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Background and Purpose Methods Results Conclusion Brain arterial luminal diameters are reliably measured with automated imaging software. Nonautomated imaging software alternatives such as a Picture Archiving Communication System are more common bedside tools used for manual measurement. This study is aimed at validating manual measurements against automated methods.We randomly selected 600 participants from the Northern Manhattan Study (NOMAS) and 260 participants from the Atahualpa Project studied with 1.5 Tesla MR angiography. Using the Radiant measuring tool, three independent readers (general practitioner, neurology resident, and vascular neurologist) measured manually the diameter of arterial brain vessels. The same vessels were also measured by LKEB Automated Vessel Analysis (LAVA). We calculated the intraclass correlation coefficient (ICC) of each rater's diameters versus those obtained with LAVA.The ICC between diameters obtained by the general practitioner or the neurology resident compared to LAVA was excellent for both internal carotid arteries (ICA) and Basilar Arteries (BA) (ICC > .80 in all comparisons) in NOMAS. In the Atahualpa Project, ICC between diameters obtained by a vascular neurologist and LAVA was good for both ICA and BA (ICC > .60 in all comparisons). The ICCs for the measurements of the remaining arteries were moderate to poor.Results suggest that manual measurements of ICA and BA diameters, but not MCA or ACA, are valid and could be used to identify dilated brain arteries at the bedside and for eventual selection of patients with dolichoectasia into clinical trials. [ABSTRACT FROM AUTHOR]

Background: Intracranial vessel tortuosity is a key component of dolichoectasia and has been associated with atherosclerosis and adverse neurologic outcomes. However, the evaluation of tortuosity is mainly a descriptive assessment. Purpose: To compare the performance of three automated tortuosity metrics (angle metric [AM], distance metric [DM], and distance‐to‐axis metric [DTA]) for detection of dolichoectasia and presence of segment‐specific plaques. Study Type: Observational, cross‐sectional metric assessment. Population: 1899 adults from the general population; mean age = 76 years, female = 59%, and black = 29%. Field Strength/Sequence: 3‐T, three‐dimensional (3D) time‐of‐flight MRA and 3D vessel wall MRI. Assessment: Tortuosity metrics and mean luminal area were quantified for designated segments of the internal carotid artery, middle cerebral artery, anterior cerebral artery, posterior cerebral artery, vertebral artery, and entire length of basilar artery (BA). Qualitative interpretations of BA dolichoectasia were assessed based on Smoker's visual criteria. Statistical Tests: Descriptive statistics (2‐sample t‐tests, Pearson chi‐square tests) for group comparisons. Receiver operating characteristics area under the curve (AUC) for detection of BA dolichoectasia or segment‐specific plaque. Model inputs included 1) tortuosity metrics, 2) mean luminal area, and 3) demographics (age, race, and sex). Results: Qualitative dolichoectasia was identified in 336 (18%) participants, and atherosclerotic plaques were detected in 192 (10%) participants. AM‐, DM‐, and DTA‐calculated tortuosity were good individual discriminators of basilar dolichoectasia (AUCs: 0.76, 0.74, and 0.75, respectively), with model performance improving with the mean lumen area: (AUCs: 0.88, 0.87, and 0.87, respectively). Combined characteristics (tortuosity and mean luminal area) identified plaques with better performance in the anterior (AUCs ranging from 0.66 to 0.78) than posterior (AUCs ranging from 0.54 to 0.65) circulation, with all models improving by the addition of demographics (AUCs ranging from 0.62 to 0.84). Data Conclusion: Quantitative vessel tortuosity metrics yield good diagnostic accuracy for the detection of dolichoectasia. Level of Evidence: 1 Technical Efficacy Stage: 2 [ABSTRACT FROM AUTHOR]

Abstract Background Dolichoectasia is a rare arterial condition characterized by the dilatation, tortuosity, and elongation of cerebral blood vessels. The vertebrobasilar artery and internal carotid artery are the common sites of dolichoectasia. However, dolichoectasia of the branch arteries, such as the ophthalmic artery (OA), is extremely rare. To the best of our knowledge, this is the first case of ophthalmic dolichoectasia that was successfully treated with endovascular internal coil trapping. Case presentation A 54-year-old female patient presented with transient left ophthalmalgia and visual disturbance. Magnetic resonance imaging revealed a dilated and elongated left OA compressing the optic nerve at the entrance of the optic canal. However, a previous image that was taken 17 years back revealed that the OA was normal, which suggested the change in dolichoectasia was acquired. Cerebral angiography showed that the dilated and tortuous OA was running from the ophthalmic segment of the left internal carotid artery into the orbit. The symptoms could have been attributed to the direct compression of the dolichoectatic OA in the optic canal. A sufficient anastomosis between the central retinal artery and the middle meningeal artery was identified on external carotid angiography with balloon occlusion of the internal carotid artery. Endovascular treatment with internal trapping of the OA was performed due to ophthalmic symptom progression. Internal coil trapping of the OA was performed at the short segment between the OA bifurcation and the entrance of the optic canal. As expected, the central retinal artery was supplied via the middle meningeal artery after the treatment. The transient visual disturbance was immediately resolved. Ophthalmalgia worsened temporarily after the treatment. However, it completely resolved after several days of oral corticosteroid therapy. Postoperative angiography showed that the origin of the OA was occluded and that the OA in the optic canal was shrunk. The flow of the central retinal arteries via the middle meningeal artery was preserved. Conclusions OA dolichoectasia is rare, and its pathogenesis and long-term visual prognosis are still unknown. However, endovascular therapy can improve symptom by releasing the pressure site in the optic canal.

Background: Dolichoectasia is a rare arterial condition characterized by the dilatation, tortuosity, and elongation of cerebral blood vessels. The vertebrobasilar artery and internal carotid artery are the common sites of dolichoectasia. However, dolichoectasia of the branch arteries, such as the ophthalmic artery (OA), is extremely rare. To the best of our knowledge, this is the first case of ophthalmic dolichoectasia that was successfully treated with endovascular internal coil trapping. Case presentation: A 54-year-old female patient presented with transient left ophthalmalgia and visual disturbance. Magnetic resonance imaging revealed a dilated and elongated left OA compressing the optic nerve at the entrance of the optic canal. However, a previous image that was taken 17 years back revealed that the OA was normal, which suggested the change in dolichoectasia was acquired. Cerebral angiography showed that the dilated and tortuous OA was running from the ophthalmic segment of the left internal carotid artery into the orbit. The symptoms could have been attributed to the direct compression of the dolichoectatic OA in the optic canal. A sufficient anastomosis between the central retinal artery and the middle meningeal artery was identified on external carotid angiography with balloon occlusion of the internal carotid artery. Endovascular treatment with internal trapping of the OA was performed due to ophthalmic symptom progression. Internal coil trapping of the OA was performed at the short segment between the OA bifurcation and the entrance of the optic canal. As expected, the central retinal artery was supplied via the middle meningeal artery after the treatment. The transient visual disturbance was immediately resolved. Ophthalmalgia worsened temporarily after the treatment. However, it completely resolved after several days of oral corticosteroid therapy. Postoperative angiography showed that the origin of the OA was occluded and that the OA in the optic canal was shrunk. The flow of the central retinal arteries via the middle meningeal artery was preserved. Conclusions: OA dolichoectasia is rare, and its pathogenesis and long-term visual prognosis are still unknown. However, endovascular therapy can improve symptom by releasing the pressure site in the optic canal. [ABSTRACT FROM AUTHOR]

