Intracranial Arterial Dolichoectasia Research Articles

English

Introduction Anomalies and variations of the anterior communicating artery are common. Fenestration has been reported as the most common variation of this artery. Dolichoectasia of intracranial arteries is a rare arteriopathy characterised by elongation and widening of the arteries, mostly involving vertebral and basilar arteries. This report discusses large anterior communicating artery fenestration associated with posterior circulation anomalies. Case Report We report here a rare case of large anterior communicating artery fenestration associated with mild dolichoectasia of the basilar artery and marked dolichoectasia of the left posterior inferior cerebellar artery, forming a large vascular loop at the area of cisterna magna, as well as hypoplasia of the left anterior inferior cerebellar artery. The study discusses the details of this case. Conclusion Dolichoectasia of intracranial arteries is a rare arteriopathy characterised by elongation and widening of the arteries and disturbance of the laminar blood flow, mostly involving vertebral and basilar arteries.

Vertebral and/or basilar dolichoectasia in human adult cadavers

Intracranial arterial dolichoectasia is a condition in which arteries demonstrate an increase in length and diameter, with the vertebrobasilar system being the most commonly affected segment. Because the criteria for and degree of vertebrobasilar dolichoectasia are usually established on three-dimensional time-of-flight MR angiography, we presented the results of an anatomic study of vertebrobasilar dolichoectasia cadaveric specimens. Examination was carried out on the brains of human adult cadavers, routinely dissected at the Institute of Forensic Medicine. Measurement of the outer diameter and length of vertebral and/or basilar arteries was performed using the ImageJ processing program. There were 14 cases (14/216) of vertebral and/or basilar (dolicho)ectasia. Their classification into special (sub)types is made according to the basilar and/or vertebral diameter. The basilar length ranged from 32.91-59.37mm, and the basilar outer diameter ranged from 3.51-8.92mm in relation to the corresponding point of its measurement. The outer diameter of the vertebral arteries ranged from 0.67-5.91mm. The Games-Howell post hoc test additionally showed that a basilar outer diameter of grade III and IV was significantly larger than in grade II (p < 0.05). We noted a predisposition of males older than 40 years to arterial dolichoectasia in the vertebrobasilar system, independently from population group, as well as its asymptomatic appearance, independently from the presence of atherosclerotic plaques.

Dolichoektazja tętnic koła tętniczego Willisa i tętniak wrzecionowaty tętnicy podstawnej mózgu – opis przypadku i przegląd piśmiennictwa

Dolichoectasia of intracranial arteries is a rare arteriopathy characterized by elongation and widening of the arteries and disturbance of the laminar blood flow. It involves mostly vertebral and basilar arteries. In advanced cases, formation of a fusiform aneurysm is possible. A sixty-four-year-old female with hypertension was admitted to the hospital with severe non-systemic vertigo and dysarthria, which had lasted for a couple of weeks. Imaging of the brain revealed dolichoectasia of arteries of the circle of Willis coexisting with a fusiform aneurysm of the basilar artery. Intracranial arterial dolichoectasia may be asymptomatic for a long time. However, in many cases it leads to neurological symptoms associated with haemodynamic disturbance (due to unstable wall clots) and mass effect caused by the widened vessel.

Unruptured Middle Cerebral Artery Aneurysm Associated With a Duplicated Middle Cerebral Artery and a Dolichoectasic Anterior Cerebral Artery

Duplicated middle cerebral artery (DMCA) has been reported in 0.7%-2.9% of autopsy cases, and sme reports have described aneurysms in the MCA. Both ruptured and unruptured aneurysms in the MCA have been reported, often requiring surgical intervention. During surgery for aneurysm in DMCA, care must be taken to avoid damaging the MCA, which supplies the territory of the temporopolar artery and the anterior and/or middle temporal artery. Regarding dolichoectasia of intracranial arteries, a relationship between dolichoectasia and ischemic brain lesions, especially lacunar infarctions, has been noted. We report a case with unruptured MCA aneurysm associated with DMCA and a dolichoectasic anterior cerebral artery. In this case, surgical intervention was not prioritized because of the narrowed and calcified parent artery of the aneurysm. In selecting treatment for a patient with multiple vascular anomalies, the pathophysiology of each anomaly should be estimated carefully.

