought a mandatory factor in development of a Moyamoya network which plays an important role in the form of collateral channels. However, based on the findings outlined above, the congenital factor may be the most important of the 4 factors listed.

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10 cases having abnormal angiographical findings which showed the stenosis or occlusion of middle cerebral artery with moyamoya phenomenon were reported.
The characteristics of these cases were summarized as follows.
1) It showed high incidence in female (3 males and 7 females).
2) These patients involved two young cases ranged in age from 4 years to 62 years.
3) Intracranial hemorrhage was cofirmed in 4 cases and onset of symptom in other 6 cases was similar to that of cerebral infarction.
4) 5 cases showed a unilateral occlusion of middle cerebral artery at its origin and 2 cases showed a unilateral stenosis of M₁ portion, with moyamoya vessels in this region.
5) Two cases demonstrated bilateral occlusion of middle cerebral artery, and one showed a right stenosis and left occlusion of middle cerebral artery, with moyamoya vessels.
6) On the other hand, we could not find a stenosis on the intracranial internal carotid artery in these cases.
7) These findings suggested that such cases may be different from the so-called“Moyamoya disease”.

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A case of progressive stenosis of bilateral I-C bifurcation with Moyamoya vessels which aggravated shortly after head injury was reported.
A 28-year-old man was admitted to our hospital about 40 minutes after car-accident. He was unconscious and had right hemiparesis.
Bilateral carotid angiogram revealed no finding of intracranial hematoma but stenosis of bilateral. internal-carotid bifurcations. CT scan showed irregular high density area in the left temporo-occipital region.
On the 6th day after trauma, stenosis of left I-C bifurcation increased. About two months after trauma, stenosis aggravated progressively and Moyamoya vessels appeared angiographically.
On the other hand, right carotid angiogram taken one month after trauma showed progressive stenosis and Moyamoya vessels as well.
It is difficult to determine wheather it is traumatic stenosis or cerebrovascular Moyamoya disease.
We discussed the possible mechanism of stenosis of bilateral internal-carotid bifurcation and appearance of Moyamoya vessels.

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In this presentation, some clinical characteristics were reported based on the statistical analysis derived from 907 patients of moyamoya disease in Japan.
Among 907 patients, 518 were of those we reported in 1973, and other 389 were collected from all of the neurosurgical institutes throughout Japan for the 3 year-period from 1976 to 1978.
The incidence was higher for female than male (_??_: 360, _??_: 547) including 391 children under 15 years old and 512 adult patients over 16 years. There were seen two high peaks at the first (0-9 years) and fourth (30-39 years) decades as age destribution.
Mortality rates were 7.6%(child 2.7%, adult 11.1%). Patients were divided by clinical symptoms into four types: hemorrhage, TIA, completed stroke and seizure. Children had higher incidence for TIA and stroke types (39% in each), while adult patients had for hemorrhage type (65%).
In CT examinations of 222 patients, low density areas were found mainly in the cortical and subcortical regions, on the contrary, high density areas were found mainly in the basal ganglia regions. These findings correspond with the higher occurrence of hemorrhage from the abnormally developed basal moyamoya vascular network and with that of ischemia in the cortical region.
Follow-up study in 271 cases out of later 389 patients revealed high mortality rates of 22% in hemorrhage type patients among four clinical types, and favourable outcome (active and self-sustained) in 58% of them.
Recently vascular reconstructive surgery has become more popular and shows good results for TIA and stroke patients.
Some authors have an idea that the vascular reconstruction will prevent the recurrence of hemorrhage in hemorrhage type patients, because the load to the basal moyamoya vessels will be decreased by an increase of blood flow to the cortical vessels through the anastomotic channels formed by surgery.
However, this is still controversial and a careful long-term study of the natural history of the patients with hemorrhage will be necessary to prove the effectiveness of the reconstructive surgery.

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9 cases of adult type of Moyamoya disease were reviewed, all of which started with intracerebral hemorrhage.
During the past 5 years, we experienced 13 cases of adult Moyamoya disease, 9 of which began with intracerebral hemorrhage and the other 2 cases started with subarachnoid hemorrhage. The age of onset ranged from 22 to 55 years old. There were 7 females and 2 males.
As the initial symptoms headache and vomiting were seen in all 9 cases and the disturbance of consciousness was noted in 8 cases. As the treatment, simple evacuation of hematoma was done in a case and 4 cases were treated conservatively. Surgical extra- and intracranial arterial bypass was performed in 4 cases after absorption of hematoma. The prognosis was generally poor. Only 2 cases could return to useful life and 4 cases subsequently died. The location of intracerebral hematoma on CT scan was mostly in paraventricular region, such as the head of caudate nucleus, thalamus and putamen. The hematoma had been perforated into ventricles in all cases, the size of which was larger than original intracerebral hematoma in 5 cases.

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Seven cases with intracranial hemorrhage associated with adult moyamoya disease were observed by computed tomography in acute stage. Intracranial hemorrhages in this condition are divided into two types according to main site of hemorrhage, a) intracerebral hematoma and b) intraventricular hemorrhage. None has obvious clots in subarachnoid spaces or basal cisterns. Intracerebral hematoma in this condition is indistinguishable from that in other etiologies. However, intraventricular hemorrhage without clot in parenchyma nor in basal cistern is thought to be specific to moyamoya disease. From the observation of cases reported here we believe that the hemorrhage occurs initially in cerebral parenchyma or near or within lateral ventricle in this disease.

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Seven cases of“Moyamoya”disease manifested with intracranial hemorrhage were presented, 2 males and 4 females, ranging 14 to 49 years old. Five of 6 cases initiated with hemorrhage, one once experienced with an old episode of TIA in the childhood. On angiogram aneurysm was demonstrated in moyamoya networks in two cases, one at the basilar top and the other in the periventricular region, and it was presumably responsible for hemorrhagic site. On CT scan of 5 cases, two different sites of hematoma, the basal ganglia and periventricular area favoring the trigone of the lateral ventricle, were characterized. According to the severity of the hemorrhage, 3 cases were treated conservatively and 4 cases surgically. Two of 4 cases required emergent surgery, ventricular drainage and hematoma evacuation respectively. After the acute stage, 2 months after the onset, STA-MCA bypass operation were performed in order to reduce the chance of repeated hemorrhage in the near future as well as to prevent ischemic problem. As long as followed-up period of 6 months to 7 years, prognosis was evaluated favorable in the most without repeated hemorrhage nor ischemic episode. Even in the hemorrhagic cases of“Moyamoya”disease the bypass operation will be available in terms of reducing the possibility of repeated hemorrhage, because of diminishing the networks, main sources of the hemorrhage, after the revasculization procedures.