Background: Intracranial arterial dolichoectasia (IADE) is a common arterial finding of dilation, elongation, or both, affecting large intracranial vessels, and associated with vascular risk factors, including hypertension. Associations of IADE with neuroimaging cerebral small vessel disease (CSVD) may be relevant for diagnosis and prognosis in patients with stroke. The study aimed to conduct an updated systematic review and meta-analysis of observational studies to investigate the relationships of IADE with well-defined CSVD markers in patients with ischaemic stroke. Methods: We systematically searched PubMed, Embase, and Scopus for studies on IADE in ischaemic stroke patients with fulfilling predefined inclusion criteria. We pooled data to conduct a meta-analysis to compare the prevalence of SVD markers between patients with and without IADE groups using risk ratios (RRs) and 95% confidence intervals (CIs). Results: From 157 retrieved abstracts, we included six studies from seven publications comprising 6102 patients with ischaemic stroke. The mean age of patients was 52.8 years, and 3691 (60.5%) were male. IADE was diagnosed in 11.4% (95% CI 8.9–13.9) (761) of included patients; 51.8% (3160) had hypertension. Compared to patients without IADE, individuals diagnosed with IADE had a significantly increased prevalence of lacune (RR 1.67, 95% CI 1.36–2.06, P < 0.01, I2 = 0.00%), cerebral microbleeds (CMBs) (RR 2.56, 95% CI 1.53–4.28, P < 0.01, I2 = 84.95%) and white matter hyperintensities (WMHs) (RR 2.17, 95% CI 1.84–2.56, P < 0.01, I2 = 0.00%). Conclusions: In patients with ischaemic stroke, IADE is associated with a higher prevalence of CSVD markers, including lacunes, CMBs, and WMHs. Further studies are needed to clarify the mechanisms underlying these associations and their potential relevance for the understanding, diagnosis, and treatment of CSVD. [ABSTRACT FROM AUTHOR]

Vertebrobasilar dolichoectasia, a rare vascular anomaly, rarely presents with hydrocephalus. The traditional treatment for hydrocephalus is a ventriculoperitoneal shunt. Conventional endoscopic third ventriculostomy can avoid shunt-related complications but is considered risky due to the presence of the dolichoectatic vessel. A subfrontal extra-axial fenestration of the lamina terminalis can circumvent this anatomic constraint and establish cerebrospinal fluid communication between the third ventricle and subarachnoid space. We performed an extra-axial endoscopic third ventriculostomy to manage hydrocephalus due to vertebrobasilar dolichoectasia in a 26-year-old male. The clinical description, surgical technique, outcome, and rationale are described. The patient had symptomatic improvement in his headaches and vision. There was also improvement in the postoperative ventricular indices: Evans index—19% reduction, frontal occipital horn ratio—14.1% reduction, and third ventricle index—39.5% reduction. A cine-phase magnetic resonance image showed cerebrospinal fluid flow void through the lamina terminalis fenestration, suggesting patency. Extra-axial endoscopic third ventriculostomy may be a suitable treatment alternative to circumvent anatomic constraints produced by vertebrobasilar dolichoectasia in performing conventional endoscopic third ventriculostomy. [ABSTRACT FROM AUTHOR]

Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker's criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37–71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment. [ABSTRACT FROM AUTHOR]

Vertebral artery agenesis is a rare congenital malformation, with few reported cases in the literature and no epidemiological data in the world at this point in time. The importance of diagnosis lies in identifying a potential risk factor for future ischemic events of the posterior circulation, in particular in the young population. It is also important to determine the etiology of chronic headache of unexplained cause that could be attributed to this entity. The present case describes this finding in a 27-year-old female patient with no pathological history, with multiple stab wounds was brought to the emergency room of our hospital .During the study of whether the wounds caused had been penetrating to the neck, a CT angiography was performed. The CT documented an incidental finding of absent left transverse foramen of the cervical vertebrae, absence of the left vertebral artery from its origin in the ipsilateral subclavian artery and a single dolichoectatic right vertebral artery crossing the midline at the entrance to the foramen magnum.

Background Vertebrobasilar dolichoectasia (VBDE) is defined as a symptomatic dilatation and tortuosity of the vertebrobasilar arteries. The risk of hydrocephalus development is due to direct compression of the third ventricle outflow or brainstem compression and related aqueduct stenosis. We present an uncommon case of a patient with symptomatic VBDE with the uniqueness of a hypoplastic third ventricle associated with biventricular hydrocephalus. A literature review concerning diagnosis and management of patients affected by biventricular hydrocephalus caused by VBDE was also performed. Case Illustration We report a case of a 54-year-old man who presented with headache, ideomotor apraxia, and gait disorder. A head computed tomography (CT) scan showed a biventricular hydrocephalus and a subsequent CT angiography documented the presence of a VBDE compressing the anterior part of the third ventricle that also appeared hypoplastic. The patient also presented a clinical history of arterial hypertension for which he was given a proper pharmacologic treatment with symptom relief. A surgical treatment of ventriculoperitoneal shunt along with endoscopic septostomy was proposed, but the patient refused, probably due to the slightly positive response to medical treatment. Conclusions The natural clinical history of patients affected by VBDE is unfavorable with 7.8 years of median survival. The therapeutic strategy is usually conservative and the role of antiplatelets or oral anticoagulants is still debated. In selected patients, ventriculoperitoneal shunt to resolve intracranial hypertension caused by biventricular hydrocephalus is the most effective treatment. In our opinion, chronic third ventricle compression could lead to anatomic–pathologic alterations like the third ventricle hypoplasia documented in our report. [ABSTRACT FROM AUTHOR]

Hypersomnia is a condition in which a person has trouble staying awake during the day. There are several potential causes of it, including sleep apnea and sleep disorders. A 43 year old male was referred to our practice with complaints of hypersomnia, snoring, slurred speech and sleep apnea for more diagnostic and therapeutic interventions. His brain MRI was significant for a vascular loop compression on medulla oblongata. The patient underwent microvascular decompression surgery subsequently and showed improvement in all of his symptoms. One of the rare causes of sleep apnea is medulla oblongata compression by a vascular loop. Vertebrobasilar dolichoectasia may cause this phenomenon probably and should be reviewed in imaging examinations more precisely. Microvascular decompression by using a synthetic Teflon patch may be helpful in management of these patients. [ABSTRACT FROM AUTHOR]

Intracranial arterial dolichoectasia is a disorder of cerebral vasculature characterized by abnormal elongation, tortuosity, and dilatation of the cerebral arteries. The posterior circulation is more frequently affected than the anterior circulation. Dolichoectasia of internal carotid arteries (ICAs) presenting with binasal hemianopia is rare. We report a case report of a 48-year-old male who presented with a history of binasal hemianopia. Magnetic resonance (MR) imaging of the brain with MR angiography showed bilateral dolichoectasia of ICAs compressing bilateral optic nerve and optic chiasm. Diagnosis of bilateral optic neuropathy due to dolichoectasia of ICAs was made after thorough investigation and ruling out of other possible etiologies of bilateral optic neuropathy. Dolichoectasia is a rare cause of bilateral optic neuropathy and should be suspected in patients who present with gradually progressive visual field loss with onset in the nasal field. [ABSTRACT FROM AUTHOR]

