Yoshiharu Sakurai

Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: An extensive follow-Up study.

BACKGROUND AND PURPOSE Revascularization surgery for moyamoya patients is believed to prevent cerebral ischemic attacks by improving cerebral blood flow. However, measures preventing the occurrence of hemorrhagic moyamoya in patients have not yet been established in the literature due to the low rate of hemorrhage onset as well as the originally limited numbers of patients with moyamoya disease, poor understanding of the clinical course of rebleeding, correct surgical management, and long-term outcome. We present here the results of an overall survey of patients with hemorrhagic moyamoya disease in a district of Miyagi Prefecture in Japan and examine their clinical course, efficacy of revascularization surgery, and long-term outcome. METHODS This study included 28 moyamoya patients with episodes of intracranial hemorrhage between 1976 and 1988. The mean follow-up period was 14.2 years. There were 4 males and 24 females, aged 7 to 69 years (mean 39.2 years). Cerebral angiography and CT scans were performed for all patients. Surgical treatment was performed in 19 patients (67. 9%), and 10 patients (35.7%) underwent revascularization surgery. We observed the clinical course of all 28 patients. We also studied the relationship between the efficacy of surgical treatment and long-term outcome. RESULTS Five of the 28 patients (17.9%) died of the initial intracranial hemorrhage, and 2 patients died of other causes. Rebleeding occurred in 6 of the remaining 21 patients (28. 6%). The interval to rebleeding ranged from 2 to 20 years (mean 7.3 years). Of these 6 patients, 4 died of rebleeding. Rebleeding was observed in 1 of 8 patients who underwent bypass surgery and in 5 of 13 patients who did not, which suggested that rebleeding was less likely to occur in patients who had undergone bypass surgery. However, there was no significant difference in rebleeding ratio or mortality between patients with and those without revascularization surgery (P>0.05). CONCLUSIONS In this study, we compiled the results of meticulous follow-up conducted over the past 10 years for patients with hemorrhagic moyamoya disease. Because hemorrhagic moyamoya disease is known for its high rate of mortality at the time of rebleeding and often causes rebleeding long after the initial episode (as much as 20 years later), implementation of long-term preventive measures for rebleeding is necessary. This suggests that a long-term prospective study of a large number of patients with hemorrhagic moyamoya disease is required to determine whether bypass surgery prevents rebleeding of hemorrhagic moyamoya disease.

Subarachnoid Hemorrhage of Unknown Cause

After the institution of computed tomography (CT), 814 cases of spontaneous subarachnoid hemorrhage (SAH) were treated during a period of 6 years and 9 months (April 1978 through December 1984). In 9 (22.0%) of 41 patients whose cause of SAH was not determined by the first four-vessel study (cerebral panangiography), ruptured aneurysms were found by repeated four-vessel study. Thus, of 814 cases, only 32 (3.9%) were diagnosed as cases of unknown etiology at discharge. The 32 cases were monitored by follow-up examination for 5 to 67 months (median, 27.1 months) after onset. No recurrence of SAH was reported, and all patients were rehabilitated except 2 who suffered terminal carcinoma. A third follow-up four-vessel study was performed 8 to 44 months (median, 22.0 months) after the second study in 14 of the 16 patients with SAH initially demonstrated by CT. In 1 of these cases, an aneurysm found 9 months after the initial SAH was treated surgically. Ultimately, 31 cases (3.8%) were diagnosed as cases of SAH of unknown cause. This incidence is low when compared with those in previous reports. Because of the strict examination schedule including repeated angiography, the incidence is lower and the prognosis is relatively favorable.

Predominant Involvement of Ipsilateral Anterior and Posterior Circulations in Moyamoya Disease

