About 40 years has passed since moyamoya disease, a unique cerebrovascular disorder occurring widely in far-east Asia, was established as a disease entity. In the present article, the authors review the historical background, disease entity, diagnosis, radiological findings, and medical and surgical management of moyamoya disease. MR imaging and angiography represent the most powerful tools for the diagnosis and follow-up of patients with moyamoya disease. Evaluations of cerebral blood flow and metabolism, using single photonemission tomography (SPECT) and positron emission tomography (PET). tend to be more important for managing cases effectively and safely. Surgical cevascularization has been accepted as valuable for preventing further ischemia attacks in both pediatric and adult patients who present with TIA or cerebral infarction. However, the efficacy of bypass surgery for patients who develop intracranial bleeding due to moyamoya disease needs to be clarified by the on going multi-center randomized clinical trial in Japan (Japan Adult Moyamoya Trial : JAM Trial). Additionally, in this article, the authors present an overview of future aspects of moyamoya disease, including research to find the responsible genes of moyamoya disease, and the manage-ment of asymptomatic or pregnant patients with moyamoya disease.

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To elucidate the clinical course and outcome of embolic occlusion in the middle cerebral artery (MCA) by non-thrombolytic therapy, we analyzed the neuroradiological and demographic data of 32 cases with cardiogenic embolic occlusion of the MCA horizontal portion who were admitted within 4 days of onset. In terms of their final outcome, 2 cases were designated as good recovery (GR), 15 cases as moderate disability (MD), 12 cases as severe disability (SD), 1 case as vegetative state the (VS) and 2 cases died (DD). Reopening of the occluded vessel was observed in 19 of the 32 cases within 30 days of onset. Twelve of the 19 cases with reopening were regarded as GR or MD, whereas 5 of the 13 cases without reopening were regarded as SD, VS or DD. There was a tendency whereby the greater the infarct volume calculated from CT images, the worse was the final outcome. As regards the sites of occlusion, GR or MD was seen in 66.7% of cases at the distal portion of the MCA horizontal portion, 50% of cases at the central portion, and 33.3% of cases at the proximal portion.

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Recent studies have established the importance of aggressive medical therapy using tissue plasminogen activator for the hyperacute stage of stroke in the USA and Europe Hospital arrival time varies among differ-ent types of stroke and area. We investigated the arrival time after the onset of different types of stroke. The critical factors determining access to acute stroke were examined in each type of stroke. Seikeikai General Hospital is located in the Sakai-city area of Osaka, Japan. A total of 200 sets of data were collected consecutively from the medical records of acute cerebral infarction patients admitted directly to the emer-gency department (ED) during a 2-year period (1998-1999), and divided into three subtypes : lacunar (n = 110) atherothrombotic (n = 50), and cardioembolic (n=40). Forty-one percents arrived within 3 hours. Excluding patients whose symptoms were first noticed on awaking, 26% of patients arrived early within 2 hours and 37% within 3 hours. The median time from the onset to ED arrival in patients witn cardioembolic infarction was 2.8 hours, which was significantly shorter than that in those with lacunar infarction (10.3 hours) or atherothrombotic infarction (7.5 hours) (p<0.01). The frequency of patients arriving at the ED within 3 hours after the onset in lacunar, atherothrombotic, and cardioembolic infarction was 28%, 34%, and 65%, respec-tively. Patients with disturbance of consciousness arrived much sooner after the onset that did those without it (66.7% vs. 32.2%) (p<0.01) . Also, patients with spouses arrived earlier than did those without spouses (45% vs. 31%) (p<0.05). However, there was no significant difference in arrival time between patients with and without a past history of stroke. A "brain attack" education program to increase public knowledge about stroke could contribute to increasing the speed of hospital presentation after the onset of stroke.

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We examined the influence of inter-hospital cooperation between an acute stroke center and rehabilitation hospital on patient outcome and length of hospital stay. Patients with acute ischemic stroke admitted between May 1999 and April 2000 were enrolled on the study. In total, 806 patients (459 men, 347 women ; 71. 0±12.2 years old) who were admitted to hospitals in Kumamoto city where good inter-hospital cooperation ex-isted were classified as group U. On the other hand, group R (217 patients ; 140 men, 77 women ; 72.2±11.9 years old) consisted of patients who were admitted to hospitals in the suburbs of Kumamoto. The average NIHSS on admission was 8.2 for group U and 9.0 for group R. The average length of hospital stay in each group was 17.3 and 38.1 days, respectively. Patients who were directly discharged to their home (43% of group U and 55% of group R) were hospitalized for 12.5 days and 32.0 days, respectively. Their modified Rankin Scale (at discharge/one year later) was 0.8/1.4 for group U and 1.0/1.6 for group R. Similarly, patients who were transferred to other hospitals comprised 44% and 21% of groups U and R, respectively. The length of hospital stay and mRS (at discharge/one year later) in the two groups were 22.7 days and 3.7/3.8 for group U, and 65.8 days and 4.0/4.0 for group R. We have been developing a new stroke management system based on the stroke unit with referral to a rehabilitation unit at another hospital (inter-hospital referral model) in Kumamoto city. The length of hospital stay has decreased remarkably in comparison with the "intra-hospital referral model". This "regional inter-hospital referral model" is regarded as a model for acute stroke management systems.