Vertebrobasilar artery nonsaccular aneurysms (VBANSAs) are associated with a 13% annual mortality. Revascularization and flow diversion are life-saving options in select cases; technical failures and rapid hemodynamic changes may contribute to unwanted outcomes. We describe a technique and report clinical outcomes of patients treated with an experimental slow-closing clip (SCC). An experimental SCC was created to gradually close the parent artery of aneurysms. Clinical, radiographic, and outcome data from patients with VBANSAs who underwent experimental treatment with the SCC were retrospectively analyzed. Among 10 patients (7 men; mean age, 49.5 years; range, 18–73 years), 6 presented with mass effect symptoms, 1 with ischemic stroke, 2 with subarachnoid hemorrhage, and 1 with hydrocephalus. Five patients underwent revascularization plus SCC application, and 5 were treated with SCC alone. The mean follow-up was 6.7 years. The expected mortality among patients with unruptured VBANSAs with previous treatment options in this period was 52.7%, whereas the observed rate was 20%. Four patients died within 12 months after treatment. Causes of death were brainstem ischemic stroke, poor-grade subarachnoid hemorrhage, poor clinical presentation, and unknown. Six patients were alive at last follow-up, with unchanged or improved modified Rankin Scale scores. Mortality was associated with posterior-projecting aneurysms and late-stage treatment. In this small case series, use of SCC overcame the natural history of VBANSAs when treatment timing and aneurysm anatomy were suitable. The SCC potentially favors aneurysm thrombosis and collateral reactivation. More studies are necessary to better develop the SCC. [ABSTRACT FROM AUTHOR]

Background: Giant dolichoectatic basilar trunk aneurysms have an unfavorable natural history and are associated with high morbidity, but their neurosurgical treatment is complex and challenging. Methods: Flow reversal reconstruction with fourth-generation bypass and proximal vertebral artery clip occlusion is performed via orbitozygomatic craniotomy with the Kawase approach under rapid ventricular pacing. Conclusion: Fourth-generation bypass is an innovative, technically challenging, and clinically effective tool in the treatment armamentarium for giant dolichoectatic basilar trunk aneurysms. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type 1 is an autosomal dominant genetic disorder with multisystem manifestations including vascular abnormalities. The condition is also associated with an increased risk of both ischemic and hemorrhagic stroke. Here we report a case of a 60-year-old male with known neurofibromatosis who presented with right sided hemiparesis. Neuroimaging work-up revealed left internal carotid artery dissection and tandem occlusion of the left internal carotid artery and left middle cerebral artery. There was associated territorial ischemic infarction. The patient was found to have extensive intra and extra cranial vasculopathy including gross basilar dolichoectasia and a right-sided cervical internal carotid artery pseudoaneurysm. This case highlights the clinical significance of neurofibromatosis associated vasculopathy which can result in stroke.

A 74-year-old man tripped while walking. He had not been administered antiplatelet or anticoagulation therapy. At presentation, the patient was well-oriented, with a blood pressure of 130/91 mmHg, while present with tetraplegia and numbness in the upper extremities. Blood work revealed normal findings, while magnetic resonance imaging of the cervical spine revealed severe cord compression at the C3/4 and C4/5 levels. Cranial computed tomography (CT) showed elongated masses in the Sylvian fissures without intracranial hemorrhage. CT taken 2 days later revealed an intraparenchymal hemorrhage located mainly in the right putaminal region, while the patient showed no signs of neurological deterioration. Three-dimensional CT angiography (3D CTA) demonstrated marked ectasia and elongation in the right internal carotid, bilateral middle cerebral, and left anterior cerebral arteries. The patient was conservatively managed. Repeat 3D CTA performed 3 months later showed no de novo abnormalities in the ectatic cerebral arteries. It is assumed that the delayed traumatic intracerebral hemorrhage was caused by disruption of the perforating vessels arising from the ipsilateral dolichoectatic middle cerebral artery. Periodical surveillance neuroimaging is recommended for patients with head trauma who are simultaneously diagnosed with incidental dolichoectasia, especially when complicated with cervical cord injury.

Background: There are many causes of episodes of vertigo and very few causes of episodes of changes in taste, and the combination of the two is very rare. Here, we describe a patient with recurrent short episodes of vertigo in combination with simultaneous episodes of recurrent paroxysmal dysgeusia and altered feeling on the left side of face. The symptoms were caused by compression of the vestibulocochlear nerve and the facial nerve due to dolichoectasia of the basilar artery. Methods: The patient was diagnosed in our routine clinical practice and underwent a complete neurological and neuro-otological examination, including video head impulse test, caloric irrigation, ocular and cervical vestibular evoked myogenic potentials, acoustic-evoked potentials, neuro- orthoptic examination, cranial MRI, and MR angiography. The patient was seen twice for follow-up. Case: A 71-year-old patient primarily presented with a 2-year history of recurrent short episodes of spinning vertigo. Each of the episodes began with an altered feeling on the left side of the face, followed by a bitter taste on the left half of the tongue, and subsequently vertigo lasting for up to 15 s. The frequency of the attacks was high: up to 80 times per day. Laboratory tests revealed signs of a peripheral vestibular deficit on the left side. There were no signs of sensory or motor deficits or of altered taste between the episodes. An MRI of the brain showed an elongated basilar artery leading to an indentation of the facial and vestibulocochlear nerves on the left side. Conclusion: We propose a neurovascular compression in the proximal part of two cranial nerves because of pulsatile compression by the elongated basilar artery with ephatic discharges as the cause of the recurrent episodes. Consistent with the theory of ephatic discharges, treatment with the sodium channel blocker lacosamide for over six months with a final dosage of 200mg per day p.o. led to a significant reduction of the attack frequency and intensity. This treatment option with a sodium channel blocker should therefore not only be considered in vestibular paroxysmia but also in cases of paroxysmal dysgeusia. [ABSTRACT FROM AUTHOR]

The pathophysiology underlying vertebrobasilar dolichoectasia (VBD) is largely unknown. However, a few reports have demonstrated that acute intramural hematoma (IMH) in VBD is associated with stroke. We aimed to investigate the clinical and radiological features of IMH in VBD and the role of IMH in predicting rupture and patient outcomes. We retrospectively reviewed the medical records of patients treated in 2 stroke centers between January 2012 and December 2021. Patients presenting with VBD and stroke were eligible for study inclusion. We excluded patients with stroke caused by arterial dissection or artery-to-artery embolism. IMH was defined as a crescent-shaped area of high signal density in the vessel wall on axial computed tomography in the absence of an intimal flap, double lumen, and pearl-and-string sign. Six patients were analyzed. All presented with symptoms of brainstem/cerebellar infarction without headache. Interobserver agreement for the presence or absence of IMH was excellent (100%). IMH was detected in 5 patients. The positive predictive value of IMH for rupture was 80% (95% confidence interval, 28%–99.5%). The median time from symptom onset to rupture was 2.5 days (range, 1.5–4). Median computed tomography values were significantly higher within the IMH than those in the lumen of the basilar artery (70 vs. 44.5 Hounsfield units; P = 0.008). The modified Rankin scale score on day 30 after onset was 5 in 1 patient and 6 in the remaining 5. IMH in patients with VBD presenting with brainstem/cerebellar infarction should be regarded as a sign associated with a high risk of rupture. [ABSTRACT FROM AUTHOR]

