A case of so-called “moyamoya” disease with intracerebral hematoma is reported. This 39-year-old man was transfered from another hospital to Kitasato University Hospital on May 24, 1977, because of disturbance of consciousness and left hemiplegia. He was well until 7 days prior to admission, when he suddenly noticed severe headache and weakness of the left arm and leg. 3 days prior to admission, he became stuporous.
On admission, he reacted only to painful stimuli. The left pupil was 4 mm in diameter while the right was 3 mm. The both pupils reacted to light briskly. Deep tendon reflexes of the left upper and lower extremities were markedly increased. The Babinski's sign was positive on both sides.
Right carotid angiogram showed occlusion of the supraclinoid portion of the internal carotid artery. There was an abnormal vascular network, which had characteristics of that of “moyamoya” disease, just above the site of occlusion. Through the abnormal vascular network, ascending branches of the middle cerebral artery were faintly opacified. The anterior cerebral artery was opacified via the posterior pericallosal artery and deviated to the left. The posterior communicating and posterior cerebral arteries were dilated and displaced downwards.
The computed tomography showed a large hematoma located in the right putaminal region. On May 25, right frontoparietal craniotomy was performed and the intracerebral hematoma was evacuated. An anastomosis between the superficial temporal artery and the cortical branch of the middle cerebral artery (STA-MCA anastomosis) was performed at the same time. The patient made a remarkable recovery following the surgery. His consciousness became normal within 3 days. The left-sided motor palsy also showed marked improvement. When seen 6 months after the operation, he had only mild residual weakness of the left hand and resumed a full-time work as a business man.
Repeated angiography (Nov. 14, 1977) showed excellent patency of the anastomosis, with extensive irrigation of the right middle cerebral territories through the anastomosis. The “moyamoya” vessels were almost completely disappeared at this time.
There are two mechanisms to develop clinical manifestations in the so-called “moyamoya” disease. One is cerebral ischemia due to occlusion of the major vessels at the base of the brain. The other one is hemorrhage which is considered to be from rupture of the fragile thin-walled “moyamoya” vessels or from aneurysms arised from these vessels. The former mechanism is mainly observed in younger age group and the latter is seen in adult cases.
There is no doubt that the creation of STA-MCA anastomosis increases blood flow and thus prevents cerebral ischemia to a certain extent in this condition. At the same time, the creation of new route of blood supply results in decreasing necessity of the “moyamoya” vessels which have been considered to be markedly dilated small collaterals. As a fact, in the present case, the “moyamoya” vessels have almost completely disappeared following the placement of the STA-MCA anastomosis as proved by follow-up angiographic studies. By decreasing blood flow through the “moyamoya” vessels by this surgery, the authors believe that the chances of hemorrhaging from these abnormal vessels will be markedly decreased.

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At present available data concerning a relationship between cerebral hemodynamics and functional prognosis in cerebrovascular disease are meager and the results are inconsistent.
An attempt was made to correlate long-term functional prognosis with cerebral hemodynamics and oxygen consumption in patients with ischemic cerebrovascular disease.
In 46 patients with ischemic supratentorial cerebrovascular disease, cerebral blood flow (CBF) and cerebral metabolic rate of oxygen (CMRO₂) were measured by the N₂O method one to six months (mean, 62.7 days) after the onset of the disease. Two or more years after the measurement, the physical condition of the patients was evaluated by the questionnaire method. The patients were grouped according to functional status in walking, sphincter function, speech, and handling of chopsticks. Mean values for CBF and CMRO₂ in patients with good functional recovery were, higher than those in patients with poor recovery. In particular, walking and sphincter function showed a significant relationship to either CBF or CMRO₂. Multivariate analysis (quantification theory group I) of the data revealed that the value of CBF or CMRO₂ was most closely related to the sphincter function of the patients.
The results suggest that CBF and CMRO₂ values measured after acute stage are useful for estimating the long-term functional prognosis of patients with stroke.

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The special stroke prophylaxis program had been conducted in 12 prefectures of Japan during 3 year period, but the effect has not yet been evaluated.
We attempted to evaluate it retrospectively as a reference for future community-based hypertension control programs. Cities, and towns and villages in the same prefectures were grouped to constitute two sets of control areas for grouped program areas of the 12 prefectures. Mortality rates for all causes and those for a stroke were calculated for men and women aged 45 to 64 in the 3 areas in the 12 prefectures between 1969 and 1975.
1. Total and stroke mortality rates decreased remarkably in both the program and the control areas. The declining trends were stronger in women.
2. The ratio of stroke mortality to total mortality declined both in the program and the control areas.
3. The amount of reduction in stroke mortality rates was larger in the program areas than in the control areas. However, the amount of decrease common to the program and the control areas was much larger than the net decrease to be interpreted as the effect of the special stroke prophylaxis program.

