Absent anterior communicating artery and varied distribution of anterior cerebral artery

During the cerebral dissection of a 67-year-old male cadaver, a unique combination of variations at the circle of Willis and anterior cerebral artery (ACA) distribution were encountered. The A1 segment of both ACA were fused without an anterior communicating artery (ACoA), forming an X shape and giving rise to a common pericallosal artery (CPA), an incomplete distal ACA, and an incomplete distal anterior cerebral artery (IACA). The IACA had an unusual course, which may be important from the surgical point of view. The CPA continued as the A2 and A3 segments, and bifurcated into 2 pericallosal arteries. Branching patterns of the varied arteries to the interhemispheric region were evaluated, and results were discussed. Additionally, both posterior communicating arteries were hypoplastic. There was no aneurysm formation at the circle of Willis and its branches.     

The arterial patterns associated with internal carotid disease and cerebral infarcts

In 20 necropsies with 15 stenosed and 17 thrombotic occluded internal carotid arteries there were 46 cerebral infarcts larger than 1 cm diameter. Using portmortem arteriographic and pathological techniques the patterns of the neck and brain artery systems were correlated with the situation and extent of the brain infarcts. Massive infarcts involving two major cerebral artery territories were associated with distal internal carotid artery occlusion and grossly ineffective cervical and circle of Willis anastomoses. Isolated middle cerebral artery territory infarcts were associated with internal carotid occlusion or stenosis and impairment of the circle of Willis anastomoses, perhaps with middle cerebral artery stenosis. The pattern of adequate size arteries determined if these infarcts were total, deep central, anterior, medium or posterior partial territory infarcts. Boundary zone infarcts were associated with internal carotid artery disease and limitation of anterior or posterior circle of Willis anastomoses. These limitations determined which boundary zones were affected. Isolated anterior cerebral artery territory infarcts were associated with bilateral internal carotid disease and an anterior cerebral artery stenosis or small caliber anterior communicating artery. Isolated posterior cerebral artery territory infarcts were associated with internal carotid disease and a direct impairment of the ipsilateral posterior cerebral artery capability.     

Evaluation of cross-circulation through circle of Willis using an ultrasonic Doppler technique. Part I. Comparison between blood flow velocity by ultrasonic Doppler flowmetry and angiogram

In 24 patients with cerebrovascular disease and 6 without organic brain lesions, the increased velocity of blood flow in both the internal carotid and vertebral arteries during a contralateral carotid compression was compared with the angiographic appearance of the circle of Willis. The flow velocity was measured using ultrasonic Doppler flowmetry. It was not possible to investigate fully the relationship between the increase of velocity of blood flow in the internal carotid artery and the anatomical variations of the circle of Willis, specifically the anterior cerebral and communicating arteries. The velocity of flow in the patients with an aplastic proximal portion of the anterior cerebral artery showed no increase. There were no differences in the increase of velocity of flow in the vertebral artery in patients with hypoplastic, normal and fetal posterior communicating arteries. It is considered that although anatomical variations of the circle vessels influence the cross-circulation via the circle of Willis, peripheral vascular factors distal to the circle also play an important role in the quantity of cross-circulation through the circle.     

Evidence for the presence of cholinergic nerves in cerebral arteries: an immunohistochemical demonstration of choline acetyltransferase

The presence of cholinergic nerves in cerebral arteries of several species was investigated by an immunohistochemical method using antibodies against choline acetyltransferase (ChAT). In cats, pigs, rats, and dogs, ChAT immunoreactivities were found to be associated with large bundles and single fibers in the circle of Willis and anterior cerebral, middle cerebral, and basilar arteries. In the rabbit, the ChAT-immunoreactive (ChAT-I) nerves were also observed in the circle of Willis and anterior and middle cerebral arteries, but only few or none were found in the basilar and vertebral arteries. The ChAT-I nerves were found only in the adventitial layer of vessels examined. Superior cervical ganglionectomy did not appreciably affect the distribution of ChAT-I nerves. These results indicate the presence of cholinergic nerves in cerebral arteries. The distribution pattern of ChAT-I nerves was different from that of vasoactive intestinal polypeptide (VIP)-like-immunoreactive nerves and acetylcholinesterase-positive nerves. The possible coexistence of ChAT and VIP-like substance in the same neuron is discussed.     