Vascular compression of neural tissue causing neurological symptoms is a well)known phenomenon. This is commonly seen in trigeminal neuralgia and, less commonly, in hemifacial spasm by small arteries, which can be treated by micro)vascular decompression. Rarely, larger arteries, such as the vertebral arteries, may compress the brainstem. This can lead to symptoms of pontine or medullary distress like hemiparesis, dysphagia, or respiratory distress. This is treated by macrovascular decompression. Due to the rare and heterogenous nature of this disease, there is no standardized approach. We describe a novel technique whereby the vertebrobasilar system is mobilized anterolaterally towards the occipital condyle with a sling to decompress the brainstem. We report two cases of vertebrobasilar dolichoectasia causing brainstem compression. A carotid patch graft sling with anterolateral mobilization to the occipital condyle is described as a surgical nuance to macrovascular decompressive surgery. Briefly, the vertebral artery was identified and dissected away from the brainstem and the bulbar cranial nerves. Bovine pericardium graft was used to create a sling around the artery by suturing the two ends together. The sling was then fixed either to the occipital condyle using cranial plating screws or suturing to the dura of the occipital condyle. A novel surgical technique for management of vertebrobasilar dolichoectasia causing brainstem compression with progressive neurological deterioration is reported. Anatomical location and the offending vessel should guide neurosurgeons to select the best surgical option to achieve complete decompression of the involved neural structures. [ABSTRACT FROM AUTHOR]

Vascular compression of the optic nerve in a patient with rapid monocular vision loss with otherwise negative diagnostic workup is a rare, but controversial dilemma. The literature is conflicted, advocating for either timely surgical decompression to preserve vision 1-6 or observation only given the prevalence of asymptomatic vascular compression and observed arrest of visual decline. 7-10 The most frequently reported sources of symptomatic compression are unruptured aneurysms and dolichoectatic vasculature, 1-6 with recent consensus reached over a need for extensive perioperative ophthalmologic evaluations and follow-up. We present an illustrative case for microvascular decompression of the prechiasmatic optic nerve. Video footage of the operative management of microvascular optic nerve compression is exceedingly rare. 5 , 6 A 50-year-old man with a past medical history of hypertension and substance use presented with a 1-week history of progressive right nasal hemianopsia (Video 1). After a negative stroke workup, magnetic resonance imaging of the brain showed prechiasmatic displacement of the right optic nerve by the right supraclinoid internal carotid artery. Formal cerebral arteriography showed a left-sided fetal posterior cerebral artery and patent vasculature without a causative lesion. Given isolated right eye symptoms and rapid progression, a right orbitozygomatic craniotomy for microvascular decompression was recommended. The patient consented to the procedure and to the publication of his image. Intraoperatively, a right calcified dolichoectatic supraclinoid internal carotid artery was found to be severely displacing and tethering its ipsilateral optic nerve. Optic canal deroofing, detethering of the optic nerve, and polytetrafluoroethylene (Teflon) patch placement was performed to achieve this decompression. His postoperative course was uncomplicated; only mild improvement of his visual symptoms was noted at 1- and 3-month follow-up. Formal acuity and computerized assessments of vision and extensive follow-up are critical for evaluating the true clinical outcome of patients with microvascular optic nerve compression., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Intracranial arterial dolichoectasia (IADE) is characterized by the dilation, elongation, and tortuosity of intracranial arteries. We aimed to investigate the association between variations of the Circle of Willis (COW) and IADE in the general population, as well as estimate the genetic correlation between COW variations and IADE., Methods: A total of 981 individuals from a population-based cohort were included. Brain magnetic resonance angiography was performed to assess COW variants and measure the diameters of intracranial arteries. IADE was defined as a total intracranial volume-adjusted diameter ≥ 2 standard deviations. Logistic regression models were used to analyze the association between COW variations and IADE. The heritability and genetic correlation were estimated using genome-wide complex trait analysis (GCTA) based on single nucleotide polymorphism (SNP) array data., Results: The prevalence of IADE was 6.2 %. Hypoplastic/absent A1 segments were associated with an increase in contralateral ICA diameter (β ± SE, 0.279 ± 0.049; p = 0.001) and a decrease in ipsilateral ICA diameter (β ± SE, -0.300 ± 0.050; p = 0.001). Fetal-type posterior cerebral artery (FTP) was associated with a larger ICA diameter (β ± SE, 0.326 ± 0.048; p = 0.001) and a smaller BA diameter (β ± SE, -0.662 ± 0.043; p = 0.001). FTP revealed a positive genetic correlation with ICA dilation (rG = 0.259 ± 0.175; p = 0.0009) and a negative genetic correlation with BA dilation (rG = -0.192 ± 0.153, p = 0.015)., Conclusions: There was an association between COW variations and larger intracranial arterial diameters in the general population. Genetic factors may play a role in the development of intracranial arterial dilation and the formation of COW variants., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

BackgroundThere are many causes of episodes of vertigo and very few causes of episodes of changes in taste, and the combination of the two is very rare. Here, we describe a patient with recurrent short episodes of vertigo in combination with simultaneous episodes of recurrent paroxysmal dysgeusia and altered feeling on the left side of face. The symptoms were caused by compression of the vestibulocochlear nerve and the facial nerve due to dolichoectasia of the basilar artery.MethodsThe patient was diagnosed in our routine clinical practice and underwent a complete neurological and neuro-otological examination, including video head impulse test, caloric irrigation, ocular and cervical vestibular evoked myogenic potentials, acoustic-evoked potentials, neuro-orthoptic examination, cranial MRI, and MR angiography. The patient was seen twice for follow-up.CaseA 71-year-old patient primarily presented with a 2-year history of recurrent short episodes of spinning vertigo. Each of the episodes began with an altered feeling on the left side of the face, followed by a bitter taste on the left half of the tongue, and subsequently vertigo lasting for up to 15 s. The frequency of the attacks was high: up to 80 times per day. Laboratory tests revealed signs of a peripheral vestibular deficit on the left side. There were no signs of sensory or motor deficits or of altered taste between the episodes. An MRI of the brain showed an elongated basilar artery leading to an indentation of the facial and vestibulocochlear nerves on the left side.ConclusionWe propose a neurovascular compression in the proximal part of two cranial nerves because of pulsatile compression by the elongated basilar artery with ephatic discharges as the cause of the recurrent episodes. Consistent with the theory of ephatic discharges, treatment with the sodium channel blocker lacosamide for over six months with a final dosage of 200 mg per day p.o. led to a significant reduction of the attack frequency and intensity. This treatment option with a sodium channel blocker should therefore not only be considered in vestibular paroxysmia but also in cases of paroxysmal dysgeusia.

Audio-vestibular symptoms can arise from vertebrobasilar dolichoectasia (VBD) and basilar dolichoectasia (BD). Given the dearth of available information, herein we reported our experience with different audio-vestibular disorders (AVDs) observed in a case series of VBD patients. Furthermore, a literature review analyzed the possible relationships between epidemiological, clinical, and neuroradiological findings and audiological prognosis. The electronic archive of our audiological tertiary referral center was screened. All identified patients had a diagnosis of VBD/BD according to Smoker’s criteria and a comprehensive audiological evaluation. PubMed and Scopus databases were searched for inherent papers published from 1 January 2000 to 1 March 2023. Three subjects were found; all of them had high blood pressure, and only the patient with high-grade VBD showed progressive sensorineural hearing loss (SNHL). Seven original studies were retrieved from the literature, overall including 90 cases. AVDs were more common in males and present in late adulthood (mean age 65 years, range 37–71), with symptoms including progressive and sudden SNHL, tinnitus, and vertigo. Diagnosis was made using different audiological and vestibular tests and cerebral MRI. Management was hearing aid fitting and long-term follow-up, with only one case of microvascular decompression surgery. The mechanism by which VBD and BD can cause AVD is debated, with the main hypothesis being VIII cranial nerve compression and vascular impairment. Our reported cases suggested the possibility of central auditory dysfunction of retro-cochlear origin due to VBD, followed by rapidly progressing SNHL and/or unnoticed sudden SNHL. More research is needed to better understand this audiological entity and achieve an evidence-based effective treatment.