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Although arteriography is the definite study for assessment of cerebral circulation, the time, risk and patient's discomfort make its use as a screening test impractical. Recently, a variety of noninvasive diagnostic methods have been developed to overcome such difficulties.
In the present study, oculoplethysmography (OPG) and carotid phonoangiography (CPA) were applied as the noninvasive methods to evaluate the accuracy for detecting the extracranial carotid occlusive disease in 73 patients who underwent bilateral carotid arteriography.
The delay of ipsilateral ocular pulse wave relative to contralateral side was taken as an abnormal OPG finding, suggesting the presence of a significant internal carotid occlusive lesion. CPA was used as a supplementary test to support the OPG findings and to discriminate the complete carotid occlusion from the stenotic lesion.
Among 42 patients diagnosed as “normal” by OPG and CPA, 19 patients showed no internal carotid lesion at all and 19 had stenosis less than 50%. Of the remaining four patients, one had stenosis greater than 50% and three showed complete occlusion of the internal carotid artery distal to the ophthalmic artery.
In 31 patients with abnormal OPG with or without CPA abnormalities, complete occlusion of the internal carotid artery was found in 17 (55%), stenosis of 50% or greater in 8 (26%), stenosis less than 50% in five, and no stenosis was present in one case. Differentiation of complete occlusion from marked stenosis of the carotid artery was not always easy, but the absence of bruit in CPA strongly suggested a presence of complete occlusion.
Major disadvantage of the present tests is incapability of detecting mild degree of stenosis, especially ulcerative plaques at the carotid bifurcation which is thought to be a potent source of microemboli, although it is a common problem in most noninvasive diagnostic devices. However, once OPG is abnormal, the patient has more than 80% possibilities of having either a complete occlusion or a marked stenosis of greater than 50% in the carotid artery. When OPG and CPA are both normal, more than 90% of the patients have no or insignificant stenotic lesion.
No complication was observed in any patients who underwent OPG and CPA.
As a combination of these two tests are easy to perform, universally applicable without producing patient's discomfort, and have extremely low risk of complication and relatively high diagnostic accuracy, it is concluded that OPG and CPA are useful noninvasive techniques for screening the candidates of carotid arteriography and for the long-term follow up of the patients with carotid occlusive disease.

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A case with lactic acidosis following rupture of cerebral aneurysm is presented, since lactic acidosis associated with subarachnoid hemorrhage has not previously been reported in the literature.
A 41-year-old male was brought to Tokai University Hospital because of sudden loss of consciousness 3 hours prior to admission. On admission, his respiration was 32/min and regular. Blood pressure was 90/60 mmHg. Fundoscopic examination showed preretinal hemorrhage and flaccid paralysis was observed in the four extremities. Deep tendon reflexes and plantar response had disappeared and he did not respond to painful stimuli. The cerebrospinal fluid was bloody and blood gas analysis showed metabolic acidosis (pH 7.292) with elevated blood lactate level. A diagnosis of lactic acidosis with subarachnoid hemorrhage was made. Intravenous administration of sodium bicarbonate was effective, and the arterial pH and lactate level returned to normal within 2 days, followed by improvement of vital signs. The value of serum amylase, which was normal on admission and on the 4th hospital day, suddenly increased up to 2560 SR/dl on the 5th hospital day. His condition gradually deteriorated and he died 9 days after the onset of illness.
Autopsy revealed severe subarachnoid hemorrhage, particularly around the anterior communicating artery and localized pancreatitis on the pancreas head which was assumed to have occurred on the 5th hospital day.
The possible causes of clinical lactic acidosis are discussed. However, these factors appear not to have contributed to this case, although it is difficult to exclude the possibility that acute hypoperfusion caused lactic acidosis in this case. As it has been shown by some investigators that blood lactate level is elevated significantly in patients with head injury, it is concluded that the lactic acidosis in this case was mainly attributable to the subarachnoid hemorrhage itself.