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Fifteen cases of Moyamoya disease, which were treated in our hospital from 1960 to 1980, are studied and followed up. Follow up terms are ranging from a year to 24 years. Seven young patients whose ages are under 15 years old and 8 adult patients are included in this series and the male to female ratio is 3: 4 in the former group and 3: 5 in the latter group. The initial symptoms are ischemic cerebrovascular attacks (75%), subarachnoid hemorrhage (20%) and intracerebral bleeding (7%). In the young patient group, the main symptoms and signs are ischemic cerebrovascular attacks which often recur and major neurological deficits are left in many cases. However, the mortality of cerebrovascular attacks is very low in this group. In adult patients, on the other hand, intracranial hemorrhage such as subarachnoid or intracerebral hemorrhage occurres at the high rate of 50%and 25%, respectively, and their mortality rate is rather high.
As the etiology of Moyamoya disease is still obscure, no effective treatment has been found as yet. However, STA-MCA anastomosis is effective to prevent the recurrence of ischemic cerebral attack in some cases and decreases the amount of abnormal vascular net in all cases. So far as the treatment of intracerebral hemorrhage in Moyamoya disease is concerned, evacuation of hematoma alone is often followed by recurrence of bleeding from the abnormal vascular net around basal ganglia (the so-called Moyamoya) later. In this series, a patient died of such recurrence of cerebral hemorrhage and his autopsy revealed a large putaminal hemorrhage from one of the abnormal vessels itself in the left basal ganglia. Because STA-MCA anastomosis decrease the burden of the abnormal vascular net-work in respect of collateral circulation, it may also be the effective treatment both for Moyamoya disease with intracranial hemorrhage, and for the prevention against the recurrent hematoma in the case that the abnormal vascular net-work should result in the origin of the hemorrhage.

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Pediatric Moyamoya disease with the onset of intracranial hemorrhage has been considered to be rare. I have recently experienced 2 cases of pediatric Moyamoya with intraventricular hemorrhage. Both patients (aged 5 and 11) developed tonic convulsion followed by coma for a few months. Angiography revealed no findings except for typical Moyamoya disease. Plain CT showed that bleeding occured in primary lateral ventricle or periventricular region as in adult Moyamoya disease.

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22 cases of Moyamoya disease were analyed clinically and an interesting case with aneurysm coincided with the hemorrhagic point was presented. 7 of 22 cases were children and 15 were adult. Female was about 3 times as much as male, i. e., 5 males and 17 females. Initial symptoms were ischemic attack in 7 of 22 cases, convulsive seizure in 5 cases and intracranial hemorrhage in 10 cases.
All cases which had intracranial hemorrhage as initial symptom were females over 17 years old. Rebleeding occurred in 2 cases, one of which associated with a small aneurysm localized in periphery of Heubner's artery. Its aneurysm ruptured during lt-cerebral angiography, which showed the extravasation into the ventricular system.
By repeating the lt-CAG, the spontaneous regression and final disappearance of this aneurysm were demonstrated. It was supposed that the aneurysm as discribed above was a false one, which suggested initial hemorrhagic point.

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The spontaneous occlusion of the circle of Willis occurs in a juvenile group and an adult one. The authors proposed a hypothesis that the symptoms belong to one disease entity which appears in a juvenile stage and, after an asymptomatic period, reappears in an adult stage as a hemorrhagic type. For proving the hypothesis, the authors tried to find a relation between both groups. At first the hemorrhagic source of intracranial hemorrhage in the adult group was investigated to decide when the symptom appeared and show the difference in the pathological conditions of both groups. The authors employed serial cerebral magnification besides conventional cerebral angiography, and precisely observed the cerebral vasculature in each age stage with magnification rentogenography. The 24 cases with spontaneous occlusion of the circle of Willis examined in this work consisted of 11 cases of the juvenile group and 13 cases of the adult one. In 7 cases of the latter which were diagnosed as subarachnoidal hemorrhage and subject to CT examination soon after the diagnosis, 3 cases had miliary aneurysm of less than 1mm diameter in the basal abnormal vascular network (Table 1). Also small aneurysms greater than 1mm in diameter were found in the anterior and posterior choroidal arteries in 3 cases (Table 2). Magnification cerebral angiography showed that the incidence of miliary aneurysm was low in 3 cases of the juvenile group: the incidence of small aneurysms greater than 1mm in diameter was 1/11 in the juvenile group and 4/13 (31%) in the adult group. Since intracranial hemorrhage due to the rupture of small aneurysms has been reported, the miliary aneurysm must be noted as an important source of hemorrhage. The fact that the incidence in the juvenile group is lower than that in the adult one suggests the increase of aneurysm with aging; it will explain the difference of the incidence of intracranial hemorrhage between both groups. And the result suggests that the disease will progressively change its pathological conditions from juvenile group to adult one and the disease belong to one disease entity.

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A 39-year-old woman, who had suffered from severe headache followed by disturbance of consciousness which was caused by the ictus of ventricular hemorrhage, was angiographically shown to have an aneurysm arising from the posterior choroidal artery associated with Moyamoya disease.
Within several days her symptoms due to ventricular hemorrhage have gradually subsided and she was discharged 3 months later without any neurological deficit.
During and after hospitalization the posterior choroidal artery aneurysm was treated conservatively under careful observation by means of follow-up CT scanning with the aid of contrast infusion.
On CT scanning, having been detected as a tiny enhanced spot adjacent to the lateral wall of the trigone of the right lateral ventricle, the aneurysm finally disappeared on the 111th day after the attack.
Corresponding to disappearance of an enhanced spot on CT scanning, the posterior choroidal artery aneurysm couldn't be angiographically demonstrated any more. This presented case and some previously reported cases show that an aneurysm arising at the Moyamoya vessel itself or its collaterals is thought to be not true one but a pseudo-aneurysm and should be treated conservatively.
Nowadays, CT scanning with contrast infusion has enabled us to complete follow-up studies as an alternative procedure of such an aneurysm safely and repeatedly, not requiring frequent hazardous angiography.