Origins and distribution of cerebrovascular nerve fibers showing calcitonin gene-related peptide-like immunoreactivity in the major cerebral artery of the dog

The origins and overall distribution of perivascular nerve fibers showing calcitonin gene-related peptide-like immunoreactivity (CGRP-LI) in the major cerebral arteries were investigated immunohistochemically in the dog by using whole-mount preparations of the arterial trees around the circle of Willis. Perivascular nerve fibers with CGRP-LI were seen most abundantly in the basilar artery, vertebral artery, common anterior cerebral artery, proximal part of the anterior cerebral artery, and terminal part of the internal carotid artery. They were far less numerous in the middle cerebral artery, posterior cerebral artery, superior cerebellar artery, and distal part of the anterior cerebral artery. Neuronal cell bodies with CGRP-LI were observed in the trigeminal, nodose, superior cervical, and dorsal root ganglia. CGRP-LI fibers in the large pial arteries in the circle of Willis were eliminated ipsilaterally after unilateral transection of the ophthalmic division of the trigeminal nerve, and slightly reduced in number ipsilaterally after unilateral transection of the maxillary division of the trigeminal nerve. They did not show any noticeable changes after unilateral transection of the mandibular division of the trigeminal nerve. On the other hand, CGRP-LI fibers in the basilar and vertebral arteries did not show any appreciable changes after unilateral transection of the trigeminal nerve, but they were eliminated after bilateral ganglionectomy of the dorsal root ganglia of the first, second, and third cervical nerves. After ganglionectomy of the ciliary, pterygopalatine, otic, nodose, or superior cervical ganglion, no changes were observed in perivascular nerve fibers with CGRP-LI in the major cerebral arteries.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cerebral arterial innervation: II. Development of calcitonin-gene-related peptide and norepinephrine in the rat

The pre- and postnatal development of trigeminal calcitonin-gene-related peptide (CGRP)- and sympathetic norepinephrine (NE)-containing nerves supplying the cerebral arteries was studied with immunohistochemistry in rats. At 18-19 days in utero (E 18-19), CGRP fibers were present only as one or two longitudinal bundles zigzagging along the anterior cerebral artery and anterior communicating artery. Growth-cone-like swellings were found at the terminals of individual fibers. In contrast, at this same prenatal age NE fibers were present as a meshwork on all cerebral arteries. The density of NE fibers was higher in the rostral than in the caudal parts of the circle of Willis; growth cones were present on individual fibers at the middle segment of the basilar artery and distal parts of major cerebral arteries. At postnatal day 1-2 (PND 1-2; date of birth = PND 1), the outgrowth of CGRP axons extended along the walls of the middle cerebral and internal carotid arteries. These axons were relatively straight and unbranched. At the same time, NE fibers increased in number and density and continued to form the meshwork pattern on all cerebral arteries. At the end of the first postnatal week, all the longitudinal NE bundles on the rostral part of the circle of Willis began to form circular arborizations. At the end of the second postnatal week, the pattern of NE innervation had completely changed, consisting almost entirely of circumferential rather than tangential fibers. Beginning in the first postnatal week, CGRP fibers increased greatly in number and density and began to form a meshwork pattern. At the second postnatal week, the pattern of CGRP innervation, compared to the pattern at fetal and neonatal stages, had changed significantly, consisting predominantly of a meshwork pattern. By 4 weeks after birth, both the NE and CGRP fiber systems achieved adult densities and patterns. The present results demonstrate the following: 1) Both sympathetic-NE and trigeminal-CGRP innervation of cerebral arteries begin in utero; the NE system innervates corresponding parts of the vessels earlier than the CGRP system. 2) Both NE and CGRP fibers are more dense in the rostral than in the caudal segments of the circle of Willis; this rostrocaudal gradient is expressed in both density and pattern by the earliest fibers of both neurochemical systems and is maintained throughout all developmental stages.(ABSTRACT TRUNCATED AT 400 WORDS)     

A prospective study of cerebral artery atherosclerosis

Atherosclerosis in the circle of Willis and its major branches was studied prospectively in 198 men in the Honolulu Heart Program who were free of cardiovascular disease at the entry examination. The level of atherosclerosis was greater in the large arteries of the circle of Willis than in the small arteries, and autopsy-verified cerebral infarction was strongly associated with increasing severity of atherosclerosis in both. Analyses of the association of atherosclerosis scores with biologic and lifestyle characteristics measured at entry into the study indicated that atherosclerosis in the large arteries was consistently related to age, diastolic blood pressure, serum cholesterol, and height (inversely). Weak trends of association were also found with increasing serum glucose concentration, increasing cigarette use, and decreasing alcohol intake. Atherosclerosis scores in the small arteries were associated with diastolic blood pressure and serum triglyceride concentration. Analysis of dietary intake indicated that atherosclerosis scores were higher for men who reported low intakes of fat and animal protein and high intakes of vegetable protein and total carbohydrates. These patterns were consistent with similar findings on the incidence of clinical stroke in this cohort. Age-adjusted and -specific atherosclerosis scores from both the large and small arteries declined significantly during the period 1965-1983.     

