Twelve patients with Moyamoya disease were treated by Encephalo-Myo-Synangiosis (EMS) for the purpose of increasing external and internal collateral circulation. Fourteen EMS were performed in 10 patients, and 3 EMS and STA-MCA anastomosis were performed simultaneously in 2. The ages of the patients ranged from 7 to 46 years, and 9 patients were under 16-year-old. Their clinical manifestations were caused by the cerebral ischemia.
They were followed for 4 to 20 months. Decrease of TIA and improvements of paresis, ataxia, involuntary movement and dysarthria were obtained markedly in most of the young patients. But there were little improvements in the adult patients.
Postoperative external carotid angiogram showed good visualization of the middle cerebral arteries via the dilated deep temporal artery in most of the patients. Increased collateral circulation via the deep temporal artery after EMS seemed to be equal to that via STA after STA-MCA anastomosis. Abnormal vascular networks in the region of the basal ganglia were reduced in most of the patients underwent EMS.
Improvements on EEG and cerebral blood flow measured by ¹³³Xe inhalation method were also obtained in most of the patients.
EMS is considered to be enough for surgical treatment of Moyamoya disease with symptoms by the cerebral ischemia, especially in the young patients.

抄録全体を表示

Thirty cases of Moyamoya disease were reported. Six cases of them were surgically treated.
The external intracranial arterial anastomosis were performed in 4 patients with Moyamoya disease. Repeated angiography showed excellent patency of anastomosis. This is no doubt that the creation of STA-MCA anastomosis increases blood flow and thus prevents cerebral ischemia due to occlusion of the circle of Willis, in addition, preventive effect of rebleeding was noted even in cases with intracerebral hematomas.
Four autopsy cases and two cases complicated cerebral aneurysm and renovascular hypertension were discussed in special reference to the pathogenesis of this disease.

抄録全体を表示

An investigation was made for indication and timing of the operation on the basis of cerebral angiogrm, CT findings and pre- and post-operative clinical symptome. Eight infantile cases out of eighteen cases of occlusion of the circle of Willis were operated on at our university hospital from 1977 to 1980.
(Result) Infantile cases of occlusion of circle of Willis were classified into 3 types.
Type 1 means eary stage of occlusion of circle of Willis. Occlusive lesion is still confined to the anterior half of the Circle of Willis and development of moyamoya vessels and collateral circulation is incomplete.
Type 2 means typical case of occlusion of circle of Willis having moyamoya vessels and transdural anastomosis. Transit type means cases like adult type.
Case of type 1 with operation (STA-MCA anastomosis+EMS) showed good improvement of clinical symptomes immediately after operation, and marked increase of blood flow to MCA teritory by postoperative cerebral angiography.
On the other hand, cases of Type 1 without operation become Type 2.
Case of Transit type were not improved well by operation.
Therefor timing and indication of operation to occlusion of circle of Willis are good on the stage of Type 1.

抄録全体を表示

Surgical treatments were performed on 10 cases with moyamoya disease. Superficial-temporal cerebral artery (STA-MCA) anastomosis was done for 3 children suffering from transient ischemic attacks (TIA's). All patients have become free from TIA's after surgery. STA-MCA anastomosis was also performed on 2 adult patients for prevention of re-bleeding and re-ischemic attack's. These patients were unchanged postoperatively. This anastomotic procedures is most effective for TIA's and may be effective for prevention of bleeding from moyamoya vessels. Two patients with subcortical hematoma were treated by craniotomy or trephination and had good result. Intracerebral hematoma which showed mass sign should be evacuated by small craniotomy not to injure collateral circulation. Ventricular drainage was performed in two hopeless cases and not effective for these patients. This procedure may be not so effective for infraventricular massive hemorrhage. A case of chronic subdural hematoma with moyamoya disease was treated by burr hole and irrigation.