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This paper presents one moyamoya case which developed so called cortical deafness following bilateral intracerebral hemorrhages. The patient is right handed 18 year-old girl whose moyamoya vessels were found incidentally when she had head-injury at 10 years of her age.
She has had hemorrhagic episodes in two times. The first attack, on May, 30, 1976, was right putaminal hemorrhage and treated conservatively. The second one, on Oct., 16, 1979 was left temporal subcortical massive hemorrhage and removed the hematoma of 150g by craniotomy. On 7th postoperative day, she seemed to be fully awake, however, she did not respond to our call at all. On 42nd day, she could make contact only by means of writing, and still could not utter any useful words at all. The results of Standard Examination for Aphasia on Jan., 20, 1980, were as follows: words repeating, comprehension of languages, writing letters and sound differentiation were disturbed markedly; reading with loud voice and sense of the sound direction were disturbed slightly; spontaneous speech or writing and comprehension of a letter were normal. Several neuro-otological examinations were performed subsequently. The pure tone audiometry showed severe hearingloss on both sides on 42nd day after the second attack, but 6 months later it recovered to the point of minimum damage. The speech audiometry, however, pointed out her complete disability of discriminating speech bilaterally even on 6 months after the second attack. The auditory brain stem response by click sounds was normal. But the middle latency component was abnormal and no clear wave components were identified. All components of the waves of the slow vertex potential were obtained in almost normal patterns, but the latencies were delayed modelately.
From the clinical features and otological examinations, it is presumed that she had so called cortical deafness in early stage, and then turned gradually to the auditory agnosia or pure word deafness. It is also suspected from CT scans and neuro-otological examinations that the responsible lesions to these symptomes are bilateral auditory radiations at sub-lenticular portion of the internal capsules and they do not include the medial geniculate bodies. In this case, not only the left transverse temporal gyrus (Heschl), but also the right one might have played some role in addition to the auditory radiation.

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Biplane computed cerebral angiotomography of Moyamoya disease is demonstrated in this paper.
The specific features of Moyamoya disease by the computed cerebral angiotomography are as follows; none or poor visualization of the carotid fork and the abnormal cloudy region consisted of irregular tortuous or patchy high density vascular components extending from the basal cistern to the basal ganglia.
Modified coronal CT and intravenous minimum dose bolus injection method seemed to be more useful for visualization of these features.
Before carotid angiography we can suspected Moyamoya disease to find these features on CT image definitely.
Cerebral angiography has been the only diagnostic method of Moyamoya disease. Instead of this invasive examination, computed cerebral angiotomography could be useful enough to detect Moyamoya disease conveniently and non-invasively.
So that, we conclude that computed cerebral angiotomography is very useful method for screening and follow up study of Moyamoya disease.

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In order to clarify the clinicopathological aspects of Moyamoya disease (spontanecus occlusion of circulus arteriosus Willisi), the conditions causing the abnormal cerebral vascular networks (Moyamoya vessels) were studied by means of cerebral angiography, computed tomography and clinical findings.
1) The cerebral juxtabasal vascular networks seemed to be the parenchymal vessels such as medial and lateral striate arteries, thalamoperforating, thalamogeniculate, anterior mamillary, anterior and posterior choroidal arteries etc., which became dilated and tortuous as collateral anastomoses caused by occlusion of carotid forks. Occlusion of the proximal internal carotid artery preserved circulation of A₁, M₁ and anterior communicating artery did not cause the cerebral juxtabasal vascular networks.
2) Leptomeningeal anastomoses and transdural cortical anastomoses seemed to be caused by occlusion of any portion of internal carotid artery.
3) Cerebral ischemic syndrome and cerebral infarction may result from insufficient collateral circulation, which may be observed in the borderland between two collaterals. Various distributions of collateral circulation may result in the varicus aspects of cerebral infarction or cerebral atrophy in this disease.

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Many kinds of the collateral cerebral circulation on moyamoya disease are already discussed by many authors, such as by the ophthalmic A, external carotid A, meningeal A, choroidal A, and sagittal sinus.
In many case of the moyamoya disease, the moyamoya vessels around the circle of Willis has the role of one part of the collateral pathway of the cerebral circulation.
In this paper, the result of the clinical research work on our moyamoya series upon the collateral circulation pass through the moyamoya vessels compared with the other collateral circulations were discussed.
The result of this study is comparatively considered with clinical course as the classification for juvenile type, hemorrhagic type and adult type.
The abundance of the collateral circulation from the moyamoya vessels to the cortical vessels has intimate relation with the clinical classification.
In the juvenile type, the collateral circulation from the moyamoya vessels to the cortical vessels is observed rather well condition, with the collateral circulation of another types.
In the hemorrhagic type, the condition of the collateral circulation is quite different from the juvenile type. The collateral circulation from moyamoya vessels is poorly observed, with, at the same time, poorly collateral circulation of another types.
On the adult type, the condition of the collateral circulation observed as the midways of the above two types.

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Transdural anastomosis in Moyamoya disease, which we have named as“Vault Moyamoya”,is reported. It seems that these vault moyamoya vessels are apt to be developed at certain fixed positions; 9 definite places in children cases and 6 in adults. Main feeding arteries of each vault moyamoya are dural arteries. In addition to them, the blood flow from superficial temporal artery (STA) and occipital artery is found.
The incidence of vault moyamoya at each position shows similar tendency both in children and adults cases. Vault moyamoya are developed more frequently around the base of the brain near the carotid fork, where occlusion exists. Less moyamoya are formed as the position is farther from the carotid fork. In children, the incidence of vault moyamoya seems to coincide with the phases of basal moyamoya.
Blood flow of vault moyamoya to the brain cannot be ignored. When STA-MCA anastomosis is applied to moyamoya cases, we had better take the degree of development and positions of vault moyamoya into consideration, becauses we have to cut STA and middle meningeal artery in the procedure.

