Artery Dolichoectasia Research Articles

Ocular neuromyotonia

AbstractOcular Neuromyotonia (ONM) is rare disorder characterized by intermittent diplopia, lasting minutes or seconds, provoked by brief, episodic contraction of muscles supplied by the oculomotor, trochlear, or abducens nerve. These episodes are induced by change in the gaze direction or after holding eccentric gaze. Previous radiation therapy of the sellar or parasellar regions is the most frequent cause. Long standing compression of the oculomotor nerve, by tumor, supraclinoid aneurysms, arachnoiditis, basilar artery dolichoectasia, and others have been reported. Although the exact pathogenesis of ONM is still unknown, there is wide acceptance of the segmental demyelination hypothesis.This presentation will review the clinical presentation, discuss diagnosis work‐up as treatment options.

In vivo assessment of rapid cerebrovascular morphological adaptation following acute blood flow increase

Pathological extremes in cerebrovascular remodeling may contribute to basilar artery (BA) dolichoectasia and fusiform aneurysm development. Factors regulating cerebrovascular remodeling are poorly understood. To better understand hemodynamic influences on cerebrovascular remodeling, we examined BA remodeling following common carotid artery (CCA) ligation in an animal model. Rabbits were subjected to sham surgery (3 animals), unilateral CCA ligation (3 animals), or bilateral CCA ligation (5 animals). Transcranial Doppler ultrasonography and rotational angiography were used to compute BA flow, diameter, wall shear stress (WSS), and a tortuosity index on Days 0, 1, 4, 7, 14, 28, 56, and 84. Basilar artery tissues were stained and analyzed at Day 84. Statistical analysis was performed using orthogonal contrast analysis, repeated measures analysis of variance, or mixed regression analysis of repeated measures. Statistical significance was defined as a probability value < 0.05. Basilar artery flow and diameter increased significantly after the procedure in both ligation groups, but only the bilateral CCA ligation group demonstrated significant differences between groups. Wall shear stress significantly increased only in animals in the bilateral CCA ligation group and returned to baseline by Day 28, with 52% of WSS correction occurring by Day 7. Only the bilateral CCA ligation group developed significant BA tortuosity, occurring within 7 days postligation. Unlike the animals in the sham and unilateral CCA ligation groups, the animals in the bilateral CCA ligation group had histological staining results showing a substantial internal elastic lamina fragmentation. Increased BA flow results in adaptive BA remodeling until WSS returns to physiological baseline levels. Morphological changes occur rapidly following flow alteration and do not require chronic insult to affect substantial and significant structural transformation. Additionally, it appears that there exists a flow-increase threshold that, when surpassed, results in significant tortuosity.

Basilar Artery Dolichoectasia Is Associated with Paramedian Pontine Infarction

Background: Basilar artery (BA) dolichoectasia (dilatative arteriopathy) (BAD) causes arterial elongation and enlargement with subsequent hemodynamic changes leading to thrombosis, microembolization and brainstem compression. Little is known about the association between BAD and the pattern of pontine infarct in patients without BA stenosis. Methods: We studied patients with isolated pontine infarcts recruited from a stroke registry. The patients with pontine infarction were divided into 2 groups based on the location of the lesion on magnetic resonance imaging as follows: paramedian pontine infarct (PPI) and lacunar pontine infarct (LPI). We compared the vascular findings and risk factors in the patients with the 2 types of infarcts. Using modified imaging criteria, we compared the following dolichoectatic components of the BA: (1) ectasia, (2) lateral displacement and (3) height. Results: There were 96 patients (45 women, 51 men). Thirty-five patients had PPI and 61 had LPI. Ectasia of the BA was more frequent in the PPI group than in the LPI group (31.4 vs. 11.5%; p = 0.016). Ectasia of the BA was associated with an elevated odds ratio in the patients with PPI (odds ratio 5.80, 95% CI 1.66–20.21) by multivariate analysis. Conclusion: Ectasia of the BA other than elongation or angulation appears to contribute to the occurrence of PPI. These findings may be helpful in predicting certain types of stroke.