抄録全体を表示

An 11-year-old boy of moyamoya disease was treated the STA-MCA anastomosis for preventing TIA's of the right hemiparesis and aphasia. This case was follow-up studied pre- and post-operatively using with the vascular imaging with ^99mTc labelled red blood cells (Tc-RBCs).
The abnormal high radioactivity at the cranial base representing the moyamoya vessels seen in the pre-operative Tc-RBCs angiography (Fig. 1) was significantly decreased in the post-operative photos (Fig. 2). The radioactivity at the left cerebral hemisphere seen in the pre-operative static photos was markedly decreased after operation (Fig. 3). The cranial blood volume index (CBVI) was obtained as the cerebral blood volume. The static image processed in the magnetic disc, is displayed on CRT and the regions of interest (ROIs) are setted at the cerebral hemisphere. The radioactivity of ROI was studied using with a minicomputer. The activity of 1 ml of peripheral venous blood was counted by the same method. CBVI was calculated, by the equation:
The CBVI of the operated side was decreased, 9.5 to 6.8, less than the non-operated side, 8.7 to 7.9, after the operation. The radioactivity at the anastomosed hemisphere was decreased because that the vascular bed of the moyamoya vessels and medullary vessels was made less after the operation.
These findings suggest that the STA-MCA anastomosis may be useful for prevention of TIAs and bleeding from the moyamoya vessels.

抄録全体を表示

1. Twenty-nine patients with “Moyamoya” disease underwent ST·MC anastomosis and encephalo-myo-synangiosis. The duration of follow-up study after surgery was ranged from 3 years 6 months to 6 years 9 months (mean 4 years 5 months). 2. Motor weakness, sensory disturbance, speech disturbance and involuntary movement were observed preoperatively in 25 cases, 9 cases, 13 cases and 3 cases respectively, but these neurological deficits disappeared postoperatively in 5, 6, 8 and 1 cases respectively. Though the deficits remained in the other cases, these improved markedly in the most of the cases. 3. The disturbance of intelligence was observed in 13 cases preoperatively. In 4 of them, it disappeared postoperatively. Only 2 cases had less than 70 of I.Q. (Intelligence Quotient) and 6 cases had more than 80 of I.Q. 4. Regarding to homonymous hemianopsia, visual disturbance and disturbance of swallowing, the operation was non-effective. 5. As to the operative result, 11 cases recovered completely, 14 improved remarkably, 1 improved slightly, and 2 adult cases were unchanged and 1 case died of myocardial infarction one year after surgery. 6. All the cases were classified by ADL: 13 cases; excellent, 8 cases; good, 6 cases; fair, 1 case; poor and 1 case; dead.

抄録全体を表示

1. It was described of sequential changes of angiography in 8 cases with “Moyamoya” disease followed up over 3.5 years after ST·MC anastomosis. 2. One month after surgery, a few cortical branches of MCA were observed by external carotid angiography. Three months after surgery, whole cortical branches of MCA were visualized through both surgical anastomosis and spontaneous anastomosis of middle meningeal artery. 3. Concerning the changes of point of anastomosis, fine vascular network appeared about 6 months after surgery, and the anastomosis was getting to obstruct one year after surgery. Afterward, cortical branches of MCA were opacified through the fine vascular network produced at the region of anastomosis. 4. The basal moyamoya vessels decreased in volume and density about 3 months after surgery, and unchanged since one year after surgery.

抄録全体を表示

Cerebral hemodynamic studies were made in 36 child cases of “Moyamoya” disease, using Technetium-99m and a Gamma scintillation camera. About 10mCi ^99mTc was injected into the anterior cubital vein as a bouls, and serial brain scintigrams in one second interval were obtained with Gamma scintillation camera (Ohio-nuclear Σ410t) and processed on an on-line radioisotope processing system including microcomputer (DEC PDP 11/34, 11/60 GAMMA 11).
Results were as follows.
1. Study of serial brain scintigraphy
The characteristic images were obtained in preoperative cases: early accumulation of radioisotope-activities in the base of the skull, corresponding to the basal “Moyamoya” vessels, and the poor filling in the area of anterior and middle cerebral artery, indicating disturbance of the cerebral circulation. After surgery, early accumulation of RI activities in the base of the skull were decreased and the evidence of improved cerebral circulation in the territories of the MCA was obtained, but poor filling in the territories. of the ACA persisted.
2. Measurement of local mode of transit time (MOTT)
Quantitative analyses were made by our modified method which was orginally reported by Ordendorf. i) Regions of interest (ROI) were settled in the area of basal “Moyamoya” vessels and in the territories. of MCA on the image of A-P view. The mean values of MOTT in the MCA territories was significantly longer than that in the basal “Moyamoya” in preoperative cases (10.2±2.8, 7.6±2.5sec., n=28, respectively). After surgery, shortening of MOTT in the MCA territory was observed (7.2±1.7sec.,n=22). ii) ROI were also settled in the area of basal “Moyamoya”, frontal part, parietal part and occipital part on the image of lateral view. The mean values of MOTT in the area of basal “Moyamoya” was significantly different from those in the areas of parietal and occipital parts in preoperative cases (7.1 ± 1.8, 10.2±3.0, and 10.0±3.8 sec., n=15, respectively) After surgery, shortening of MOTT in the areas of parietal and occipital parts was also observed (6.8±1.4, 6.8±1.5 sec., n= 12, respectively).