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Attempts were made to evaluate the EEG findings in 34 children and 28 adults with Moyamoya disease.
1) The children revealed specific findings such as posterior slow, centrotemporal slow and Rebuild up after the end of hyperventilation.
2) Posterior slow was observed in EEG with high frequency examined within one year after the initial onset. After one year from the initial onset, centrotemporal slow was revealed. After 3 years and a half from the onset diffuse inactive pattern was observed.
3) Rebuild up was most characteristic findings in children Moyamoya.
4) In adults moyamoya, no specific findings have been observed yet.

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A pathological study on 10 autopsy cases of Moyamoya disease is reported. Two cases were children under 15 years old, and 8 cases adults. Six of 8 adult cases died of intracerebral hemorrhage. Bleeding point of 3 cases was suspected to be putamen, 2 cases thalamus and one claustrum. It was verified that hematoma ruptured into the lateral ventricles in all the cases.
Microsection of the affected main cerebral arteries at the base of the brain showed a marked thickening of the intima due to cellular or fibrous tissue, a tortuous appearance and reduplication of the internal elastic fibers and an atrophy of the media. No difference was found on pathological findings of arteries between children and adults. Some of the perforating arteries, which consisted basal moyamoya vessels, were dilated, while the other were occluded due to thickening of intima.
In 2 cases, main arteries in the whole body were examined. Similar changes to those of the cerebral arteries were observed in renal, coronal and aortic arteries. Therefore, it is possible that Moyamoya disease would be the manifestation of the general vasculitis at the brain.

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It is well recognized that some patients with Moyamoya disease frequently suffer from subarachnoideal and/or intracerebral hemorrhages. The morphogenesis of final massive encephalic hemorrhage is unsettled controversy. Moreover, little attention is paied for the histology of the perforating arteries which are probably responsible for intracerebral hemorrhage in patient with Moyamoya disease.
The present report dealed with an electron microscopic observation of the perforating arteries and circle of Willis in three cases of Moyamoya disease who dies of intracerebral hemorrhages. The perforating arteries in distal portion distributed in the basal ganglia revealed marked degeneration of the media; moth eaten-like atrophy of smooth muscle cells and intercellular deposition of increased basement membrane-like materials with electron dense cell debris. This medial alteration is provisionally termed “exhausted degeneration” and is similarly occurred in the same area of perforating arteries in patients with severe hypertension, frequently suffered from intracerebral hemorrhage. On the other hand, the perforating arteries in proximal portions showed well preserved media-smooth-muscle cells.
Ultrastructurally the circle of Willis in Moyamoya disease consisted of co-existence of a phase of remarkable intimal cell proliferation with vasospasm and a phase of consequent ischemic degeneration of the media and deeper thickened intima.

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A 42 year-old male with cerebrovascular moyamoya disease died of massive intracerebral hemorrhage Contrastmedium-injected brain was examined by microangiography and translucidation to know the role of moyamoya vessels in the cisterns and the brain.
Moyamoya vessels in the cistern branched from the posterior communicating, anterior choroidal and middle cerebral arteries connected each other. Microangiogram and translucidation revealed moyamoya vessels in the basal ganglia and the lateral ventricle, which connected with cortical arteries. Thin and long branches, which ramified from the moyamoya vessels in the basal ganglia and the lateral ventricle, ran from the ventricle wall to the cerebral cortex. They may be called “ventriculofugal medullary arteries”. Anastomotic vessels were also visualized in the moyamoya vessels and the medullary arteries.
Moyamoya vessels play a role as collateral way to maintain normal ventriculopetal perfusion. And ventriculofugal perfusion newly developed.
Moyamoya vessels developed around the lateral ventricle wall are probably responsible for ventricular hemorrhage.

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Twenty two autopsied cases with Moyamoya disease were examined clinicopathologically. Pathologically, fibrosing stenoses or occlusions involved the circle of Willis and its major branches in all cases. In 14 of 20 patients numerous collateral channels, muscular in type, paralleled the circle, bypassing the occluded natural passages. Fresh and massive cerebral hemorrhage was confirmed in 17 and cerebral infarcts in 4 among 22 patients. Among these 17 patients, massive hemorrhage was found in the basal ganglia, thalamus and hypothalamus of 12 patients, and in the thalamus, cerebral peduncle and midbrain of 5 patients. Rupture of dilated small muscular arteries was demonstrated in fresh hemorrhagic lesions in 5, and old hemorrhagic lesions in 1 of the 17 patients. Saccular aneurysm of cerebral arteries in the subarachnoid space was present in 2 of 22 patients. In one patient posterior cerebral-posterior communicating aneurysm was ruptured in the arachnoid space. No rupture involved the perforating arteries in the arachnoid space. These findings suggest that rupture of overgrown perforating arteries as collaterals in brain may be the main cause of single or repeated cerebral hemorrhage in Moyamoya disease.
On the other hand, stenosing lesions were found in perforating arteries. Elastofibrotic thickening of the intima and the media, edematous intimal thickening and luminal narrowing due to organized thrombi were observed. These stenotic lesions are an important factor in the occurrence of cerebral infarcts.
Coronary arteries and renal arteries were examined in 11 patients. In one patient, concentric intimal fibroplasia resembling intimal fibroplasia type of fibromuscular dysplasia caused marked stenoses in coronary and renal arteries. Eccentric fibrous intimal thickening or atheromatous lesions were present at the bifurcation of coronary and renal arteries in majority of adult cases. These findings suggest that systemic arterial involvement including intracranial vessels may exist in Moyamoya disease.

