烟雾病

烟雾病 烟雾病 1.Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan.   Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Kita-ku, Sapporo, Japan. skuroda@med.hokudai.ac.jp兰州大学第二医院神经外科任军 BACKGROUND AND PURPOSE: Although the development of a noninvasive MR examination has increased the opportunity to identify asymptomatic patients with moyamoya disease who have experienced no stroke episodes, their clinical features are still unclear. This was the first multicenter, nation-wide survey focused on asymptomatic moyamoya disease in Japan and was designed to clarify their clinical features. METHODS: A clinical database of asymptomatic patients with moyamoya disease was collected from 12 participating hospitals in Japan between 2003 and 2006. In total, 40 patients were enrolled in this historical prospective cohort study. Of these, 6 underwent surgical revascularization, including superficial temporal artery to middle cerebral artery anastomosis and/or pial synangiosis. Their demographic and radiological findings as well as outcome were evaluated. RESULTS: On initial evaluation, cerebral infarction and disturbed cerebral hemodynamics were detected in approximately 20% and 40% of the involved hemispheres, respectively. Angiographical stage was more advanced in more elderly patients. Of 34 nonsurgically treated patients, 7 experienced transient ischemic attack (n=3), ischemic stroke (n=1), or intracranial bleeding (n=3) during follow-up periods (mean, 43.7 months). The annual risk for any stroke was 3.2%. Disease progression was associated with ischemic events or silent infarction in 4 of 5 patients. No cerebrovascular event occurred in the 6 patients who underwent surgical revascularization. CONCLUSIONS: The findings revealed that asymptomatic moyamoya disease is not a silent disorder and may potentially cause ischemic or hemorrhagic stroke. Asymptomatic patients with moyamoya disease should be carefully followed-up to further clarify their outcome and to establish the management guideline for them. 2. Stepwise revascularization for prevention of postoperative hyperperfusion. Abrupt normalization of cerebral blood flow (CBF) after surgical procedures to improve excessive cerebral hypoperfusion can cause irreversible brain parenchymal damage. Such hyperperfusion, which is caused by inflow at normal blood pressure into maximally dilated fine vessels, is an important complication following carotid endarterectomy (CEA). Strict control of blood pressure in the perioperative period can prevent this complication except in a few patients, who have severe cerebral hypoperfusion and poor cerebrovascular reserve due to extremely severe stenosis of the ipsilateral or the bilateral carotid arteries, for which CEA is indicated. The requirement for improved CBF and the risk of postoperative hyperperfusion conflict in the pathogenesis of these patients. We tried to prevent abrupt improvement in perfusion by attempting gradual restoration of CBF. Superficial temporal artery-middle cerebral artery anastomosis was first performed to improve the poor cerebrovascular reserve by allowing insufficient blood flow. A few weeks later, CEA was performed to completely restore CBF. This surgical approach obtained good results without postoperative problems in four patients. The indications of this surgical management and efficacy of stepwise restoration of CBF to prevent postoperative hyperperfusion depend on careful preoperative evaluation of perfusion studies. 3. A modified technique for bypass of the external carotid artery to the proximal posterior cerebral artery: an anatomical and technical study. CONCLUSIONS: Our results support the proposition that bypass between the external carotid artery (ECA) and proximal posterior cerebral artery (PCA) can be achieved by using a short saphenous venous graft. The diameters of the ECA and vein graft may provide an increased blood flow with a straighter course. This technique may be helpful for management of patients with vertebrobasilar insufficiency or those requiring a high volume blood flow to the posterior circulation. OBJECTIVES: We aimed to describe a modified technique using a short saphenous vein graft for bypass between the ECA and the PCA in order to use a small length of graft material and increase the patency of the anastomosis. MATERIALS AND METHODS: Ten sides of five cadavers were dissected bilaterally. After a frontotemporal craniotomy and zygomatic arch osteotomy, the middle cranial fossa was exposed. A hole located approximately 2-3 cm posterolateral to the foramen rotunda was created extradurally. The sylvian fissure and the interpeduncular and ambient cisterns were opened. The proximal P2 segment of the PCA was identified. The ECA was found through a cervical incision. A short interposition saphenous vein graft was conducted to pass just behind the ramus mandible to the infratemporal fossa. The bypass between the ECA and P2 segment of the PCA was performed by using a short saphenous vein graft. The diameters of the ECA, P2 segment of PCA and both ends of the saphenous vein graft and its length were measured using an electronic micrometer. RESULTS: The mean cross-clamping time of the PCA was 10.4+/-1.8 min. The mean diameters of the P2 segment of the PCA and ECA were 2.2+/-0.15 mm and 3.83+/-0.28 mm, respectively. The mean length of the saphenous vein graft was 88.8+/-3.8 mm. 4. Experience of treatment of moyamoya disease at the Clinic of Neurosurgery of Kaunas University of Medicine. BACKGROUND: Moyamoya disease was first described in Japan and represents characteristic