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We studied the changes of von Willebrand Factor (vWf) activity after acute cerebral infarction (ACI) in order to identify differences in each subtype of ACI and to determine the relationship between the vWf activities and stroke severity. In addition, we investigated the influence of anti-platelet drugs on the vWf activities retrospectively. Eighty-three consecutive patients admitted to our hospital within 48 hours after the onset were enrolled. We diagnosed the patients as ACI by cranial CT and MRI, and classified them into 3 subtypes (atherothrombotic, cardioembolic and lacunar infarction) according to the classification of NINDS. The severity of ACI was evaluated by the Japan Stroke Scale (JSS), and the plasma vWf activities were determined both on admission and one month later. The patients showed significantly higher vWf activities on both ad-mission and after one month as compared to those in the controls. Furthcrmore, the vWf activities on admis-sion increased significantly over a month. There was no significant difference in sequential changes of vWf activities among the ACI subtypes. The severity of ACI was not associated with the vWf activities on admis-sion. The elevation of the vWf activities could be suppressed in the group of patients treated with cilostazol as compared to the other groups. We conclude that although the vWf activities were increased over one month in the patients with ACI, there was no significant difference among the ACI subtypes, and no relationship with the severity of ACI. The significance of the suppressive effect of cilostazol on the vWf activities in patients with ACI should be investigated in furthers studies.

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150 kDa-oxygen-regulated protein (ORP150) is a novel stress protein localized in the endoplasmic reticulum, which is induced by hypoxia/ischemia. In order to elucidate the role of ORP150 in cerebral infarction following ischemia/reperfusion, transient focal cerebral ischemia was produced in ORP150 transgenic (TG) and knockout (KO) mice in the present study. Mice were subjected to 1 or 3 hours of middle cerebral artery (MCA) occlusion followed by reperfusion for 24 hours. At 24 hours after 1 hour-occlusion, a significant reduction in volume of infarction was found in the cerebral cortex, but not in the striatum, in the ORP150 TG mice as compared to the ORP150 KO mice (P<0.001). However, no significant difference in infarct volume was noted between the two groups at 24 hours after 3 hours-occlusion. Immunohistochemically, loss of microtubule-associated protein 2 (MAP2) staining in the MCA area was observed in the ORP150 KO mice at 24 hours after 1 hour-occlusion. On the other hand, MAP2 staining was still present in the affected cortex of the ORP150 TG mice where a markedly enhanced ORP150 immunoreactivity was demonstrated MAP2 staining disappeared in the affected area at 24 hours after 3 hours-occlusion in both groups, but ORP150 antigenicity was preserved there in the ORP150 TG mice group. At 6 hours after 1 hour-occlusion, when MAP2 staining was evident in the affected cortex, some cortical neurons of the TG mice were Bci-xs/L positive. These findings suggest that ORP150 is a cytoprotective participant against ischemia/reperfusion injury through a reduction of endoplasmic reticulum stress and probably through its inhibitory effects on apoptosis.

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We experienced two ischemic stroke patients with floating thrombus (FT) coupled with a high-grade stenosis of the internal carotid artery (ICA). They demonstrated different clinical outcomes after conventional treatment. An 83-year-old woman exhibited drowsiness, motor aphasia, and right-sided hemiparesis. Magmetic resonance (MR) images on admission displayed multiple infarctions in the territory of the left deep border zone, and MR angiograms revealed poor visualization of the left ICA. Emergency cerebral angiograms disclosed a high-grade ostial stenosis of the left ICA with FT. Brain perfusion-weighted MR images (PWI) showed no laterality. The patients was treated with intravenous heparin and oral antiplatelets, and her neuro-logical symptoms improved. Carotid ultrasonography revealed no FT at 16 days later, and the patient underwent carotid stenting. Her modified Rankin Scale was 1 at 3 months after her ischemic stroke onset. An 82-year-old man exhibited drowsiness, total aphasia, conjugate deviation and right-sided hemiparesis. MR images on admission displayed infarction in the territory of the left corona radiata, and MR angiograms revealed poor visualization of the left ICA. Emergency cerebral angiograms disclosed a high-grade ostial stenosis of the left ICA with FT. Brain PWI showed no laterality. Although the patient was treated with intravenous heparin and oral antiplatelets, his neurological symptoms deteriorated, FT disappeared at 27 days later, and the patient underwent carotid stenting. However, his neurological symptoms were no longer improved after the procedure. His modified Rankin Scale remained 5 at 3 months after his ischemic stroke onset. When neurologi-cal symptoms begin to deteriorate after starting conventional treatment, carotid artery reconstruction may be a treatment of choice. The 3-month clinical outcome after intravenous heparin and oral antiplatelets varies in patients with FT coupled with a high-grade stenosis of the ICA.

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A 77-year-old man who suffered from bilateral hearing disturbance followed by apparent cerebral infarc-tion signs is reported. He complained of right hearing disturbance and, after 4 hours, left hearing disturbance. Neurological examinations revealed gaze-evoked nystagmus to right-ward gaze.The next day, right hemi-paresis and paralytic aphonia appeared, and treatment for cerebral infarction was initiated. A CT scan at 4 days after the onset revealed an infarcted area in the left middle pons, and 3D-CTA after 6 days showed that the middle portion of the basilar artery was occluded. 3D-CTA after 1 month demonstrated a recanalization of the basilar artery.
Since hearing disturbance could be an initial symptom of cerebrovascular disease, closer observation of the cerebral signs and further investigations of the cerebral artery are necessary.

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Employing analysis of CBF confidence maps, we investigated the usefulness of a head holder using a negative pressure bag packed with plastic beads in a xenon CT CBF study. A total of 272 consecutive patients for the CBF study were enrolled and classified into 3 groups : 88 patients with a negative pressure bag (M group), 87 patients with an air pillow (A group), and 97 patients with a sponge pillow (S group). The degree of effect of head movements on the CBF measurement in each patient was expressed as a confidence value (mean of the confidence values at one CT slice). The mean of confidence value in the M group (0.461) was statistically lower than that in the A group (0.866) and that in the S group (1.043). These findings showed that the head holder described here was useful for obtaining CBF maps of high quality in a xenon CT CBF study.

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