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Regional cerebral blood flow (rCBF) was measured in 13 cases of Moyamoya disease by means of the ₁₃₃Xe intracarotid injection method. Patients' age ranged from 5 to 55 (average 32), 6 were male and 7 were female.
Mean hemispheric CBF (mCBF) in the symptomatic hemisphere ranged from 19.0 to 41.0 (average 31.3±7.5) ml/100g/min. and from 33.0 to 57.6 (average 48.2±7.0)ml/100g/min. in the asymptomatic side. In the asymptomatic hemisphere, 6 cases had normal mCBF and only one case with cerebral infarction, confirmed by CT had abnormally low flow (33.0 ml/100g/min.). Decrease of mCBF correlated well with the severity of clinical signs. But, at the same level of decreased mCBF, clinical signs were more milder in Moyamoya disease than in the other cases of cerebral infarction such as occlusion of middle cerebral artery. This phenomenon may be explained by the slow progress of steno-occlusive process with concomitant development of netlike collateral circulation (Moyamoya-vessels).
Relative hyperemic regions were found mainly on the base of the frontal lobe, but absolute hyperemic area was not observed in the regions of angiographically verified Moyamoya-vessels.
CO₂ response appeared to be disturbed during CO₂ inhalation and preserved during hyperventilation tests. This dissociation of CO₂ response is known as one of the characteristic features of Moyamoya disease. The authers' explanation is, that the vessels are already maximally dilated under normocapnic circumstance in order to maintain adequate CBF and cerebral function. Therefore, further dilatation due to hypercapnia is impossible, however, hypercapnia by hyperventilation is still able to cause vasoconstriction.
Autoregulation observed during blood pressure changes was disturbed frequently, but further analysis will be necessary. The ₁₃₃Xe intracarotid injection method bears many problems in evaluating rCBF in Moyamoya disease. Three dimensional rCBF and metabolism measurements by Emission CT will be of great help to know more precise pathophysiological condition of Moyamoya disease.

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Many reports about moyamoya disease were published, but there were a few reports dealing with hemodynamic aspects in cases with moyamoya disease.
In this study, regional cerebral blood flow values (rCBF values) were measured at resting state and during 5% CO₂ inhalation by Xe-133 clearance method in 11 cases with moyamoya disease and in 5 cases with unilateral moyamoya phenomenon in cerebral angiography and hemodynamic aspects in those cases were discussed.
The examination was carried out in chronic phase more than 3 months after subarachnoid hemorrhage or transient ischemic attack. In 11 cases with moyamoya disease, 3 cases were children under 20 years-old and 8 cases were adult. Five cases with unilateral moyamoya phenomenon were all adult.
At the resting state, rCBF values were normal in 5 cases and abnormally low in 3 cases out of 8 adult cases, and abnormally low in all 3 young cases. In cases with unilateral moyamoya phenomenon, rCBF values were normal in 3 cases and slightly low in 2 cases. Clinical features and EEG findings were not different between those 2 groups.
rCBF measurements during 5% CO₂ inhalation were carried out in 9 cases with moyamoya disease and 4 cases with unilateral moyamoya phenomenon. Increase of rCBF values compaired with the values at resting state was found in 3 cases out of 7 adult cases with moyamoya disease and in all 4 cases with unilateral moyamoya phenomenon, but was not noted in 2 young cases with moyamoya disease.
Those results indicate that discrepancy of hemodynamic conditions is noted between cases with moyamoya disease and cases with unilateral moyamoya phenomenon. Furthermore, the discrepancy is also found between adult cases and young cases with moyamoya disease. The reason of those discrepancy can not be fully explained yet, but it is supposed to be mainly due to the difference of anastomotic conditions in small cortical branches in those cases.

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The purpose of this study is to report the results of regional cerebral blood flow (rCBF) in cases with cerebro-vascular Moyamoya disease. The rCBF was measured in their chronic state using a gamma camera system (Toshiba DAP 5000N) with Xe133 clearance method. The subjects consist of 2 cases suffered from subarachnoid hemorrhage, 2 cases primary intraventricular hemorrhage and 3 cases cerebral infarction. The results obtained from this series were as follows;
1) rCBF in frontal region at rest showed relatively high value in 5 cases and in 2 cases low value comparing with mean CBF.
2) rCBF in parieto-occipital region at rest showed relatively low value in 5 cases and high value in 2 cases comparing with mean CBF.
3) rCBF in the area corresponding to Moyamoya region generally showed slightly low value and showed relatively high value in 2 cases comparing with mean CBF. These findings coincide rather well to the development of Moyamoya in cerebral angiography.
4)rCBF changes by hyperventilation test revealed flow reduction in frontal, central and parietooccipital region, but the flow was almost unchanged in Moyamoya region by this test.
Thus, from the data mentioned above following consideration might be indicated, i. e. the flow was relatively maintained in Moyamoya region as well as in other cortical regions of the same hemisphere. Additionally, vascular CO₂ response to hyperventilation test seemed to fairly be abolished in the area corresponding to Moyamoya region, but it seemed rather intact in the other cortical regions.

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A role of basal moyamoya networks on cerebral circulation has not been clarified, mainly because of methodological limitation. The authors measured regional cerebral blood flow (rCBF) in 7 patients with moyamoya disease, by means of ¹³³Xe intracarotid injection method with reliable numbers of matrix as many as 87 and the following results were obtained.
In 9 out of 10 sides in 7 cases, rCBF in the hemispheres and the white matters covering moyamoya vessels were significantly higher than those in the areas other than“the areas covering moyamoya vessels”although rCBF in the areas covering moyamoya vessels were still low compared to control. Therefore, it was concluded that basal moyamoya vessels played a significantly efficient role on maintenance of cerebral blood flow in the basal ganglionic areas and the white matters.

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Regional cerebral hemodynamics on twelve cases with Moyamoya disease was investigated by intra-venous ¹³³Xe injection method using“Valmet rCBF Analizer”Eight cases out of twelve were children and other four cases were adults. Although their initial symptoms were ischemic attacks, no or only slight neurological deficits were found at the cerebral blood flow studies.
In the adult group, hemispheric cerebral blood flow was within the normal range. While in the child group, it was higher than the adult group. The distribution of the regional cerebral blood flow was characteristic in both groups. Namely, regional cerebral blood flow in the frontal and temporal region was relatively lower than the parietal and occipital region. It seems to indicate the importance of blood supply from vertebro-basilar system in Moyamoya disease.
Cerebral blood flow changes by hyperventilation was investigated in order to solve the mechanism of“Rebuild up”on EEG. But there was no difference between the cases showing“Rebuild up”and no“Rebuild up”though the cerebral blood flow after hyperventilation was decreased in both groups.