Background and Purpose— We encountered several patients with childhood onset of moyamoya disease in whom the ipsilateral anterior and posterior circulations were predominantly involved. This study investigated whether this is an angiographic characteristic of this disease. Methods— We evaluated steno-occlusive lesions on angiograms of 85 patients with pediatric onset of moyamoya disease, using two 4-stage angiographic classification scales for the internal carotid artery and posterior cerebral artery systems (ICA and PCA staging, respectively) and determined whether lesions with more advanced ICA and PCA stages were on ipsilateral sides. Results— When positive laterality was defined as the presence of a difference by ≥1 stage between the stages on both sides, lateralities in the ICA stages and in the PCA stages were present in 40 (47%) and 27 patients (32%), respectively. Lesions with more advanced ICA and PCA stages were on the same side, with significant probability (P =0.024, Fisher’s exact test). Lateralities in both ICA and PCA lesions were found in 17 patients. In 14 (82%) of the 17 patients, the more advanced side of ICA lesions was the same as that of PCA lesions, while it was contralateral in 3 patients (18%). Conclusions— In pediatric-onset moyamoya disease, asymmetrical involvement of bilateral ICAs and PCAs was common, and the ipsilateral ICA and PCA tended to be predominantly involved.

Regional cerebral blood flow with age: changes in rCBF in childhood

Changes in cerebral blood flow (CBF) and regional cerebral blood flow (rCBF) throughout the entire age range including childhood were reported. The CBF in grey matter of children was markedly higher than that found in adults and showed a negative correlation with age. In contrast, the blood flow in white matter did not show a notable decrease with age. The regional distribution of CBF in children did not exhibit the frontal lobe dominance typical of adults. The rCBF pattern began to approach that of adults with growth and by 10 yr the rCBF pattern of children was similar to that of adults.

Urokinase Cisternal Irrigation Therapy for Prevention of Symptomatic Vasospasm After Aneurysmal Subarachnoid Hemorrhage A Study of Urokinase Concentration and the Fibrinolytic System

BACKGROUND AND PURPOSE Cisternal irrigation therapy with urokinase (UK) was performed in multiple institutions to prevent symptomatic vasospasm. The efficacy and safety of this therapy were evaluated, and the optimal concentration of UK was estimated. METHODS This therapy was performed in 28 patients who underwent surgery within 72 hours of the onset of severe subarachnoid hemorrhage (Fishers group 3, CT number [Hounsfield units] >60). After the aneurysm was clipped, irrigation tubes were placed in the Sylvian fissure (inlet) unilaterally and in the prepontine or chiasmatic cistern (outlet). Lactated Ringers solution with UK (30, 60, or 120 IU/mL) was infused at a rate of 30 mL/h. The presence of symptomatic vasospasm was evaluated by changes in the clinical symptoms and the presence of a new low-density area on CT scan. Drained irrigation fluid and peripheral blood were examined chronologically to evaluate the fibrinolytic system. RESULTS Symptomatic vasospasm was observed transiently in 3 cases (10.7%) without any low-density area on CT scan. In the 120-IU/mL group, no symptomatic vasospasm occurred. Analysis of drainage fluid suggested that UK 120 IU/mL is effective. The mean values of total drained blood volume for the respective groups were as follows: 58 mL in 30 IU/mL, 106 mL in 60 IU/mL, and 143 mL in 120 IU/mL. No abnormal changes were observed in the coagulative and fibrinolytic systems after UK irrigation. CONCLUSIONS These results suggest that cisternal irrigation therapy with UK is safe and effective for the prevention of symptomatic vasospasm after aneurysmal subarachnoid hemorrhage.

Limitation of temporary vascular occlusion during aneurysm surgery: Study by intraoperative monitoring of cortical blood flow

The limitations of temporary vascular occlusion during aneurysm surgery as evidenced by the postoperative ischemic symptoms in relation to cerebral blood flow were studied. Six of the 39 cases had postoperative ischemic neurological deficit. When residual cerebral blood flow was below 15 mL/min/100 g, transient symptoms were seen when temporary clipping was for 10-20 minutes. When more than 20 minutes of clipping was required, irreversible deficits were found. Correlations between residual cerebral blood flow, duration of temporary clipping, and postoperative ischemic symptoms were found. The monitoring of cortical cerebral blood flow is an effective means for determining the limits of temporary vascular occlusion.