appearance on cerebral angiography an abnormal network ("puff of smoke") of collaterals around stenotic arteries. This disease is characterized by progressive intracranial vascular obliterations of the circle of Willis, resulting in successive ischemic or hemorrhagic events. Moyamoya disease primarily occurs among orient people (Japanese, Koreans, Caucasians) and is very rare in Lithuania. AIM OF THE STUDY: To evaluate long-term effectiveness of cerebral revascularization in order to prevent cerebral ischemic and hemorrhagic events in patients with moyamoya disease. MATERIAL AND METHODS: Since 1995, 14 patients with moyamoya disease have been treated at the Clinic of Neurosurgery of Kaunas University of Medicine. All patients underwent neurological examination, computed tomography or/and magnetic resonance imaging, transcranial Doppler sonography, single photon emission computed tomography and four vessels cerebral angiography investigations. The diagnosis of moyamoya disease was confirmed by cerebral angiography. Cerebral ischemia was observed in 7 cases and cerebral hemorrhage was detected in the other 7 moyamoya disease patients. Neurological disability was evaluated using the Scandinavian Stroke Scale, and performance in the active daily life using the Barthel Index. The degree of handicap was assessed with the Rankin Scale. Different surgical procedures (superficial temporal artery to middle cerebral artery anastomosis, encephalosynangiosis, bifrontal free omental flap) were applied for cerebral revascularization of moyamoya disease patients. RESULTS: The diagnosis of moyamoya disease was confirmed by cerebral angiography in all our patients. Ten patients were selected for surgical brain revascularization. The main criterion for selection of patients for surgery was hypoperfusion of the brain on single photon emission computed tomography. During follow-up period (mean follow-up period was 36 months) there were no rebleeding or ischemic events in both surgical groups. CONCLUSIONS: Cerebral angiography is the main diagnostic procedure which confirms the diagnosis of moyamoya disease. Cerebral hypoperfusion on single photon emission computed tomography is the main criterion for selection of patients for cerebral revascularization. Extra-intracranial anastomosis is an effective procedure for preventing both ischemic and hemorrhagic events in moyamoya patients. 5. Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease. BACKGROUND: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving CBF, but little is known about the change in CBF and its effect on neurologic status during the acute stage after revascularization. METHODS: 123I-IMP-SPECT was performed 1 and 7 days after STA-MCA anastomosis on 34 sides of 27 consecutive patients with adult-onset moyamoya disease (6 men, 21 women; 22-62 years old). The follow-up period ranged from 5 to 28 months (mean, 17.6 months). RESULTS: Thirteen patients (13 sides, 38.2%) suffered temporary neurologic deterioration due to hyperperfusion several days after surgery, which was sustained for several days (7.4 days in average). Postoperative magnetic resonance imaging/angiography showed the STA as a higher intensity signal than the preoperative finding without ischemic changes in all 13 patients. Postoperative SPECT revealed focal intense increase in CBF at the sites of anastomosis in all 13 patients. Eleven patients (32.4%) had transient focal neurologic deficit mimicking ischemic attack. Two patients (5.9%) had cerebral hyperperfusion syndrome associated with subarachnoid hemorrhage extending to the ipsilateral sylvian cistern. Symptoms were relieved by intensive blood pressure control, and no patients had permanent neurologic deficit or delayed neurologic deterioration during the follow-up period. CONCLUSIONS: Surgical revascularization including STA-MCA anastomosis is a safe and effective treatment for moyamoya disease, although temporary neurologic deterioration due to hyperperfusion could occur at a substantial rate. Routine CBF measurement is recommended for accurate diagnosis of postoperative hyperperfusion in moyamoya disease because its treatment is contradictory to that for ischemia. 6. Surgical therapy for adult moyamoya disease. Can surgical revascularization prevent the recurrence of intracerebral hemorrhage? BACKGROUND AND PURPOSE: It is well recognized that revascularization surgery using direct and/or indirect bypass provides effective surgical management for pediatric moyamoya disease. However, surgical treatment of the adult hemorrhagic type remains controversial. In this study, the effect of surgery for adult moyamoya disease was investigated. METHODS: We analyzed 35 patients with adult moyamoya disease (patient age, over 20 years), 24 patients with initial onset of intracerebral hemorrhage, and 11 patients with initial onset of cerebral ischemia who underwent both direct bypass surgery of the superficial temporal artery to the middle cerebral artery anastomosis and indirect revascularization of encephalo-duro-arteriomyo-synangiosis. RESULTS: Of 24 patients with hemorrhagic-type disease, 3 showed rebleeding: of 11 patients with the ischemic type, 2 showed intracerebral hemorrhage after surgery. Overall, 5 of 35 patients (14.3%) had hemorrhage after revascularization surgery (mean follow-up period, 6.4 years). Postoperative angiography revealed that direct anastomosis is effective whereas indirect revascularization is not always effective for adult moyamoya disease. Moyamoya vessels, which are supposed to be responsible for hemorrhage, decreased in 25% of patients. CONCLUSIONS: Revascularization surgery cannot always prevent rebleeding. However, a decrease in moyamoya vessels was induced by surgery, which may reduce the risk of hemorrhage more effectively than conservative treatment. In cases of adult moyamoya disease, direct bypass is particularly important, since the indirect revascularization is not as useful in adult cases as in pediatric cases. 