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¹³³Xe inhalation rCBF measurements were performed twenty-one times on seven patients with“Moyamoya”disease, four of whom were studied both pre- and post-operatively. Furthermore six of them were also studied with CO₂ response of rCBF. Because of its easiness to re-examine and non-invasiveness inhalation method is thought to be clinically useful, especially studying with CO₂ response on the patients with the cerebrovascular disease. And our study showed firstly the impairment of physiological response to hypercapnea on the young patients with“Moyamoya”disease, which have been well investigated with other methods. Secondary we observed the increase in inhomogeneous distribution of the cortical blood flow in post-operative period on the patients who were surgically treated with the vascular reconstruction method.

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Fluorescein angiography (FAG) was performed at 12 operations of 9 patients with Moyamoya disease in order to know the epicerebral microcirculation. As Encephalo-Myo-Synangiosis (EMS) was performed for the purpose of increasing extra- and intra-cranial collateral circulation in all patients, the cortical surface of the frontotemporal lobe was exposed widely. At first 0.05ml/weight kg of 1% fluorescein was injected into the common carotid artery of the side of the brain (CCA method), and then 5m1 of 10% fluorescein was injected into the peripheral vein (IV method).
The intervals from carotid injection to first appearance of the dye in the cortical arteries were various in each gyrus and were delayed in the most part. In 6 hemispheres of 6 patients, the vessels of some cortical surfaces were not visualized even at the last frame, 26.9 seconds after carotid injection. But the dye appeared in all of the exposed areas by IV method. Regional circulation time was also prolonged in the most part and took more than 10 seconds in some areas of 4 hemispheres of 3 patients. Extravasation of the dye from the small arteries was seen in 5 hemispheres of 4 patients.
FAG findings were nearly correlated with findings of conventional cerebral angiography, but were not always correlated with CT findings and cerebral blood flow measured by ¹³³Xe inhalation method.
On the basis of FAG findings, we consider that it is difficult to analyze cerebral blood flow in Moyamoya disease by ¹³³Xe intracarotid injection method and that EMS is more reasonable than STA-MCA anastomosis for the purpose of increasing external and internal collateral circulation.

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To evaluate causes of anesthesia induced acute neurological symptoms in children with “Moyamoya” disease, we studied the relationships between internal jugular oxygen tension (PjO₂) and arterial carbon dioxide tension (PaCO₂), and PjO₂ and mean arterial blood pressure (MAP) during diazepam-fentanyl (mNLA) or halothane anesthesia (GOF) for ST-MC anastomosis, EMS or CAG.
The linear regression equation, PjO₂=0.971×PaCO₂-0.703 (r=0.789, p<0.01), was obtained for PjO₂and PaCO₂during mNLA. However no significant relationship between PjO₂and MAP was recognized during mNLA. The equation, PjO₂=0.949×PaCO₂-0.022 MAP+2.682 (R=0.572, p<0.01), was obtained by multiple regression analysis for mNLA. During GOF the equation, PjO₂=1.064×PaCO₂+2.603 (r=0.803, p<0.01) and PjO₂=0.308×MAP+16.631 (r =0.861, p<0.01) were obtained respectively. Multiple regression analysis revealed the equation, PjO₂=1.103×PaCO₂+0.111×MAP-9.618 (P=0.741, p<0.01), during GOF.
These findings suggest that hypocarbia is one of the important factors which deteriorate neurological symptoms during anesthesia in “Moyamoya” disease and moderate hypercarbia should be maintained during anesthesia, especially in mNLA. During GOF hypotension, as well as hypocarbia, is a deteriorating factor.

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Microsurgical anatomy of the middle cerebral artery, important for extracranial-intracranial bypass surgery in the Moyamoya disease, was studied in 50 hemispheres removed at autopsy, using 3-20 X magnification. The lateral surface of the brain was divided into 12 areas as proposed by Salamon and Michotey. Special attention was directed to the relationship of the branches to cerebral cortex and Sylvian fissure. The largest cortical branches found were the temporo-occipital and angular arteries. The distance of each vessel's superficial course, important factor for anastomosis, averaged more than 12mm and there was no regional difference. The posterior end of the Sylvian fissure, where suitable vessels of sufficient intraluminal diameter and length to permit bypass surgery are ordinarily available, was 6.6cm on average above the external auditory meatus. Using the cantho-meatal line and the standard points obtained from the results, a new, simple, and accurate method for approaching each vessel of 12 areas is proposed. The implication of these findings for bypass surgery for Moyamoya disease is reviewed.

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Adult cases of moyamoya disease are often admitted as stroke. The purpose of this paper is to clarify the clinical features of moyamoya disease admitted as stroke comparing those of other cases without abnormal vascular networks.
In our hospital, during last five years, 1426 strokes were admitted and examined by cerebral angio-graphy and computerized tomography, the letter of which was omitted in some cases. Among the 1426 cases, there were 9 cases of moyamoya disease comprising 3 males and 6 females. Five cases had attacks of cerebral infarction and 4 cases had those of intracerebral hemorrhage. The age of 9 cases ranged from 30 to 59 years old (47.1 years in average), which was younger than ordinary cases in stroke. All cases of moyamoya disease with cerebral infarction had two or more attacks, while only 20% of the ordinary cases of cerebral infarction repeated attacks. The mortality of the 9 cases was 44% which was almost the same as ordinary cases of stroke and higher than that ever reported.
In consequence, the clinical features of adult cases of moyamoya disease admitted as stroke are almost the same as that of ordinary stroke except for the younger onset and repeating of attacks in cases of cerebral infarction.