Intracranial hemorrhage in autopsy cases with occlusion of the circle of Willis (author's transl)

Seventeen autopsy cases with occlusion of the circle of Willis were examined clinicopathologically. These patients ranged in age from 8 years to 64 years and consisted of 6 males and 11 females. The following results were obtained. 1. Fresh and massive intracerebral hemorrhage was confirmed in 13 of 17 patients and cerebral infarct in 4 of 17 patients. 2. Among these 13 patients, massive hemorrhage was found in basal ganglia, thalamus and hypothalamus of 9 patients, and in thalamus, cerebral peduncle and midbrain of 4 patients. 3. Rupture of the dilated muscular-type arteries was noted in the thalamus and basal ganglia in 2 of 13 patients. Ruptured arteries with organization of the lumen were found in the old hemorrhagic foci in one of them. 4. Overgrown and dilated arteries, branching off from the circle of Willis, were confirmed in 11 patients. These arteries consisted of "perforating" arteries well developed as collateral circulation. 5. No ruture in these arteries of the subarachnoidal spaces was found in 11 patients. In one of them, a small saccular aneurysm was found. These findings strongly suggest that in patients with occlusion of the circle of Willis intracerebral rupture of overgrown "perforating" arteries as collateral circulation may be the main cause of intracranial hemorrhage and intracerebral hemorrhage due to rupture of the arteries may be repeated.     

Saccular Aneurysm of the Azygos Anterior Cerebral Artery: Three Case Reports

The azygos anterior cerebral artery, a rare anomaly in the circle of Willis in which only a single vessel supplies the medial aspects of both anterior cerebral hemispheres, is closely associated with saccular aneurysms. We present three cases of azygos anterior cerebral artery aneurysms among the 781 cerebral aneurysms surgically treated at our institution in an 11-year period. These three cases all involved elderly women who presented with subarachnoid hemorrhage. Conventional cerebral angiography and CT angiography revealed small saccular aneurysms at the distal ends of the azygos anterior cerebral arteries. These aneurysms were clipped successfully using a bifrontal interhemispheric approach. Hence, the pathogenesis of these particular aneurysms relating to hemodynamic change, associated anomalies, and surgical pitfalls is discussed with review of literature.     

Arterial supply of limbic structures in the guinea pig.

This study outlines the vascular territories of the cerebral arteries that originate from the Willis circle to supply limbic structures in the guinea pig brain. The entire cerebral vascular system was visualized in four preliminary experiments by performing superselective microangiographic studies with iodine contrast medium perfusion of the whole brain after in vitro isolation according to a technique described previously (de Curtis et al. [1991] Hippocampus 1:341-354). Subsequently, the perfusion territory of the different arteries that originate from the Willis circle was characterized after cannulation and perfusion of individual arteries with a gelatin solution that contained waterproof black ink. The analysis was performed by identifying the brain regions that contained the black stain on 150-microm-thick coronal sections that were cut after brain fixation with paraformaldehyde for at least 1 week. The middle cerebral artery and the rostral and caudal posterior cerebral arteries supply the limbic cortices and some related subcortical regions. In particular, large portions of the hippocampal formation are supplied by both the rostral posterior cerebral artery and the rostral branch of the caudal posterior cerebral artery, whereas the ventral temporal part of the hippocampus is served exclusively by the rostral posterior cerebral artery. The amygdala, the periamygdaloid cortex, and the piriform cortex are served by the middle cerebral artery and in part by the perforating arteries. The entorhinal, perirhinal, and postrhinal cortices are vascularized by the posterior and middle cerebral arteries, with a very broad overlap between the distal territories of these vessels. The demonstration of an extensive superimposition between the arterial supply of the entorhinal and the perirhinal regions suggests the presence of anastomotic connections that potentially are protective against ischemic events. Such an arrangement was not observed for the arteries that supply the ventral portion of the hippocampal formation and the basolateral amygdala, which showed nonoverlapping boundaries. The pathophysiological consequences of a similar vascular organization are discussed.     