7. 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. 8. Symptomatic hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in a child with moyamoya disease. OBJECT: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). It is undetermined, however, how rapid increase in CBF affects ischemic brain at acute stage, especially in children. CASE REPORT: A 4-year-old girl with moyamoya disease underwent right superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. She suffered temporary left facial palsy 5 days after surgery. Postoperative N-isopropyl-p-[(123)I]iodoamphetamine single-photon emission computed tomography ((123)I-IMP-SPECT) revealed focal intense increase in CBF at the sites of anastomosis. Magnetic resonance imaging/angiography showed the apparently patent STA-MCA anastomosis as a thick high signal without ischemic changes. Her symptom improved 9 days after surgery, and single-photon emission computed tomography (SPECT) 2 months later showed normalization of CBF. Surgical revascularization completely relieved the transient ischemic attack on her left hand that was seen before surgery. CONCLUSION: We demonstrated, for the first time, that delayed focal neurological deficit after STA-MCA anastomosis can be caused by focal hyperperfusion in childhood moyamoya disease. 9. Focal hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease. Case report. Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is a standard surgical therapeutic option in patients with moyamoya disease. Most patients experience improvement in their clinical symptoms immediately after surgery. The authors report on the case of a 39-year-old man with moyamoya disease who suffered from temporary and frequent neurological deterioration after undergoing a left STA-MCA anastomosis. Hemodilution and hypervolemia therapies did not improve his course. Technetium-99m hexamethylpropyleneamine oxime single-photon emission tomography scans demonstrated focal intense accumulation of the tracer in the frontal operculum on the side of the surgery. Although diffusion-weighted magnetic resonance (MR) imaging demonstrated no abnormalities except for the postoperative change, perfusion-weighted MR images and MR digital subtraction angiography revealed focal hyperperfusion in the left frontal operculum where the cerebral perfusion reserve was severely disturbed preoperatively. This evidence strongly supports the notion that focal hyperperfusion observed after STA-MCA anastomosis could occur in the poor perfusion reserve area preoperatively and could cause temporary neurological deterioration. 10. Neural damage caused by cerebral hyperperfusion after arterial bypass surgery in a patient with moyamoya disease: case report. OBJECTIVE AND IMPORTANCE: The prognosis of cerebral hyperperfusion syndrome after vascular reconstructive surgery, including extracranial-intracranial arterial bypass, is not poor unless intracerebral hemorrhage develops secondary to hyperperfusion. CLINICAL PRESENTATION: A 48-year-old man with symptomatic moyamoya disease with misery perfusion in the right cerebral hemisphere underwent double right superficial temporal artery-to-middle cerebral artery bypasses. The postoperative course was uneventful until the patient developed headache and agitated delirium on the 4th postoperative day. INTERVENTION: Perfusion computed tomographic imaging demonstrated hyperperfusion in the right temporal lobe. The symptoms resolved by institution of intensive blood pressure control. Positron emission tomography performed 2 months after surgery demonstrated a postoperative reduction of the cerebral metabolic rate of oxygen in the right temporal lobe, where brain atrophy was observed on magnetic resonance images 3 months postoperatively. Neuropsychological testing performed 3 months postoperatively showed worsening digit span, which adversely affected the patient's quality of life. CONCLUSION: The current case suggests that cerebral hyperperfusion after vascular reconstructive surgery can cause irreversible neural damage, which results in cognitive impairment. 11. Effectiveness of superficial temporal artery-middle cerebral artery anastomosis in adult moyamoya disease: cerebral hemodynamics and clinical course in ischemic and hemorrhagic varieties. BACKGROUND AND PURPOSE: The efficacy of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in adult moyamoya disease was evaluated by clinicopathophysiological studies. METHODS: Fifteen patients with cerebral ischemic attacks (ischemia group) and 15 patients with intracranial hemorrhages (hemorrhage group) were investigated. Clinicoangiographic features and regional cerebral blood flow (rCBF) of the MCA territory were preoperatively and postoperatively investigated, and cortical arterial pressure (CAP) and anastomotic blood flow (AF) were intraoperatively measured. RESULTS: In the ischemia group, the preoperative rCBF of 38.4 mL/100 g per minute was significantly increased to 42.1 mL/100 g per minute with a diminution of angiographic moyamoya vessels in 67% of patients after surgery. The mean CAP and AF were 25.6 mm Hg and 34.7 mL/min, respectively. Proximal and distal