A case of moyamoya disease accompanied by an arteriovenous malformation

It is well known that moyamoya disease sometimes is accompanied by cerebral aneurysm; however, no case of moyamoya disease with arteriovenous malformation has previously been published. We report such a case. A 33-year-old man suffered from transient left motor weakness and visual disturbance. Plain computed tomography (CT) showed a low density area in the left parietooccipital cortex. Enhanced CT revealed a ribbon-shaped enhanced area in the right temporooccipital cortex and a spotty enhanced area in the right frontal subcortex. Cerebral angiography revealed moyamoya disease. On the basis of Suzukis angiographic classification, the patient was diagnosed as Stage III. Right carotid angiography revealed an arteriovenous malformation fed by basal moyamoya vessels and draining to the vein of Trolard and the sylvian vein of the right frontal lobe. This arteriovenous malformation consisted of feeders, the nidus, and drainers. Trolards vein, which was one of the draining veins of the arteriovenous malformation, flowed to the moyamoya vessels of the right parietal vault.

Regional Cerebral Blood Flow in Patients With Hypertensive Intracerebral Hemorrhage

Regional cerebral blood flow was measured by 183Xe clearance method in 44 patients with hypertensive intracerebral hemorrhage (HIH) within three weeks from attack. Mean CBF in cases with the more disturbed consciousness was the lower value. There was no definite relationship between mean CBF and the duration from last stroke to measurement. In 44 cases, 35 showed hematoma of so-called “lateral type” and nine showed a “mesial type.” There was no difference of mean CBF between these two groups. In the group with lateral-type hematoma, 14 cases were thought to show large space-occupying signs, and their mean CBF was significantly lower than that of the other 21 cases. In 16 cases, relative hyperemic regions were observed in both the focal and nonfocal areas. In seven cases, relative ischemic regions were frequently in nonfocus rather than focus. Response of hyperemic region to carbon dioxide was good in seven and poor in three regions in focal area, and was good in eight and poor in one region in nonfocal area. Reactivity of ischemic region to 5% CO2 inhalation was good in three and poor in zero regions in focus, and was good in three and poor in two regions in nonfocus. Early venous filling (EVF) was observed in 13 cases and all of them were with lateral-type hematomas. Only four out of 13 cases showed good correlation between angiographical findings and cerebral circulation.

Clinical Manifestations, Character of Aneurysms, and Surgical Results for Unruptured Cerebral Aneurysms Presenting with Ophthalmic Symptoms

OBJECTS Cases with unruptured cerebral aneurysms presenting with visual symptoms were investigated about their site, size, symptom, operative methods and results. MATERIAL Between 1984 and 1999, 8 cases were treated in Sendai National Hospital. One man and 7 women, mean age 66.4 years. Ophthalmic symptoms were as follows: diplopia in 6, visual acuity deterioration in 2, impaired visual field in 2 and ptosis in 3. Aneurysm location was IC cavernous in 3, IC ophthalmic in 3, ICPC in 1 and Acom in 1. Aneurysms of more than 25 mm numbered 6 cases. RESULTS Operative methods and results were as follows: Direct clipping 3 cases, parent artery occlusion + EC/IC bypass 4 cases, Aneurysm trapping + EC/IC bypass 1 case. One patient who underwent direct clipping died following intraoperative complication. Of the remaining 7 cases, visual symptoms were improved in 4, remained unchanged in 2 cases, worsened in 1 case. CONCLUSIONS These results suggest that in cases with unruptured large or giant aneurysms presenting with ophthalmic symptoms, especially in IC cavernous or IC ophthalmic aneurysms, parent artery occlusion + EC/IC bypass is the safest operative procedure.

Non-traumatic recurrent dissection and its spontaneous repair in the circle of Willis: Report of two autopsy cases

Post‐mortem examinations of the circle of Willis in two cases of subarachnoid hemorrhage disclosed a wide spectrum of vasculopathy ranging from a minimal tear between the intima and media, and between the media and adventitia, to complete transmural disruption leading to the formation of pseudoaneurysms. The presence of coexistence of the focal lesions with complete replacement of the entire arterial wall with thick fibrous connective tissues and the vasculopathy was suggestive of the spontaneous repair of recurrent non‐traumatic dissection of intracranial arteries. The patients were 58‐year‐old and 43‐year‐old females. There was no history of injury to the head or neck in either case. They were hypertensive, but the degree of atherosclerotic changes in the circle of Willis was compatible with age. There was no histological evidence of vasculitis. The role of hypertension and medial mucoid degeneration in the genesis of non‐traumatic dissection of intracranial arteries was discussed.