Cerebral aneurysms and variations in the circle of Willis

In order to obtain information about the relationship between variations in the circle of Willis and aneurysms, 44 complete circles of Willis with aneurysm were studied macroscopically. The incidence of variations was significantly higher in the aneurysm series than in the control circles without aneurysm. There was a definite correlation between asymmetric proximal segments of the anterior cerebral artery and aneurysms of the anterior communicating artery, and a tendency to correlation was found in the case of asymmetric posterior communicating arteries and aneurysms on the internal carotid artery-posterior communicating artery junction. In the light of these findings it seems likely that through hemodynamic changes variation in the circle of Willis plays some role in the development of cerebral aneurysms.     

Histochemical studies of the adrenergic innervation of canine cerebral arteries

The glyoxylic acid method was used to examine the adrenergic innervation of the major cerebral arteries of the circle of Willis in dogs. Fluorescent nerve fibers were observed in whole-mount preparations of the part of the internal carotid artery lying in the cavernosus sinus, the anterior, middle and posterior cerebral arteries, the posterior communicating artery and the basilar artery. Adrenergic nerve fibers were most abundant in the proximal portions of the anterior, middle and posterior cerebral arteries as well as in the posterior communicating artery. The distal smaller arteries were less densely innervated than larger ones. The basilar artery contained a moderate number of fluorescent fibers while the internal carotid artery lying in the cavernosus sinus had a very sparse innervation. The peak wavelengths of the excitation and emission spectra of the fluorescence were analyzed by means of a microepifluorescence spectrophotometer and were about 415 and 465 nm, respectively. These values suggest that the fluorescent fibers are adrenergic ones containing norepinephrine.     

缺血性卒中病变血管和侧支循环代偿的研究

目的 观察缺血性卒中患者的责任病变血管及其侧支循环代偿方式,探讨脑动脉闭塞或严重狭窄时侧支循环的代偿作用与牛津郡社区卒中项目(OCSP)临床症状分型之间的关系.方法对211例缺血性卒中患者采用OCSP分型(完全型前循环梗死36例,部分前循环梗死94例,后循环梗死31例,腔隙性梗死50例),进行数字减影全脑血管造影检查,判定梗死的责任血管、侧支循环是否建立及代偿方式.结果 检出有病变血管的患者198例,共累及病变血管206支,责任血管为颈内动脉98条、大脑中动脉54条、椎动脉27条、颈总动脉6条、基底动脉5条、锁骨下动脉4条、大脑前动脉及大脑后动脉各2条;经Willis环代偿98例,软脑膜支吻合115例,颅外代偿46例.结论脑动脉病变最多位于颈内动脉、大脑中动脉,其次位于椎动脉,前循环病变较后循环病变具有更高的梗死发生率;侧支循环代偿以Willis环最充分,软脑膜支吻合最常见;脑梗死的临床分型受病变血管与侧支循环代偿的综合影响.     

脑梗死患者脑血管病变及侧支循环状态的研究

目的探讨脑梗死患者的责任病变血管及侧支循环主要方式。方法对110例脑梗死患者行牛津郡社区卒中项目（OCSP）分型,进行全脑数字减影血管造影（DSA）检查,判定梗死的责任血管、侧支循环是否存在及方式。结果各型患者,病变血管共137条,其中颈内动脉（ICA）54条（39．4％）、锁骨下．椎动脉（SUB—VA）42条（30．7％）、大脑中动脉（MCA）29条（21．2％）、颈总动脉（CCA）和基底动脉（BA）各4条（各2．9％）、无名动脉2条（1．5％）、大脑前动脉（ACA）和大脑后动脉（PCA）各1条（各0．7％）;责任血管ICA49条（44．5％）、MCA27条（24．5％）、SUB—VA19条（17．3％）及CCA3条（2．7％）、IA2条（1．8％）,余3支血管各1条（均占0．9％）。7例（6．4％）未发现异常。经软脑膜支吻合代偿59例,Willis环代偿49例,来自各种颅外代偿计27例,两种及以上方式联合代偿19例,罕见代偿3例,未发现代偿6例。结论脑动脉病变最多位于ICA、SUB-VA主干,其次位于MCA;前循环病变较后循环病变具有更高的梗死发生率。侧支循环代偿以Willis环最充分,软脑膜支吻合最常见;脑梗死的类型和预后受血管病变和侧支循环状态的综合影响。     