Dilative arteriopathy and basilar artery dolichoectasia complicating late-onset Pompe disease

Strokes related to intracranial aneurysm or arteriopathy have been reported in a few patients with late-onset Pompe disease. These reports suggested that cerebral vessel involvement could be an underrecognized complication of this disease. We report cerebral artery involvement in three French patients with late-onset Pompe disease. The first patient died at age 35 years from complications of a giant fusiform aneurysm of the basilar artery, and her 34-year-old sister showed evidence of dolichoectatic basilar artery on magnetic resonance angiography. A dilative arteriopathy complicated with carotid artery dissection was diagnosed in the third patient, aged 50 years. Two patients are currently being treated with enzyme replacement therapy (alglucosidase alfa), and regular angiographic follow-up showed the absence of progression of vascular abnormalities in one of them. These observations, combined with previously reported cases, confirm that Pompe disease should be recognized as a predisposing condition to dilative arteriopathy and cerebral aneurysm formation, although the real incidence of these vascular complications remains unknown.

Medulla compression caused by vertebral artery dolichoectasia

A 75-year-old man was admitted with recently developed hiccough and a 1 year history of ataxia. He denied drug overuse, stroke, head trauma and a family history of ataxia. He...

Oligosymptomatic and giant basilar artery dolichoectasia discovered after a stroke: case report

The most frequently diagnosed complication of vertebrobasilar dolichoectasia (VBD) is the compression of structures adjacent to the vertebral and basilar arteries. A giant VBD with only slight compressive symptoms is unusual. In this setting, the diagnosis of VBD may be casually revealed after the occurrence of a posterior circulation stroke, another potential complication. We report a 48-year-old woman who presented a two-month history of continuous buzz and a slight right-sided hearing loss that was followed by a cerebellar ischemic stroke. Brain CT and MRI revealed a marked compression of the brainstem due to an ectatic, tortuous and partially thrombosed basilar artery (BA). The largest cross-sectional diameter of BA was 18 mm. The patient had a good functional recovery within the two-month follow-up after stroke with modified Rankin scale score (mRSS)=2. At the one-year follow-up, patient still kept the complaints of continuous buzz, slight right-sided hearing loss and the mRSS was the same. We call attention for an unusual giant VBD that caused an impressive brainstem compression with displacement of important structures in an oligosymptomatic patient. Diagnosis was made only after the occurrence of a stroke. Despite of the good functional recovery after stroke, the presence of significant atherosclerotic changes and the large BA diameter may indicate a poor outcome. However, after one year, she remains oligosymptomatic.

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.

Basilar artery dolichoectasia in a boy with a combination of partial monosomy 18p and partial trisomy 20q

We describe an 11-year-old boy with facial dysmorphism consisting of a round and flat face, hypertelorism, short nose, and down turned corners of the mouth. In addition, he had severe mental retardation, short stature, imperforate anus, and basilar artery dolichoectasia. Cytogenetic evaluation revealed an unbalanced paternally inherited translocation t(18;20)(p11.2q13.3), resulting in partial monosomy 18p and partial trisomy 20q. The combination of deletion 18pduplication 20q has not been previously described and we suggest that the unusual finding of basilar artery dolichoectasia may be a feature of one of the imbalances.

Basilar Artery Diameter and 5-Year Mortality in Patients With Stroke

Few and conflicting data exist on the case fatality rate in stroke patients with basilar artery dolichoectasia. We analyzed basilar artery characteristics (diameter, height of bifurcation, transverse position) and 5-year mortality (all-cause, nonstroke vascular, and stroke) in patients with brain infarction. The basilar artery diameter was measured with a 16-diopter lens in 466 consecutively recruited patients with brain infarction confirmed by magnetic resonance imaging. The height of the bifurcation and the transverse position of the basilar artery were assessed on semiquantitative scales. Patients were followed up for a median of 5.3 years (range, 1.5 to 6.6) and were classified as having had stroke, nonstroke vascular, and nonvascular death according to the French national registry of death certificates. Of the 157 deaths, 88 were vascular (including 54 stroke deaths). Basilar artery diameter was associated with an increased 5-year stroke mortality rate but not with all-cause or nonstroke vascular mortality. The adjusted hazard ratio (HR) of stroke mortality per 1-mm increase in basilar artery diameter was 1.23 (95% confidence interval [CI], 1.07 to 1.41). A higher risk of stroke death was associated with basilar artery diameter at the 95th percentile (diameter >4.3 mm; adjusted HR 3.69; 95% CI, 1.63 to 8.38) and the height of bifurcation (adjusted HR for score >1, 2.08; 95% CI, 0.93 to 4.68) but not with transverse position. Basilar artery diameter was independently associated with cerebrovascular mortality. A diameter >4.3 mm may be a marker for a high risk of fatal stroke.