Introduction: The association between cervical internal carotid artery (cICA) tortuosity and atherosclerosis is a matter of debate. Additionally, some genetic syndromes characterized by connective tissue remodeling are associated with arterial tortuosity, raising the possibility that cICA tortuosity may not only be atherosclerotic. In this study, we hypothesized that cICA tortuosity is not associated with imaging biomarkers of atherosclerosis. Methods: The Northern Manhattan Study (NOMAS) was a prospective, multiethnic cohort of stroke-free individuals who underwent brain MRA, carotid ultrasound and transthoracic echocardiogram from 2003–2008. The cICA tortuosity was scored in each carotid as 0 = no tortuosity, 1 = tortuosity <90°, 2 = tortuosity ≥90°. A summary cICA tortuosity score (possible range 0–4) was created by adding up the tortuosity score from each carotid. Participants were assessed for atherosclerotic markers by using B‑mode carotid sonography and transthoracic echocardiography. Results: Of 558 participants 178 (31.9%) had any cervical ICA tortuosity (tortuosity score >0). The cICA tortuosity score was higher in women and was associated with diastolic and systolic blood pressures and height (all P < 0.05). In models adjusted for demographics and risk factors, only the association with diastolic blood pressure remained significant (β = 0.002, P = 0.02). Similarly, cICA tortuosity was associated with larger aortic root diameter (B = 1.03 ± 0.36, P = 0.004) but not with other markers of carotid or aortic atherosclerosis. Conclusion: Cervical ICA tortuosity is associated with a higher diastolic blood pressure and larger aortic root diameter but not with other measures of atherosclerosis. Determining the risks of vascular events associated with this non-atherosclerotic phenotype may help for a better risk stratification for individuals with cICA tortuosity. [ABSTRACT FROM AUTHOR]

Our objective was to investigate brain structure, cerebral vasculature, and cognitive function in a cohort of patients with late‐onset Pompe disease, with particular reference to the differences from those with the classic infantile phenotype, where extensive white‐matter abnormalities (WMA) and impaired cognition on long‐term enzyme treatment are reported in a subset of patients. Brain imaging (T1, T2, T2 fluid‐attenuated inversion recovery, susceptibility‐weighted images, and magnetic resonance angiography–time of flight) was combined with extensive cognitive testing of general intelligence (Wechsler IQ Test, Montreal Cognitive Assessment [MoCA]) and specific neuropsychological domains (verbal fluency, cognitive flexibility, attention, memory, and visuospatial abilities). We included 19 patients with late‐onset Pompe disease (age range 11‐56 years). Two patients showed mild punctate WMA within normal range for age, with a Fazekas score (FS) of 1 to 2. Magnetic resonance angiography revealed a slight vertebrobasilar dolichoectasia in two patients yet did not show any aneurysms or vascular dissections. Most patients had age‐adjusted scores within the normal range for the Wechsler index scores (verbal comprehension, perceptual reasoning, working memory, and processing speed) and combined total intelligence (IQ) score (median 101, interquartile range 91‐111; one patient had a below‐average score for total IQ) as well as for the specific domains verbal fluency, attention, and memory. A subset of patients performed suboptimally on the Rey Complex Figure Test (9/14 patients) or cube‐copying/clock‐drawing test of the MoCA (8/10 patients). We therefore concluded that our study showed no brain abnormalities, other than minor microvascular lesions considered within normal range for age, nor general cognitive impairment in late‐onset Pompe patients. These findings are in sharp contrast with the widespread WMA and cognitive problems found in some classic infantile patients. [ABSTRACT FROM AUTHOR]

Hemifacial spasm is a common peripheral movement disorder that usually results from neurovascular conflict involving the facial nerve motor root exit zone. Although it is a sporadic disease, some cases of familial clustering have been reported. In this case report, we present a case of familial occurrence of hemifacial spasm with dolichoectasia of the vertebrobasilar system in three siblings. The patients responded well to botulinum toxin therapy. A genetic mechanism predisposing them to vascular abnormalities is likely to be responsible for the clinical–radiological picture in our patients.

Brain arterial diseases, including atherosclerosis, vasculitis, and dissections, are major contributors to cerebrovascular morbidity and mortality worldwide. These diseases not only increase the risk of stroke but also play a significant role in neurodegeneration and dementia. Clear and unambiguous terminology and classification of brain arterial disease phenotypes is crucial for research and clinical practice. This review aims to summarize and harmonize the terminology used for brain large and small arterial phenotypes based on pathology studies and relate them to imaging phenotypes used in medical research and clinical practice. Arteriosclerosis refers to hardening of the arteries but does not specify the underlying etiology. Specific terms such as atherosclerosis, calcification, or non-atherosclerotic fibroplasia are preferred. Atherosclerosis is defined pathologically by an atheroma. Other brain arterial pathologies occur and should be distinguished from atherosclerosis given therapeutic implications. On brain imaging, intracranial arterial luminal stenosis is usually attributed to atherosclerosis in the presence of atherosclerotic risk factors but advanced high-resolution arterial wall imaging has the potential to more accurately identify the underlying pathology. Regarding small vessel disease, arteriosclerosis is ambiguous and arteriolosclerosis is often used to denote the involvement of arterioles rather than arteries. Lipohyalinosis is sometimes used synonymously with arteriolosclerosis, but less accurately describes this common small vessel thickening which uncommonly shows lipid. Specific measures of small vessel wall thickness, the relationship to the lumen as well as changes in the layer composition might convey objective, measurable data regarding the status of brain small vessels. [ABSTRACT FROM AUTHOR]

BackgroundThe treatment of dolichoectatic basilar trunk aneurysms has been ineffectual or morbid due to nonsaccular morphology, deep location, and involvement of brainstem perforators. Treatment with bypass surgery has been advocated to eliminate malignant hemodynamics and to stabilize aneurysm growth.ObjectiveTo validate that flow alteration with bypass and parent artery occlusion favorably impacts aneurysm progression.MethodsSurgical management evolved in 3 phases, each with different hemodynamic alterations.ResultsDuring a 17-year period, 37 patients with dolichoectatic basilar trunk aneurysms were retrospectively identified, of whom 21 patients were observed, 12 treated immediately, and 4 selected for treatment after clinical progression. In phase 1, flow reversal was overly thrombogenic, despite heparin (N = 5, final mortality, 100%). In phase 2, flow reduction with intracranial-to-intracranial bypass was safer than flow reversal, but did not prevent progressive aneurysm enlargement (N = 3, final mortality 67%). In phase 3, distal clip occlusion of the basilar trunk aneurysm preserved anterograde flow in the aneurysm without rupture, but reduced flow threatened perforator patency, despite treatment with clopidogrel (N = 8, final mortality 62%).ConclusionShifting treatment strategy for dolichoectatic basilar trunk aneurysms improved surgical (80% to 50%) and final mortalities (100% to 62%), with stabilization of aneurysms in the phase 3 survivors. Good outcomes are determined by perforator preservation and mitigating aneurysm thrombosis. Occlusion techniques with increased distal run-off seem to benefit perforators. The treatment of dolichoectatic basilar trunk aneurysms can advance through concentrated management in dedicated centers, concerted efforts to study morphology and hemodynamics with computational methods, and widespread collection of registry data.Abbreviations4D PC-MRI, time-resolved phase-contrast MRIAICA, anterior inferior cerebellar arteryCE-MRA, high-resolution contrast-enhanced MR angiographyEC-IC, extracranial-to-intracranial bypassMCA, middle cerebral arteryMR, magnetic resonancemRS, modified Rankin ScalePCA, posterior cerebral arteryPICA, posterior inferior cerebellar arterySCA, superior cerebellar arterySTA, superficial temporal arteryVA, vertebral artery.