Collateral variations in circle of willis in atherosclerotic population assessed by means of transcranial color-coded duplex ultrasonography

BACKGROUND AND PURPOSE: Transcranial color-coded duplex ultrasonography combined with common carotid artery (CCA) compression can be used to assess the collateral function of the circle of Willis. The aim of this study was to assess the unknown fraction of hemodynamic functional anterior and posterior communicating arteries (AcoA and PcoA, respectively) in an atherosclerotic population with no cerebrovascular symptoms. METHODS: In 76 patients with a mean age of 61 (35 to 89) years, the blood flow velocity changes in the precommunicating parts (A1 and P1, respectively) of the anterior and posterior cerebral arteries were measured during CCA compression. The AcoA was defined as functional if blood flow was reversed in the ipsilateral A1 and enhanced in the contralateral A1 during CCA compression. The PcoA was defined as functional if the flow velocity in the P1 was enhanced >20% during ipsilateral CCA compression. RESULTS: It was possible to assess cross flow through the anterior part of the circle of Willis in 95% of the subjects. Failure of this collateral pathway was caused by a hypofunctional AcoA in 4% and a hypofunctional A1 in 1% of the subjects. Anomalies in the posterior part of the circle of Willis hampering collateral flow from the basilar to the internal carotid artery were found in 45% of the hemispheres. Thirty-eight percent of PcoAs were hypofunctional, and 7% of the posterior cerebral arteries had a persistent fetal anatomy. CONCLUSIONS: We found that in subjects with no cerebrovascular symptoms, the anterior collateral pathway of the circle of Willis was nearly always functional. In contrast, the posterior collateral pathway was nonfunctional in almost half of the total number of hemispheres. Comparing these basic data with data from patients with cerebral ischemic disease might further help to elucidate the importance of the collateral capacity of the circle of Willis.     

Interaction of cardiovascular disease and neurodegeneration: transcranial Doppler ultrasonography and Alzheimer's disease

OBJECTIVE: Recent post-mortem studies have reported that the severity of atheromatous deposits in the circle of Willis is significantly greater, relative to non-demented (ND) elderly persons, in subjects with neuropathologically diagnosed Alzheimer's disease (AD). Additionally, the severity of intracranial atherosclerosis correlates significantly with the densities of neuritic plaques and neurofibrillary tangles. In this study, we examine the arteries of the circle of Willis by transcranial Doppler (TCD) ultrasonography. METHODS: TCD was used to measure, in 25 AD patients and 30 ND elderly subjects, mean flow velocities and pulsatility indices in 16 different segments of the circle of Willis. The data were compared with and without adjustment for age, gender and systolic blood pressure. RESULTS: The AD patients had systematically higher pulsatility indices (p<0.005) than the ND group. Incremental increases of pulsatility indices in these segments had odds ratios ranging from 1.8 to 48 for the presence of AD when adjusted for age, gender and systolic blood pressure. The left internal carotid artery siphon and the left posterior cerebral artery were the two vessels that were strongly associated with AD diagnosis. Mean flow velocities were generally lower in patients with AD but the differences did not reach the significance level. DISCUSSION: The pulsatility indices of the arteries of AD patients were generally greater than those of similarly-aged ND subjects. This difference is most likely due to increased arterial wall rigidity imposed by atherosclerotic changes. Atherosclerotic disease of intracranial arteries may be a risk factor for AD.     

Ventral porencephaly: A cerebral defect associated with multiple congenital anomalies

Summary An infant with multiple congenital anomalies was found at autopsy to have a porencencephalic defect on the ventral surface of the left frontal lobe. The intracranial defect was seen in association with an anomalous configuration of the circle of Willis. The zone of tissue destruction corresponded to the vascular territory of the anterior choroidal and lenticulo-striate branches of the proximal middle cerebral arteries, which were absent on the left. The developmental anomaly of the circle of Willis may have predisposed to tissue destruction by compromising cerebral perfusion at midgestation, a stage of rapid brain growth.Supported in part by USPS grants. M 16674, MH 25515 and MH 01215     

Visualization of the Cerebral Circulation Using Three–dimensional Transcranial Power Doppler Ultrasound Imaging