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The influence of sympathetic nervous activity on the cerebral circulation and cerebrovascular CO₂ reactivity were investigated through the inhibition of dopamine-beta-hydroxylase (DBH), the final enzyme in the biosynthesis of norepinephrine.
1) Intravenous infusion of fusaric acid (a potent DBH inhibitor) increased cerebrocortical PO₂ and CBF shortly after the administration, with no significant changes in cerebrocortical PCO₂. The mean arterial blood pressure decreased in a few minutes after infusion of fusaric acid.
2) The increase in cerebrocortical PO₂ and CBF during 5% CO₂ inhalation was significantly augmented after DBH inhibition with fusaric acid. There were no significant differences in the changes of arterial blood gases and pH during CO₂ inhalation before and after DBH inhibition. The increase of arterial blood pressure during CO₂ inhalation was reduced after DBH inhibition.
3) The decrease in the cerebrocortical PO₂ and CBF during hyperventilation was slightly attenuated after DBH inhibition, which was not statisticaly significant.
The cerebral vasodilatation caused by fusaric acid and augmented cerebrovascular CO₂ reactivity after DBH inhibition suggest that the sympathetic nervous system influences cerebral vascular tone and plays a role in modulating chemical vasomotor activity.

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A 36-year-old housewife was admitted to the hospital, because of consciousness disturbance. She had no particular past history with the exception of measles at the age of four years.
On admission, she was comatose and neurological examinations revealed left hemiplegia with decerebrate rigidity. Her blood pressure was 130/80 mmHg. Physical examinations were otherwise unremarkable. The CT scan demonstrated massive cerebral hemorrhage in the right hemisphere. A right common carotid angiogram showed occlusion of the internal carotid artery associate with abnormal vascular network at the base of the brain and occlusion of the external cerebral artery (Fig. 1 A B). She died on the third hospital day.
The main autopsy findings of the case were as follows :
1) Massive hemorrhage was observed in the right hemisphere. No particular histological change was obtained except that there were acute changes due to cerebral hemorrhage.
2) Cerebral vessels : The numerous abnormal small vessels were observed at the base of the brain, predominantly in the right. Bilateral internal carotid arteries were occluded macroscopically from their origins to terminal portions. The terminal portion looked like strings (Fig. 3). The superficial temporal artery and middle meningeal artery were also occluded. Histological examinations disclosed marked fibrous intimal thickening with fragmented internal elastic lamina and thinning of the media (Fig. 4 A B). No atheromatous deposits nor inflamatory cells were observed within those vessels. The anterior cerebral artery (A₁) was stenosed due to intimal thickening and the internal elastic lamina showed wave-like bending. The cortical and intraparenchymal vessels did not show significant change except for the abnormal small vessels in the base of the temporal lobe (Fig. 2).
3) Systemic arterial system : Relatively severe atheromatous changes for her age were observed especially in the arch and thoracic aorta. A localized ossification with the formation of bone marrow was observed in the lower part of the thoracic aorta (Fig. 6). The common histological features of the aorta and its branches were fibrous intimal thickening with atheromatous deposits, severe thinning of the media, fibrosis of the adventitia and marked concentric intimal thickening of he vasa vasorum (Fig. 5). The pulmonary arteries were also affected.
4) Visceral organs : Histological findings of the other organs were unremarkable except for scattered fibrosis in the myocardium.

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A clinicopathological study was performed on 45 cases of the elderly persons, who showed the pathological changes of progressive subcortical vascular encephalopathy of Binswanger type. The results were as follows :
1) Progressive subcortical vascular encephalopathy (PSVE) was observed in 3.8% of all autopsies of the elderly persons and consisted of 6.7% of the brains with cerebrovascular diseases. The grades of the white matter lesions were divided into I-III (slight to severe) for analysis. Small infarcts in the basal ganglia, thalamus and pons were usually observed, but cerebral cortex was generally preserved.
2) Neuropsychiatric symptoms were dementia, urinary incontinence, hemiplegia, pseudobulbar palsy, psychosis, parkinsonism, mutism, etc. Cases of grade III showed a pseudobulbar palsy, parkinsonism and mutism in high incidence. There were also NPH cases in this series.
3) Pathologically almost all cases showed a marked cerebral arteriosclerosis. Angionecrosis was observed in 60-80%. Fibrotic and stenotic changes of the blood vessels in the deep white matter were also remarked, particularly in 90% in the cases of grade III.
4) As the pathomechanism and etiologic factors of PSVE, it is considered that various complications, such as hypertension, cardiac disease, malnutrition, etc., may play an important role for PSVE, when they occurred in the elderly persons with a history of hypertension of long duration, marked cerebral arteriosclerosis and arteriolar changes in the white matter.

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