Matrix metalloproteinase‐3 and intracranial arterial dolichoectasia

Intracranial arterial dolichoectasia (IADE), also called dilatative arteriopathy of the brain, is defined as an increase in length and diameter of intracranial arteries. Abdominal aortic aneurysm and ectasia of coronary arteries have been reported in association with IADE. In both conditions, a dysfunction of matrix metalloproteinases (MMP)-2, -3, and -9 have been found. Our aim was to investigate these MMP pathways in stroke patients with IADE. Five hundred ten Caucasians patients with brain infarction were consecutively recruited at 12 centers. The diagnosis of IADE was made by consensus between 2 neurologists based on magnetic resonance imaging scans. Determination of MMP-2, -3, and -9 plasma levels was centralized in 1 laboratory. Because we found a threshold effect of MMP-3 plasma levels with the risk of IADE, determination of the MMP-3 5A/6A polymorphism was carried out. IADE was identified in 12% of stroke patients. There was no association of IADE with mean MMP-2, -3, and -9 plasma levels. After categorization of MMP plasma levels into tertiles, we found a higher risk of IADE with the lowest MMP-3 tertile (adjusted odds ratio [OR], 2.48; 95% confidence interval [CI], 1.17-5.23). In genotype analysis, there was a significant additive effect of the 5A allele on the risk of IADE, with an adjusted OR of 1.62 (95% CI, 1.03-2.55). In this cohort of stroke patients of Caucasian ancestry, IADE was associated with low MMP-3 plasma levels and with the 5A/6A polymorphism of the promoter region of MMP-3. These results suggest that MMP-3 may play a role in IADE.

Association of Small-Vessel Disease With Dilatative Arteriopathy of the Brain

Clinical and imaging studies have reported an independent and intriguing association between intracranial arterial dolichoectasia (IADE) and markers of small-vessel disease (SVD) such as lacune(s). We used a large brain-autopsy collection to investigate the relation between IADE and pathologically assessed cerebral SVD. The entire arterial tree from the heart to the intracranial small intracerebral arteries was investigated in 381 consecutive autopsies from patients with stroke. Vascular risk factors, heart status (weight, coronary atherosclerosis, and myocardial infarction), prevalence and severity of atherosclerosis from heart to brain (aortic arch, carotid, vertebral, and intracranial arteries), dolichoectasia, cerebral SVD, and brain tissue lesions (lacune(s), état criblé) were evaluated. Analyses were adjusted for age, sex, and heart weight. Twenty-three (6%) of 381 patients had IADE, affecting mainly the basilar artery, with a median basilar artery diameter of 6 mm (range, 4 to 9 mm). Patients with IADE had a >2-fold increase in the prevalence of basilar artery plaques and ulcerated plaques in the aortic arch (both P=0.006), but there were no associations with coronary or cervical artery plaques. SVD was more frequent in IADE-positive than in IADE-negative patients (45% vs 18%; P=0.004). The adjusted odds ratio was 3.85 (95% confidence interval, 1.56 to 9.52). Cerebral amyloid angiopathy was not observed in IADE-positive patients. Compared with stroke patients without IADE, those with IADE were more likely to have pathologic evidence of cerebral SVD and its consequences, independent of age, arterial hypertension, or diabetes mellitus. IADE and cerebral SVD may have unidentified biologic processes in common.

Middle cerebral artery dolichoectasia in a young woman with a previous stroke

We observed a 32-year-old female who had suffered from a left hemisphere ischemic stroke with right hemiparesis at the age of seven. At that time, a CT scan demonstrated a left ischemic lesion in nucleo-capsular region and a cerebral angiogram documented a complete occlusion of the supraclinoid segment of the internal carotid artery. When we observed the patient neurological examination demonstrated a moderate right brachio-crural hemiparesis. A brain MRI showed an old ischemic lesion involving the left nucleo-capsular and 'flow voids' suggestive for a vascular malformation in the left sylvian region. A cerebral rotational angiogram with 3-D reconstructions demonstrated a dolichoectatic left middle cerebral artery with an unusual 'corkscrew' aspect. Middle cerebral artery dolichoectasia is a rare pathological condition that may manifest with a stroke. The patients with intracranial arterial dolichoectasia (IADE) are most often hypertensive elderly men, and, to the best of our knowledge, an ischemic stroke associated with IADE has never been reported in children.

Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta

Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta

Intracranial arterial dolichoectasia and small‐vessel disease in stroke patients

Two studies found that stroke patients with intracranial arterial dolichoectasia (IADE) had lacunar infarction more often than the control subjects. The relation between IADE and other manifestations of small-vessel disease (multilacunar state, leukoaraiosis, and état criblé) was not investigated. The magnetic resonance images of 510 patients with brain infarction in the Etude du Profil Genetique de l'Infarctus Cerebral (GENIC) study were evaluated for the diagnosis of multilacunar infarction (>1 lacunar infarct), leukoaraiosis (assessed in periventricular and subcortical regions according to Scheltens and colleagues' scale, with severe leukoaraiosis defined as a score > 8), état criblé (using a semiquantitative score ranging from 0-8, with severe EC > 4), and IADE (using the consensus method). By comparison with stroke patients without IADE (n = 447), IADE(+) stroke patients (n = 63) had significantly more frequent multilacunar state (51 vs 33%), severe leukoaraiosis (34 vs 19%), and severe état criblé (32 vs 12%). After adjustment for potential confounding factors, the odds ratios (95% confidence interval) for the presence of IADE were 2.05 (1.08-3.87) for multilacunar state, 2.40 (1.11-5.20) for severe leukoaraiosis, and 3.60 (1.43-9.08) for severe état criblé. The factors significantly associated with état criblé were older age and a familial history of stroke. IADE in stroke patients was independently associated with parenchymal manifestations of small-vessel disease. These conditions may have in common underlying pathophysiological processes.

Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta

Intracranial arterial dolichoectasia (IADE) is defined as an increase in length and diameter of the intracranial arteries and is present in 12% of stroke patients. The pathophysiology is unknown; some data suggest that IADE is not merely a complication of atherosclerosis, but a distinct arteriopathy characterized by loss of elastic tissue in the media. To investigate the relationship between IADE and transesophageal echocardiography (TEE) variables such as ascending and descending thoracic aorta diameters. The sample included 154 patients with brain infarction (BI) and with measurement of the descending thoracic aorta on TEE. IADE was diagnosed by consensus between two neurologists. Information on demographic characteristics and risk factors was collected using a structured questionnaire, and a carotid ultrasound scan was performed. IADE was identified in 23 of the 154 stroke patients (15%). The mean diameter (+/-SD) of the descending thoracic aorta was significantly higher in the IADE(+) than in the 131 IADE(-) stroke patients (mean +/- SD 26.6 +/- 3.6 vs 24.8 +/- 2.7 mm). The proportion of IADE increased regularly with the quartiles of descending thoracic aorta diameter: 5%, 13%, 18%, and 24% (test of linear trend, p = 0.02). The adjusted OR (95% CI) of IADE associated with a 1 mm increase in descending thoracic aorta diameter was 1.22 (95% CI, 1.02 to 1.45). Patients with intracranial arterial dolichoectasia (IADE) have larger descending thoracic aorta diameters than non-IADE patients, suggesting that the underlying process causing IADE also affects the descending thoracic aorta.

December 14 Highlights

Koton et al. studied the association between exposure to seven potential triggers and ischemic stroke onset. Thirty-eight percent of patients reported exposure to at least one potential trigger during the 2-hour hazard period. Triggers with elevated odds ratios: negative emotions; anger; and sudden changes in body posture in response to a startling event. see page 2006 In the years after patients have carotid endarterectomy (CE), they remain at risk for ischemic vascular events. Betancourt et al. assessed the use of medications proven to reduce vascular risk in the 1-year period following CE. Use of statins, angiotensin-converting enzyme inhibitors, and antiplatelet agents was suboptimal. see page 2011 In this MRI case control study of stroke patients, Pico et al. showed that intracranial arterial dolichoectasia was associated with enlarged descending thoracic aorta by TEE with a dose response relationship. The underlying process causing intracranial arterial dolichoectasia may affect the descending thoracic aorta. see page 2016 Soelberg Sorensen et al. treated 76 MS patients who had suffered an acute relapse with either IV immunoglobulins (IVIG) 1g/kg or placebo 24 hours before treatment with IV methylprednisolone. Both groups improved, but without any difference between …

The vasculopathy of autosomal dominant polycystic kidney disease: Insights from animal models