Abstract Background Symptomatic medulla oblongata compressing lesions due to dilated vertebral artery are rare in the literature. The symptoms are extremely heterogeneous and not correlated to the severity of compression in many cases. Case presentation This paper describes two cases with vertebral artery compression of medulla oblongata complaining only from dizziness without any other neurological symptoms or signs. In both cases, videonystagmography revealed positional nystagmus. Head magnetic resonance imaging showed abnormal dilatation, elongation, and tortuosity of the vertebral artery compressing the medulla oblongata. Vestibular rehabilitation was described in both cases and had a significant effect on symptom improvement. Conclusion In the study cases, the sense of vertigo and/or unsteadiness is due to vertebral artery compression of medulla oblongata and can be an isolated symptom. Positional nystagmus is the only sign in vestibular evaluation.

Vertebrobasilar dolichoectasia (VBD) is a rare condition characterized by a significant dilation, elongation and tortuosity of the arteries emerging from the vertebrobasilar system. In this study, we report a 57-year-old male, with medical history of recurrent migraines and right peroneal nerve palsy, which presents the sudden onset of clinical signs of brainstem and cerebellar stroke. The brain CT scan revealed an inhomogeneous, hyperdense prepontine lesion, situated in the area of basilar artery (BA) projection. The cerebral angiography showed in the V4 segment of the left vertebral artery (VA) a focal, concentric stenosis of 70%, followed by a fusiform dilation extended to the BA bifurcation, a saccular aneurysmal dilation in the communicant segment of the internal carotid artery and tortuous trajectory of the abdominal and thoracic aorta, including the supra-aortic vessels. The brain MRI revealed a bulbopontin and left cerebellum infarction. Patient developed at 48 hours after hospitalization livedo reticularis at the level of the right thoracic wall and thighs. The nerve conduction studies revealed mononeuritis multiplex. The vascular anomaly that was identified in the presented case respects the criteria for a VBD, however the involvement of the aorta with the presence of tortuosities, the simultaneous presence of the internal carotid artery aneurysm, together with the presence of the mononeuritis multiplex and the skin condition (livedo reticularis) suggest the possibility of coexistence of a systemic vasculitis, possible polyarteritis nodosa.

Hemifacial spasm is a common peripheral movement disorder that usually results from neurovascular conflict involving the facial nerve motor root exit zone. Although it is a sporadic disease, some cases of familial clustering have been reported. In this case report, we present a case of familial occurrence of hemifacial spasm with dolichoectasia of the vertebrobasilar system in three siblings. The patients responded well to botulinum toxin therapy. A genetic mechanism predisposing them to vascular abnormalities is likely to be responsible for the clinical–radiological picture in our patients. [ABSTRACT FROM AUTHOR]

Background and Purpose: It is unknown whether intracranial atherosclerotic disease (ICAD), in addition to causing stenosis, also associates with abnormal arterial enlargement, a condition known as intracranial dolichoectasia (IDE). Across symptomatic ICAD patients, we aim to determine IDE prevalence and IDE impact on cerebral hemodynamics and recurrent cerebral ischemia. Methods: We analyzed 98 participants (mean age 63.8 ± 11.9 years, 56.1% men) of the prospective observational study MYRIAD. Participants were enrolled within 21 days of an ischemic stroke or transient ischemic attack caused by moderate‐to‐severe ICAD. Semi‐automatic vessel segmentation was used to determine diameters, length, and tortuosity‐index of proximal intracranial arteries. Either ectasia (increased diameter) or dolichosis (increased length or TI) defined IDE. We assessed IDE association with new infarcts during 12‐month follow‐up, and IDE correlation with cerebral hemodynamics determined by quantitative MR‐angiography (QMRA), MR‐perfusion weighted‐imaging, and transcranial Doppler breath‐holding index. Results: IDE was present in 35.7% of patients and 10.2% of symptomatic arteries. Basilar stenosis was associated with higher IDE prevalence (27.8% vs. 8.8%, p = 0.04), whereas other symptomatic arteries showed no association with IDE. Symptomatic arteries with IDE had lower hypoperfusion prevalence on MR‐PWI (11.1% vs. 28.4%, p = 0.03). Increased diameter (r = 0.33, p<0.01) and tortuosity‐index (r = 0.29, p = 0.01) showed positive correlation with QMRA flow rate. IDE was not associated with new infarcts during follow‐up. Conclusions: IDE was common among symptomatic ICAD patients. IDE was not associated with stroke recurrence. Instead, increased diameter and tortuosity correlated with improved blood flow across the stenotic artery, suggesting that IDE may originate as an adaptive mechanism in ICAD. [ABSTRACT FROM AUTHOR]

Vertebrobasilar dolichoectasia is relatively rare, and it can compress the brainstem and surrounding brain cistern due to its volume. It is frequently associated with arterial dissection, obstructive hydrocephalus, and infarction in the brainstem or cerebellum. We present a case of a 57-year-old man with a dissection localized to the basilar artery. However, the patient developed an infarction to the right posterior inferior cerebellar artery region, acute brainstem compression, and obstructive hydrocephalus caused by entrapment between the dissecting aneurysm and cerebellar infarction. Posterior fossa decompression and extraventricular drainage were performed immediately and alleviated his condition. We suggest that such treatments may be needed at early stages, and we also review existing cases in the literature. [ABSTRACT FROM AUTHOR]