Concurrent dolichoectasia of basilar and coronary arteries

We read with great interest the article by Pico et al.1 concerning combined dolichoectasia of basilar artery (BADE) and coronary artery (CAE). We also encountered two patients with those arterial abnormalities and considered possible genetic and acquired profile in BADE and CAE. Sixty-four subjects (60 men and 4 women) were diagnosed with BADE on physical and brain check-up, including brain MRI and three-dimensional time-of-flight MR angiography (1.5 T; Hitachi Medical), in PL Tokyo Health Care Center, Japan. Mean age (SD) of subjects was 59.5 (11.2) years. Two non-related men had BADE and CAE without abdominal aortic aneurysm (AAA). Their common clinical aspects revealed younger onset (less than 40 years) and grade 2 of hypertension, hypercholesterolemia, chain smoking, and obesity. An 80-year-old man was treated …

Isolated IVth (Trochlear) Nerve Palsy due to Basilar Artery Dolichoectasia

Dolichoectasia (elongation, dilatation and tortuosity) of the basilar artery can cause an isolated cranial neuropathy. The trigeminal nerve and facial nerve are most frequently affected. Dysfunction of one of the ocular motor cranial nerves due to basilar artery dolichoectasia is uncommon, and an isolated IVth (trochlear) nerve palsy has not been previously described in the literature. Two men, ages 70 and 59 years, respectively, presented with vertical diplopia due to a IVth nerve palsy. In one patient, the onset of the IVth nerve palsy was painless and gradual and in the other patient, the onset was acute and associated with periorbital pain. Neuroimaging in both patients revealed pathological tortuosity of the basilar artery around the midbrain and displacement of the artery toward the side of the affected trochlear nerve. The patients were observed clinically. One patient had gradual worsening of his palsy for three and one-half years then suffered a stroke. The second patient whose IVth nerve palsy had an acute onset experienced spontaneous resolution of his palsy but later developed dysfunction of other cranial nerves. Basilar artery dolichoectasia should be considered in the differential diagnosis of an isolated IVth nerve palsy. The clinical course may be variable, and the prognosis is not always benign.

Basilar artery dolichoectasia with partial thrombosis

Basilar artery dolichoectasia with partial thrombosis

椎骨動脈の脳幹圧迫により生じた舌咽・迷走神経麻痺

We report a 43-year-old male-to-female transsexual patient with gender identity disorder (GID), suffering from neurovascular compression causing glossopharyngeal and vagal nerve palsy. Cross-sex hormone therapy has been approved for 5 years. He had complained of difficulty inn speaking and singing for 3 years, with symptoms of right soft palate palsy, lower sensitivity of the right pharynx and curtain sign. However, recurrent nerve palsy was not identified so his vocal cord mobility was good. We investigated brain MRI and MRA, and detected dolichoectasia of the right vertebral artery on the medulla. We suspected that the neural symptoms on the same side were caused by the vertebral artery dolichoectasia compressing his glossopharyngeal and vagal nerve on the medulla.

Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta

Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta

Concurrent dolichoectasia of basilar and coronary arteries

Concurrent dolichoectasia of basilar and coronary arteries

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.

Isolated abducens nerve palsy caused by contralateral vertebral artery dolichoectasia

Isolated abducens nerve palsy caused by contralateral vertebral artery dolichoectasia

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 …