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We have experienced 100 cases of Moyamoya disease these 18 years. Symptoms due to cerebral ischemia such as motor disturbance, mental handicap and so on were observed in 31 (85%) out of 46 children cases and in 17 (37%) out of 54 adult cases. Perivascular sympathectomy (PVS) and superior cervical ganglionectomy (SCG) were performed on these cases in order to increase the cerebral blood flow.
As a result, improvement of symptoms were observed in 19 (61.3%) out of 31 children cases and 8 (47.1%) out of 17 adults cases without any particular complications. These results are relatively good compared with those of intracranial surgical procedures such as STA-MCA anastomosis, encephalo-myo-synagiosis and encephalo-duro-arterio-synangiosis, which are very often performed in Japan recently.
Therefore, we advocate that PVS and SCG should be the first choice for the surgical treatment in Moyamoya disease. If no improvement is observed in spite of surgical operation at the neck, intracranial procedures such as STA-MCA anastomosis, encephalo-myo-synangiosis and so on might be available.

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As a mean of treating pediatric moyamoya patients, we have developed an operative procedure termed“encephalo-duro-arterio-synangiosis (EDAS) which consists of making a narrow craniotomy through which to transplant and suture a partial, free stem of the scalp artery attached with a strip of galea onto linear cut wound made in the dura mater, for the purpose of assisting anastomosis formation between the extracranial and intracranial arteries, which we apply on pediatric moyamoya patients and have achieved improvement of blood flow in the brain and general conditions.
We used the EDAS procedure on 9 pediatric cases and gained good results, and part of the results were reported at the 39th congress of the Japan Neurosurgical Society.
We had a chance of application of EDAS to an adult patients, which we are reporting hereunder.
The case was a 20-year-old female, who developed this disease with onset of subarachnoid hemorrhage. Three months after the onset of the disease we conducted EDAS using the left superficial temporal artery and now the patient is in the firth month after the operation.
According to our observations in children up to this time, the progress of revascularization of the brain by EDAS seems to follow the following steps (1) Thickening and increase of dural arteries. (2) Thickening of donor scalp artery. (3) Visible spontaneous anastomoses formation between the donor scalp artery and cerebral blood vessels. (4) In accordance with the thickening of the donor scalp artery, the abnormal vascular network at the bettom of the brain (moyamoya vessels) shrink away.
In this case, at 1.5 months after the EDAS, the above condition (1) was observed when the cerebral angiography was done, and at 4 months, the above (3) was seen partially formed. In view of these facts, we conclude that EDAS can be at least an alternative procedure of EC-IC bypass method in adult cases as well as in pediatric cases of moyamoya disease.

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The authors experienced 12 operative cases of moyamoya disease and the following conclusion was obtained.
A) As to indication of reconstructive operation:
1. Best indication: TIA or RIND or completed stroke with minor neurological deficits
2. Preferable indication : Cerebral hemorrhage and/or intraventricular hemorrhage with minor neurological deficits
3. No indication at the present time: Incidentally discovered, headache only, seizure only, mental retardation in adults
B) As to ideal operative methods:
STA-MCA bypass with encephalo-myo-synangiosis seemed to be the most preferable operative methods. In addition, usefulness of encephalo-arterio-synangiosis (EAS) as a supplemental operative method was emphasized.

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Twelve Patients of so-called moyamoya disease were treated surgically. The surgical procedure were STA-MCA bypass, encephalo-myo-synangiosis (EMS) and omentum transplantation. Eighteen operations were carried out. The follow-up period ranged seven months to four years. The results were as follows; one complete recovery from neurological deficit, three improvement, four unchanged and two worse. Disappearance of TIA were observed three out of fifteen. Decrease of TIA were observed three out of fifteen. Overall results suggest that the surgical treatment of so-called moyamoya disease is promising. Very low cortical arterial pressure of the moyamoya disease suggests the importance of performing multiple anastomosis combined with EMS or omentum transplantation. Also surgery should not be indicated when the patient is in the acute infarction. This may be avoided by frequent examination by CAT.

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The pre-, intra-, and postoperative hemodynamics were examined by a bidirectional ultrasonic Doppler flowmeter with sound-spectrograph in 12 patients of moyamoya disease undergoing 14 STA-MCA anastomoses, 2 encephalo-myo-synangioses and/or 2 omentum transplantation. In the preoperative transcutaneous flow measurement, flow pattern of bilateral STAs was the internal carotid type in 8 cases. In the three of them, apparent transdural anastomoses by way of STA were revealed by preoperative angiography. In the intraoperative direct flow measurement before anastomosis, flow was easily detected in the cortical branches of the MCA in 8 of 12 STA-MCA anastomoses. The prominent flow changes in the cortical branches of MCA with on-off clipping of the STA, namely the flow increase in distal to and the decrease or reverse of flow in proximal to anastomosis, were detected in 4 of 10. Postoperative bypass function evaluated according to the results of transcutaneous Doppler flowmetry of STA was good in 9 of 14 STA-MCA anastomoses. The results of intraoperative studies were not in agreement with those of intraoperative studies in 4. Bypass function in patients of moyamoya disease was relatively poor and labile in comparison with those in patients with the other cerebrovascular occlusive disorders.

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This is a report of a follow up study of 26 bypass operations on 14 patients done since April of 1978. Eleven patients showed improved neurological status after operation. Decreased Moyamoya mass and the incresaed size of the superficial temporal artery were shown angiographically with the lapse of time. Good filling of the middle cerebral artery was also demonstrated 10 to 12 months postoperatively. It may be supposed that the bypass surgery in the early stage of Moyamoya disease will produce a good result.

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The extracranial-intracranial arterial anastomosis was performed on two cases of cerebrovascular Moyamoya disease. One case is child and another case is adult. All excellent results were obtained.
The extracranial-intracranial arterial anastomosis with encephalo-myosynangiosis (EMS) was performed on the adult case. Frequency of SAH was decreased and left hemiparesis was improved on adult case. The child case showed an improvement of right hemiparesis of RIND type and motor aphasia. SEP, EEG and rCBF showed good results.