抄録全体を表示

The authors studied the sequential changes of cerebral blood flow and cerebral metabolism after surgical treatment in“moyamoya”disease. The CBF studies were made in 21 patients, 28 sites with“moyamoya”disease; ST·MC anastomoses combined with or without encephalo-myo-synangiosis (EMS) in 16 patients, 18 sites, and EMS only in 9 patients, 10 sites, The measurement of CBF were made by use of Argon and medical massspectrometry, according to the Fick's principle.
CBF increased in 2 days after operation, followed low CBF values in a month later, But CBF met with good results at over 3 months after operation, not only in ST·MC anastomosis group, but also in EMS group. CMRO2 decreased in 2 days after operation in the patients with postoperative transient neurological deficits, then, increased to high values compared with preoperative values in accord with the disappearance of postoperative neurological deficits
These CBF results supported that ST·MC anastomosis and EMS for“moyamoya”disease are effective.

抄録全体を表示

We investigated coagulability of blood and fibrinolysis before and after ST·MC bypass in 25“Moyamoya”patients.
Six of 25 cases deteriorated postoperatively but improved about 10-14 days after surgery.
In the 6 cases, plasminogen in the blood decreased pre-operatively and continued for about 5 days, and fibrinogen remarkably increased for about 10 days after surgery.
These facts suggest that the increase in fibrinogen would cause diminution of the cerebral microcirculation induced by the raised blood viscosity.

抄録全体を表示

EEG changes were investigated in 30 children with“Moyamoya”disease with special reference to surgical vascular reconstruction.
1) The most characteristic EEG finding of the children with“Moyamoya” disease was a delayed slowing after hyperventilation, which was seen in 22 patients out of 30. This phenomenon seemed to originate from the basal“Moyamoya”vessels.
2) The worse findings of emergent EEG monitor by BERG analysis consisted of slowing of the basic activity, decrease of the power density and laziness of the sleep spindle. They appeared on the next day of the reconstructive surgery and continued for a week, which correlated with the changes of clinical symptoms.
3) Expected EEG monitor showed worse findings a month after surgery. However, it revealed improvement of the basic rhythm and back ground activity, and disappearance or marked decrease of paroxysmal slow wave and delayed slowing after hyperventilation, three months after surgery.
4) Surgery for another side should be performed over three months after the first operation.
5) According to these results, surgical vascular reconstruction was effective for“Moyamoya”disease.

抄録全体を表示

Somatosensory evoked potentials to median nerve stimulation were examined on 16 children of“Moyamoya”disease and 9 normal children. In 16 children of“Moyamoya”disease, 6 children were examined before ST·MC anastomosis, and other 10 children were done more than 3 months after bilateral anastomosis.
In normal children, SEP waves consisted of 3 negative waves (N17: 17.1±1.3msec. N32: 31.9±3.9msec. N55: 55.4±7.0msec.) and 3 positive waves (PH: 11.4±1.5msec. P24: 24.2±2.1msec. P42: 41.8±3.6msec.). In children with“Moyamoya”disease, latency of early component (P11-N17) were slightly shorter than in normal children. In 10 children with bilateral ST·MC anastomosis, latencies of P11-N32 and P11-N55 were significantly shorter than in 6 preoperative children.
Negative peak amplitudes were compared without operation to with bilateral anastomosis, only N17 peak amplitudes in bilateral operated group were significantly higher than in pre-operated group.
Then SEP were examined following hyperventilation in“Moyamoya”disease. In several minutes after hyperventilation, amplitude of N55 peak were decreased abruptly in the cases of“Moyamoya”disease with TIA, who has not been done with ST·MC anastomosis. However, when operation has been done, amplitude of N55 peak has not been decreased yet after hyperventilation.
These results indicated that ST·MC anastomosis were effective in cases of“Moyamoya” disease with TIA.

抄録全体を表示