Background: The occurrence of intracranial aneurysms is higher in patients with autosomal dominant polycystic kidney disease (ADPKD) than in the healthy population. However, research concerning the factors related to the risk of intracranial aneurysm rupture in patients with ADPKD is still insufficient. Objectives: The aim of the study was to investigate the prevalence of intracranial aneurysms and aneurysmal subarachnoid hemorrhage (SAH) and to analyze the systemic factors associated with high-risk aneurysms in patients with ADPKD. Methods: We screened patients who underwent cerebral angiography between January 2007 and May 2017 in the ADPKD registry. Patients were examined for the presence of intracranial aneurysms and subsequently reclassified into 3 groups based on the risk of aneurysmal rupture: the aneurysm-negative (group 1), low-risk aneurysm (group 2), or high-risk aneurysm (group 3). Various systemic factors were compared, and independent factors associated with high-risk aneurysms were analyzed. Results: Among the 926 patients, 148 (16.0%) had intracranial aneurysms and 11 (1.2%) had previous aneurysmal SAH. Patients with intracranial aneurysms were further classified into group 2 (low-risk aneurysms, 15.5%) or group 3 (high-risk aneurysms, 84.5%). Age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.01–1.05, p = 0.004), female sex (OR 3.13, 95% CI 1.94–5.0 6, p < 0.001), dolichoectasia (OR 8.57, 95% CI 1.53–48.17, p = 0.015), and mitral inflow deceleration time (DT) (OR 1.01, 95% CI 1.00–1.01, p = 0.046) were independently associated with high-risk aneurysms, whereas hypercholesterolemia (OR 0.46, 95% CI 0.29–0.72, p = 0.001) was negatively associated. Conclusion: In the present study among patients with ADPKD, the prevalence of intracranial aneurysms and aneurysmal SAH was 16 and 1.2%, respectively. Age, female sex, dolichoectasia, and mitral inflow DT were positively associated with high-risk aneurysms, whereas hypercholesterolemia was negatively associated. A subsequent large-scaled longitudinal study is needed to define the plausibility of the clinical parameters. [ABSTRACT FROM AUTHOR]

Cervical carotid artery (cCA) dolichoectasia (DE) is characterized by elongation, tortuosity, and/or dilatation. The prevalence of cCA DE has been reported 13‐31% in population‐based and 14‐58% in hospital‐based studies. The exact mechanisms of this aberrant arterial remodeling are unknown. Although atherosclerosis has often been implicated, the evidence has conflicting results that would support atherosclerosis as the underlying pathology. Actually, other nonatherosclerotic mechanisms related to connective tissue remodeling may play a role. Such mechanism is supported by epidemiological evidence that cCA DE is associated with carotid dissections. Similarly, cCA DE has been associated with vascular risk factors, but inconsistently. Fewer studies have evaluated the risk of vascular events in people with cCA DE. Cross‐sectionally, cCA DE is associated with cerebrovascular disease, including white matter hyperintensities, lacunar stroke, and stroke overall. The often‐conflicting results may in part be due to the heterogeneity of the population studies and variable definitions used. Preferential use of objective measure of cCA DE, such as carotid length, is advisable, and may help comparing result among different studies. Prospectively, people with cCA DE have a higher risk of vascular events, although it is uncertain if the risk of stroke is also higher in this population. In the absence of alternative stroke etiologies, stroke patients with cCA DE should be considered to have had a cryptogenic stroke and treated with daily antiplatelet therapy. Further population‐based studies are needed to clarify whether specific therapies may be implement to reduce the risk of events among people with cCA DE. [ABSTRACT FROM AUTHOR]

Skeletal overgrowth accompanied by de novo heterozygous activating mutations in PDGFRB (platelet‐derived growth factor receptor beta), that is, p.Pro584Arg and p.Trp566Arg, defines Kosaki overgrowth syndrome (OMIM #616592). Emerging evidence suggests a role of PDGFRB in the genesis of cerebral aneurysms. The delineation of the range and progression of the vascular phenotype of Kosaki overgrowth syndrome is urgently needed. Herein, we conducted subsequent analyses of serial neurovascular imaging studies of two original patients with a de novo heterozygous mutation in PDGFRB, that is, p.Pro584Arg. The analysis showed the progressive dilation of basilar and vertebral arteries and coronary arteries commencing during the teenage years and early 20s. The radiographic appearance of the basilar vertebral aneurysms showed signs of arterial wall dilation, compatible with the known vascular pathology of vascular‐type Ehlers‐Danlos syndrome and Loeys‐Dietz syndrome. The dolichoectasia in cerebrovascular arteries can lead to fatal complications, even with neurosurgical interventions. To prevent the progression of artery dilation, preventative and therapeutic medical measures using tyrosine kinase inhibitors may be necessary in addition to optimal control of the systemic blood pressure. Kosaki overgrowth syndrome is a clinically recognizable syndrome that can exhibit progressive dilatory and tortuous vascular changes in basilar/vertebral and coronary arteries as early as in the teenage years. We recommend careful counseling regarding the risk of future vascular complications, optimal blood pressure control, and regular systemic vascular screening during follow‐up examinations. [ABSTRACT FROM AUTHOR]

Background: Intracranial dolichoectasia (IADE) is a rare vascular disease characterized by distension, elongation and tortuosity of an artery. IADE rarely involves pediatric aged groups. It is either asymptomatic or manifests as ischemic or hemorrhagic attacks. Case description: A healthy, 30-year-old, female teacher presented with recurrent attacks of bi-frontal headaches associated with dizziness and dropping attacks of two-week duration. She was referred by her general physician to our institution of Neurosurgery Teaching Hospital in Baghdad, Iraq with a suspicion of medial temporal lesion on a cranial computed tomography (CT) scan. Magnetic resonance imaging study excluded the diagnosis suggesting a dolichoectatic middle cerebral artery that was confirmed by CT-angiography. Conclusion: Dolichoectasia of the middle cerebral artery is a rare and benign lesion. However, it can masquerade as cerebral cavernous malformation or intracranial arterial aneurysm. Thus, careful radiological evaluation with the suggested diagnostic criteria are of paramount importance to prevent its misdiagnosis.

Background. Intracranial dolichoectasia (IADE) is a rare vascular disease characterized by distension, elongation and tortuosity of an artery. IADE rarely involves paediatric aged groups. It is either asymptomatic or manifests as ischemic or haemorrhagic attacks. Case description. A healthy, 30-year-old, female teacher presented with recurrent attacks of bi-frontal headaches associated with dizziness and dropping attacks of twoweek duration. She was referred by her general physician to our institution of Neurosurgery Teaching Hospital in Baghdad, Iraq with a suspicion of medial temporal lesion on a cranial computed tomography (CT) scan. Magnetic resonance imaging study excluded the diagnosis suggesting a dolichoectatic middle cerebral artery that was confirmed by CT-angiography. Conclusion. Dolichoectasia of the middle cerebral artery is a rare and benign lesion. However, it can masquerade as cerebral cavernous malformation or intracranial arterial aneurysm. Thus, careful radiological evaluation with the suggested diagnostic criteria are of paramount importance to prevent its misdiagnosis. [ABSTRACT FROM AUTHOR]

Anatomic compression of the optic nerve secondary to a dolichoectatic cerebrovascular compression is a rare clinical entity. Because of the limited number of published cases and variable clinical presentation, the natural history remains ambiguous and no consensus exists regarding management. In addition, there is an ongoing debate regarding whether a dolichoectatic cerebral blood vessel can actually cause optic neuropathy, or it merely represents an incidental finding. As a result, it is thought that a diagnosis of compressive optic neuropathy from an adjacent dolichoectatic internal carotid artery (ICA) should be considered only after other possible etiologies are excluded. Although this might seem straightforward, the clinical scenario becomes complex if the patient is also found to have additional incidental pituitary lesions. Such coexistence has not been reported previously in the literature. A 52-year-old left-handed man presented to us with intermittent headache and painless progressive visual deterioration in the right eye for 1 month. Screening magnetic resonance imaging (MRI) scan revealed a 9-mm eccentrically placed pituitary adenoma with right optic nerve compression because of dolichoectatic ICA. He underwent microvascular decompression of the right optic nerve. On follow-up, significant vision improvement was noticed and MRI scan revealed no change in the size of the pituitary adenoma. The compression of the optic nerve by dolichoectatic ICA is commonly thought to be a diagnosis of exclusion. However, the presence of a coexisting pathology should not prompt the exclusion in every case and a case-based approach is highly recommended to correctly manage this rare clinical condition. [ABSTRACT FROM AUTHOR]