A 37-year-old white man with autosomal dominant polycystic kidney disease (ADPKD) was admitted to Boston's Beth Israel Hospital with sudden severe headache of two-hour duration. He was unconscious on admission. Emergency angiography revealed a ruptured 15 mm cerebral aneurysm. Emergency surgery to clip the aneurysm was unsuccessful, and the patient died 36 hours after admission. His serum creatinine was 3.2 mg/dL on admission; the rest of his laboratory values were within normal limits. A request for an autopsy was denied. At age 20, the patient and his then 15-year-old sister had abdominal ultrasound examinations that revealed bilateral renal cysts, consistent with the diagnosis of ADPKD. At age 32, the patient had a sudden, relatively severe, acute myocardial infarction (MI). Cardiac catheterization revealed three-vessel disease, and he subsequently underwent a triple coronary artery bypass operation. Throughout his medical course, the patient's blood pressure was in the 120/80 to 140/80 mm Hg range while he was taking lisinopril, 10 mg/day, which was started after his MI. The patient's mother has ADPKD and end-stage renal disease, and she is on hemodialysis. She recently underwent unsuccessful renal transplantation. There is no known family history of cerebral aneurysms. However, the mother had two cerebrovascular accidents and recovered from both. The patient's father had an MI at age 32, but he lived to age 64. A paternal uncle also died at an early age of an MI. The patient's younger sister was later screened for cerebral aneurysms and the test was negative. The patient's two young children have not been screened for ADPKD.

Dolichoectasia of the intracranial arteries in patients with first ischemic stroke: a population-based study.

The objective of this study was to estimate the frequency of intracranial arterial dolichoectasia among patients with first ischemic stroke and to compare clinical characteristics, survival, and recurrence in those with and without the abnormality. Dolichoectasia may cause cerebral infarction by thrombosis, embolism, stenosis, or occlusion of deep penetrating arteries. The chi-square, Fisher's exact, and logrank tests were used to compare clinical characteristics, survival, and recurrence for patients with and without dolichoectasia among the 387 residents of Rochester, MN, who had brain CT or MRI for first cerebral infarction from 1985 through 1989. Twelve patients (3.1%) had dolichoectasia. Patients with dolichoectasia were more likely to have had stroke fitting a clinical and radiographic pattern of lacunar infarction than those without (42% and 17% respectively; p=0.04). Dolichoectasia was detected in the vertebrobasilar system in eight patients (66.7%), in the carotid system in two patients (16.7%), and in both circulatory systems in two patients (16.7%). There were no significant differences in the following characteristics among those with and without dolichoectasia: age, sex, hypertension, diabetes, smoking, and preceding transient ischemic attack. Patients with dolichoectasia had better survival (relative risk [RR] for death, 0.26; p=0.04) after first cerebral infarction but higher rates of stroke recurrence (RR, 2.4; p=0.02). Dolichoectasia is detected in 38 of patients with first cerebral infarction and is associated with better survival but higher rates of stroke recurrence.

Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder with a variety of cardiovascular manifestations. This study presents a group of patients with ADPKD who had intracranial arterial dolichoectasia. One hundred seventy-eight ADPKD patients were screened with magnetic resonance angiography, 40 ADPKD patients had conventional angiography, and 98 ADPKD patients underwent a brain autopsy. For comparison, 360 patients without ADPKD who had magnetic resonance angiography and conventional angiography or brain autopsy were also studied. The prevalence of asymptomatic intracranial arterial dolichoectasia was 2.2% (4 of 178), 2.5% (1 of 40), and 2.0% (2 of 98) in the three ADPKD groups, respectively. None of the patients without ADPKD had intracranial arterial dolichoectasia. In addition to the seven patients with asymptomatic disease, two ADPKD patients with vertebrobasilar dolichoectasia had posterior circulation ischemic symptoms. The mean age of the nine patients (five men and four women) was 56.6 yr (range, 41 to 67 yr). The posterior circulation was involved in five patients, the anterior circulation was involved in two patients, and both were involved in two patients. Arterial dissection was believed to have caused middle cerebral artery dolichoectasia in one patient, and intracranial arterial dissections were strongly suspected in two other patients. Six of the nine patients with intracranial arterial dolichoectasia had additional vascular manifestations of ADPKD. Some patients with ADPKD are at an increased risk of developing intracranial arterial dolichoectasia and dissections. Recognizing this association is important because (1) it may be a cause of stroke; (2) it may mimic a saccular aneurysm on radiographic studies; and (3) it suggests that the arteriopathy of ADPKD may be more generalized than previously believed.

Dolichoectatic Intracranial Arteries: Review of Selected Aspects

Dolichoectasia of intracranial arteries may cause a variety of neurological problems including cranial nerve and brain tissue compression, hydrocephalus, and cerebral ischemia. Different mechanisms ma