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Last seven years we operated on four cases of moyamoya disease, one of which was the ischemic type and three the hemorrhagic ones.<br[I] Left STA-MCA anastomosis and encephalomyosynangiosis were performed on a three year-old girl with six times of TIA (case 1). This case developed total aphasia postoperatively. The postoperative CT scan showed bilateral cortical infarctions and left thin subdural fluid collection. Afterthere this case has been aphasic for two years. We thought that low PaCO₂ (below 35mmHg) induced by the hyperventilation and the operative sacrifice of the middle meningeal artery were main causes of these cortical infarctions and aphasia. [II] Total removal of intracerebral hematomas were performed in three cases of adults (case 2, 3 and 4). All intracerebral hematomas were on the left side of the hemisphere. Ventricular ruptures were present in case 2 and 3. The postoperative conscious level was III-1 in case 2 and 4, and II-2 in case 3. Postoperative CT scan showed low density areas located in the subcortical areas but sparing the basal ganglia in case 2 and 3, while the low density area located within the basal ganglia in case 4. The follow-up period was 2Y3M in case 2, 2Y6M in case 3 and 6M in case 4. The outcome was Grade I in case 2 and 3, and Grade III in case 4. We thought that difference of ADL between case 2, 3 and 4 chiefly depended on the location of the intracerebral hematoma. In conclusion
In case of the operation for the moyamoya disease, (1) the hypocapnea should not be induced and the transdural anastomosis should not be sacrificed, particularly in the ischemic type, and (2) the early removal of intracerebral hematoma should be done whenever the patient's consciousness deteriorates progressively.
* A. D. L.: classified by Kanaya et al.

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The authors have analized morphological changes of cerebral vasculature following STA-MCA anastomosis performed on 4 adult cases with Moyamoya disease. The analysis was made by repeated angiography following the anastomosis. The first follow-up angiography was made 14 to 16 days after the surgery and the second ones were performed 6 to 24 months later.
In postoperative follow-up angiography, the authors have recognized disappearance or remarkable decrease of “Moyamoya” vessels at the base of the brain and remarked dilatation of the anastomosed superficial temporal artery.
On the basis of these findings, the authors believe that the STA-MCA anastomosis not only increases the cerebral blood flow but also it should have a preventive effect on hemorrhage from the abnormally dilated collateral blood vessels, which is the largest cause of death in this disease.

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Pathophysiology of moyamoya disease is still obscure. In recent years, surgical treatments for this disease have been reported by several authors, especially for the case with ischemic attacks. Five cases (4 children, 1 adult) were reported in correlation with the changes in postoperative angiographical findings. In four cases STA-MCA anastomosis was performed on both sides and in one case encephalo-myo-synangiosis was made bilaterally. Representive cases are as follow.
Six year-old boy presented with motor aphasia and right hemiparesis on admission. Four months prior to admission he experienced progressive left hemiparesis which improved remarkably thereafter. The STA-MCA anastomosis was performed bilaterally. Right carotid angiogram revealed improved cerebral circulation over the territory of right middle cerebral artery and diminished moyamoya vasculatures one year after the surgery.
Six year-old girl showed progressive left hemiparesis which was remaining slightly on admission. STA-MCA anastomosis was performed on both sides. Despite good circulation through bilateral STAs was noted in angiograms made 6 weeks after the operation, angiograms made one year after the surgery revealed poor patency of anastomosis and major blood supply to the territory of middle cerebral artery seemed to be through middle meningeal arteries which became prominent. Although stenosis of C-1 segment of both internal carotid arteries was aggravated and the artery was completely occluded at C-2 segment bilaterally, moyamoya vasculatures were diminished.
Development of anastomosis between meningeal arteries and cerebral arteries was postoperatively in 9 out of 10 cerebral hemispheres, irrespective of the patency of STAs. Preoperative symptoms were improved in all cases except for motor aphasia in one case. Ischemic attack has never been observed in all cases during the follow-up period for 5 to 24 months.
In view of the diminution of moyamoya vasculatures and improvement of symptoms, the surgical treatment of moyamoya disease seems to be justified to prevent further ischemic attacks.

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Ten surgical cases of Moyamoya disease are discussed in this report. EC-IC bypass (STA-MCA anastomosis and encephalo-myo-synangiosis) were performed on seven cases. Five cases were cerebral ischemia and two were intracerebral or intraventricular hemorrhage. For the other three cases, another operation was performed. Several months after EC-IC bypass, excellent filling from anastomosis was seen and Moyamoya networks were disappeared in all cases. We discuss the effect of EC-IC bypass not only for the cerebral ischemia but also for the intracerebral or intraventricular hemorrhage of Moyamoya disease.

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As far as we know, an angiographical early venous filling (deep and cortical), red cortical vein, intraarterial pressure and pre- and postoperative CBF values in Moyamoya disease are not reported. We discussed about these points respectively.
(1) Angiographical early venous filling
In Moyamoya disease it was thought that the blood circulation time in the brain was slow, therefore the veins of the brain appeared very late angiographically. But we observed this time in our Moyamoya cases the angiographical early venous filling of the internal cerebral vein, the basal vein of Rosenthal and the ascending cortical vein. In Moyamoya brain there must be some parts where the blood circulation time is rather fast.
(2) Red cortical vein On a cortical surface, we observed the increase in number of pial vessels and red cortical veins in which the arterial and venous blood flowed forming a laminar flow. These findings were thought to be characteristic in Moyamoya disease. As the mechanism of the red cortical vein, there might exist an arteriovenous shunt, be based on luxury perfusion in ischemic regions, or reduction of O₂ consumption in Moyamoya brain.
(3) Intraarterial pressure
In Moyamoya disease the proximal stump pressure of the MCA was very low due to the stenosis or occlusion of the terminal portion of the internal carotid artery, and the distal stump pressure of the MCA was rather high that means the relative development of leptomeningeal anastomosis.
(4) CBF values
After the extra- and intracranial bypass surgery, the Mean rCBF and the gray matter flow (Fg) which were lower preoperatively became high, and the white matter flow (Fw) which was higher preoperatively became low. This postoperative decrease of the Fw might be parallel that the Moyamoya vessels were reduced in number angiographically after the bypass surgery. These findings might mean that the bypass surgery could be a treatment not only for cerebral ischemia but also for intracranial bleeding in Moyamoya disease.

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