The purpose of this study was to evaluate the anatomy of the cerebral circulation, particularly the circle of Willis, using three–dimensional ultrasound (3DUS) imaging. Image data were obtained through the right transtemporal window from 8 young, healthy volunteers by acquiring gray–scale and color Doppler spectral (CDI) and energy (CDE) images using two–dimensional ultrasound equipment with a 2–MHz probe. Images and transducer position coordinates were fed into a graphics workstation, reprojected, analyzed to extract the blood flow signal, volume rendered, and displayed interactively. The architecture of the cerebral circulation was evaluated from multiple orientations using stereo viewing glasses and rotation to enhance the understanding of vessel position. The primary vessels of the cerebral circulation including the circle of Willis and bilateral views of the branching arteries (middle, anterior, and posterior cerebral arteries and internal carotid artery) could be imaged readily with 3DUS through one transtemporal window. Acquisition time was typically less than 30 seconds. Volume–rendering methods greatly assisted in showing the overall spatial relationships and continuity of cranial vessels. Secondary branches of the cerebral arteries were seen in 2 patients. Color data from two–dimensional ultrasound imaging that otherwise might be identified as artifact was found to represent continuous small vessels on three–dimensional viewing. 3DUS facilitates imaging of cranial vascular anatomy by clarifying overall spatial relationships and enhancing comprehension, compared to two–dimensional ultrasound methods. The method is rapid and the circle of Willis can be visualized from one side of the head.     

Collateral configuration of the circle of Willis: transcranial color-coded duplex ultrasonography and comparison with postmortem anatomy

BACKGROUND AND PURPOSE: The anterior communicating artery (AcoA) and posterior communicating arteries (PcoA) of the circle of Willis provide the main route for collateral blood flow in cases of carotid artery obstruction. Transcranial color-coded duplex ultrasonography (TCCD) allows real-time measurement of the collateral function of the AcoA and PcoA. The primary objective of this study was to determine the collateral artery threshold diameters for supplying collateral flow. METHODS: In 12 acute stroke patients with a median age of 75 years (51 to 91 years), the collateral integrity of the circle of Willis as assessed by TCCD and carotid compression tests was compared with their postmortem anatomy. The lengths and diameters of the collateral arteries were measured. RESULTS: TCCD demonstrated absent anterior collateral flow in 3 patients. In 1 of these patients, absence of anterior cross-flow was due to an occluded anterior cerebral artery, which was revealed at autopsy. Absent posterior collateral flow was found in 14 hemispheres. In 2 of these hemispheres, autopsy revealed a fetal configuration of the posterior cerebral artery hampering posterior collateral flow. The median (range) diameters as found at autopsy of the functional (n=19) and nonfunctional (n=16) collateral arteries of the circle of Willis were 1.1 (0.4 to 2.0) and 0.5 (0.3 to 0.7) mm, respectively (P=0.003). PcoA diameters were found to correlate negatively (rho=-0. 50, P=0.01) to the diameters of their accessory P1 segments. CONCLUSIONS: The threshold diameter allowing for cross-flow through the primary collateral arteries of the circle of Willis is between 0. 4 and 0.6 mm.     

Pathological and immunohistochemical findings of an autopsy case of adult moyamoya disease

Moyamoya disease is vaso‐occlusive disease involving the arteries of the circle of Willis that is accompanied by a compensatory recruitment of a vascular network. The pathological and immunohistochemical findings of an autopsy case of hemorrhagic moyamoya disease in a 69‐year‐old woman are described in the present report. The autopsy findings of the brain revealed cerebral and intraventricular hemorrhage with edema. The left anterior cerebral artery, bilateral middle cerebral arteries and left posterior cerebral artery were marked narrowing, and the other arteries revealed mild narrowing. Microscopically, the arteries of the circle of Willis showed narrowed lumen, fibrocellular intimal thickening, marked tortuousness of internal elastic lamina and attenuation of media. The thickened intima was composed of smooth muscle cells. The vessels with dilated or irregular‐shaped lumen suggested abnormal vascular networks demonstrated by angiography. In this case, no correlation between the abnormal vascular network and expression of VEGF or VEGF receptor was disclosed. It was hypothesized that abnormal vascular networks might be composed of collateral vessels in relation to various pathological changes of the arteries, such as occlusion and stenosis, and intracranial hemorrhage in patients with moyamoya disease might occur as a result of rupture of arteries including abnormal vascular networks.