Background: In Pompe disease, glycogen deposition results in an augmentation of blood flow and abnormal remodeling, with resultant weakening of the arterial walls, which may result in pathologic dilatation of the cerebral arteries. This complication is rare in patients with late-onset Pompe disease, but it has not been well-described in infantile-onset Pompe disease. The effect of enzyme replacement therapy on this process is not known.Methods: We examined clinical and imaging data on two patients who exhibit cerebrovascular arteriopathy: a 14-year-old boy with infantile-onset Pompe disease on enzyme replacement therapy and a 23-year-old woman with late-onset Pompe disease who was also receiving enzyme replacement therapy.Results: Our 14-year-old patient exhibits cerebrovascular arteriopathy, primarily proximal and vertebrobasilar, while the 23-year-old patient has a more diffuse pattern. The 14-year-old patient is unique because cerebral dolichoectasias have not been described in infantile-onset Pompe disease. The 23-year-old patient is notable given the age and history of enzyme replacement therapy since age 15 years.Conclusions: Dilative cerebral arteriopathy in infantile-onset Pompe disease is novel and similarly atypical is the diffuse vascular dilation seen in our young patient with late-onset Pompe disease, both receiving enzyme replacement therapy. We should be cognizant of the risk of cerebrovascular disease in Pompe disease regardless of the disease variant and enzyme replacement therapy status. [ABSTRACT FROM AUTHOR]

Dolichoectasia of the distal internal carotid artery, posterior communication artery (PCoA) and posterior cerebral artery is an extremely rare abnormality. Dolichoectasia of the internal carotid artery, PCoA and the P1 segment of posterior cerebral artery can be postulated its pathogenesis by the embryological perspective basis from caudal ramus of the internal carotid artery terminus. The pathogenesis and treatment strategy are not well established. We reviewed and proposed embryological perspective, pathogenesis, clinical setting, radiological findings and management of this rare malformation. [ABSTRACT FROM AUTHOR]

We report a case of cervical radiculopathy caused by an anomalous vertebral artery (VA) and the efficacy of endovascular vertebral artery sacrifice. A 62-year-old woman was referred to neurosurgery because of an 8- to 9-year history of progressive left C6-7 radiculopathy refractory to other forms of treatment. Radiologic evaluation showed an abnormally tortuous loop of VA at V2 causing direct neurovascular compression at the C6-7 level. Initial attempts at microvascular decompression with posterior foraminotomy were unsuccessful due to strong adhesion between the VA and C7 nerve root. This was followed by an endovascular VA sacrifice relieving the patient's symptoms. Cervical root compression by an aberrant or anomalous extracranial VA is a rare cause of radiculopathy. Endovascular VA sacrifice provided symptom relief in our patient, when other options failed. To our knowledge, this is the first report of endovascular VA sacrifice for management of cervical radiculopathy due to VA loop. [ABSTRACT FROM AUTHOR]

Coronavirus disease (Covid-19) can manifest with numerous neurological disorders. We present a case of stroke in a patient with acute intraluminal thrombus in giant basilar tip fusiform aneurysm. A 71- yearold woman with past medical history of arterial hypertension and dilated cardiomyopathy was hospitalized in intensive care unit (ICU) because of decreased consciousness (GCS 6) and a right- sided hemiplegia which occurred twelve hours before admission. Five days earlier she was tested positive for Covid-19. ECG revealed new-onset atrial fibrillation (AF). She was intubated and mechanically ventilated. Brain CT and CT angiography of the head and neck vessels revealed dolichoectatic BA with giant fusiform aneurysm of the tip of BA. BA lumen was partially filled with hyperdense, acute thrombus. Hypodensity of superior cerebellar artery (SCA) irrigation was consistent with acute ischemic stroke. She was treated with low molecular weight heparin, acetylsalicylic acid and corticosteroid therapy. During hospitalization her respiratory function deteriorated, and she died. To our knowledge this is the first case of stroke caused by acute thrombus in a giant tip of the BA aneurysm in patient with Covid-19 infection. It is important to increase prehospital stroke awareness since stroke in patients with Covid-19 is related to worse outcomes, and in appropriate time window can be treated with acute recanalization techniques. [ABSTRACT FROM AUTHOR]

Background: Tortuosity of intracranial arteries is rare and usually mistaken for arteriovenous malformations. In the world literature, the term «pure arterial malformations» is used to refer to this disease., Objective: To summarize the experience of the Burdenko Neurosurgery Center on diagnosis of intracranial artery tortuosity, management and treatment of these patients, as well as to review appropriate literature data., Material and Methods: Tortuosity of intracranial arteries was detected in 11 patients (8 women and 3 men) aged 7-48 years who underwent outpatient or inpatient examination and treatment at the Burdenko Neurosurgery Center between 2009 and 2022. We analyzed angiographic, clinical and follow-up data of these patients, as well as appropriate literature data., Results: According to angiography data, all patients had moderate dilatation, elongation and tortuosity of intracranial arteries without signs of arteriovenous shunting. The most common finding was tortuosity of several segments of internal carotid artery (5 cases). Lesion of PCA, PComA, MCA and ACA was less common. In 7 cases, the walls of the deformed vessels had calcified zones. In two cases, there were saccular aneurysms in the walls of the tortuous vessels. In one case, tortuosity was combined with kinking of the left subclavian artery, in another one - tortuosity of C1 segment of the right ICA. No patient had specific clinical manifestations. The follow-up period was 1-10 years in 7 patients. There were no changes in structure of tortuosity or appearance of new aneurysms., Conclusion: Tortuosity of intracranial arteries is an extremely rare disease with the highest incidence in young women. This abnormality has no specific clinical manifestations and does not require surgical or conservative treatment. Tortuosity of intracranial arteries should be differentiated from arterial dolichoectasia, fusiform aneurysms and AVMs.

Vertebrobasilar dolichoectasia (VBD) is a rare condition characterized by a significant dilation, elongation and tortuosity of the arteries emerging from the vertebrobasilar system. In this study, we report a 57-year-old male, with medical history of recurrent migraines and right peroneal nerve palsy, which presents the sudden onset of clinical signs of brainstem and cerebellar stroke. The brain CT scan revealed an inhomogeneous, hyperdense prepontine lesion, situated in the area of basilar artery (BA) projection. The cerebral angiography showed in the V4 segment of the left vertebral artery (VA) a focal, concentric stenosis of 70%, followed by a fusiform dilation extended to the BA bifurcation, a saccular aneurysmal dilation in the communicant segment of the internal carotid artery and tortuous trajectory of the abdominal and thoracic aorta, including the supra-aortic vessels. The brain MRI revealed a bulbopontin and left cerebellum infarction. Patient developed at 48 hours after hospitalization livedo reticularis at the level of the right thoracic wall and thighs. The nerve conduction studies revealed mononeuritis multiplex. The vascular anomaly that was identified in the presented case respects the criteria for a VBD, however the involvement of the aorta with the presence of tortuosities, the simultaneous presence of the internal carotid artery aneurysm, together with the presence of the mononeuritis multiplex and the skin condition (livedo reticularis) suggest the possibility of coexistence of a systemic vasculitis, possible polyarteritis nodosa. [ABSTRACT FROM AUTHOR]