Intracranial Artery Research Articles

RNF213 p.Arg4810Lys Variant Is Associated with Higher Stenosis Progression in Asymptomatic Intracranial Artery Stenosis.

Intracranial artery stenosis (ICAS) is a significant contributor to ischemic stroke, with the RNF213 p.Arg4810Lys variant identified as a related genetic factor. We explored the clinical outcomes of the RNF213 genotype in patients with asymptomatic ICAS. Between November 2011 and March 2019, 139 patients with asymptomatic ICAS were enrolled in this study. Genotyping for RNF213 p.Arg4810Lys was performed using Sanger sequencing. A comprehensive analysis was conducted to compare the RNF213 genotype with background characteristics and clinical outcomes such as ipsilateral ischemic cerebrovascular events and stenosis progression. RNF213 p.Arg4810Lys was found in 25% of cases, revealing distinct clinical features between carriers and non-carriers. The incidence of ipsilateral ischemic cerebrovascular events was 4.3% (6/139 cases), and stenosis progression was observed in 13% (18/139 cases) during a mean follow-up period of 58months. Stenosis progression rates were notably higher in the RNF213 variant group (25.7%; 9/35 cases) than in the RNF213 wild-type group (8.7%; 9/104 cases). Cumulative stenosis progression rate was significantly higher in the RNF213 variant group than in the RNF213 wild-type group (log-rank test, P = 0.0004). Multivariate Cox regression analysis indicated a significant association between the RNF213 p.Arg4810Lys variant and an increased risk of stenosis progression (P = 0.03, odds ratio 3.2; 95% confidence interval, 1.1-9.0). The RNF213 p.Arg4810Lys variant exhibits clinical disparities in asymptomatic ICAS and is notably linked to a heightened risk of stenosis progression. These results suggest a distinct difference in the vascular stenosis mechanism associated with this variant, warranting further investigation into its clinical implications and potential mechanistic insights.

Imaging characteristics and treatment strategies for carotid artery occlusion caused by skull base fracture.

The internal carotid artery occlusion caused by head and neck trauma, also known as traumatic intracranial artery occlusion, is relatively rare clinically. Traumatic skull base fracture is a common complication of traumatic brain injury. Traumatic skull base fracture is one of the causes of traumatic internal carotid artery occlusion. If not detected early and treated in time, the prognosis of patients is poor. This editorial makes a relevant analysis of this disease.

Efficacy and safety of drug-eluting stents versus bare-metal stents in symptomatic intracranial and vertebral artery stenosis: a meta-analysis

ObjectivesThis study aims to present the first comprehensive meta-analysis assessing the effectiveness and safety of drug-eluting stents (DES) versus bare-metal stents (BMS) in treating intracranial and vertebral artery stenosis.MethodsA comprehensive examination was undertaken to compare the effectiveness and safety of DES and BMS in individuals experiencing symptomatic stenosis in the intracranial and vertebral arteries through an in-depth analysis of clinical research. We conducted an extensive search across multiple databases including PubMed, Embase, Web of Science, and the Cochrane Library up to September 2024. The emphasis of our investigation was on various outcomes including rates of in-stent restenosis, symptomatic occurrences of in-stent restenosis, incidence of stroke, procedural success, mortality rates, complications associated with the procedure, and any adverse events.ResultsOur analysis included 12 studies with a total of 1,243 patients (562 in the DES group and 681 in the BMS group). The findings demonstrated a significantly lower rate of in-stent restenosis in the DES group for both intracranial [odds ratio (OR): 0.23; 95% confidence interval (CI): 0.13 to 0.41; p < 0.00001] and vertebral artery stenosis (OR: 0.38; 95% CI: 0.20 to 0.72; p = 0.003) compared to the BMS group. Additionally, the DES group showed a significantly reduced rate of postoperative strokes in vertebral artery stenosis cases (OR: 0.38; 95% CI: 0.16 to 0.90; p = 0.03), with no significant differences noted in the intracranial artery stenosis comparison (OR: 0.63; 95% CI: 0.20 to 1.95; p = 0.42). The study also revealed no significant disparities in symptomatic in-stent restenosis, procedural success, mortality, adverse effects, and perioperative complications between the two groups across the conditions studied.ConclusionThe comparison indicates that DES significantly reduces the risk of in-stent restenosis and postoperative strokes in patients with vertebral artery stenosis, compared to BMS. For both intracranial and vertebral artery stenosis, DES and BMS exhibit comparable safety profiles.Systematic review registrationhttps://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=439967.

Intracranial Internal Carotid Artery Pseudoaneurysm: A Narrative Review and Update for Otolaryngology Residents

Intracranial Internal Carotid Artery Pseudoaneurysm: A Narrative Review and Update for Otolaryngology Residents

Medial intracranial carotid artery calcifications and hematoma expansion in deep intracerebral hemorrhage.

Medial intracranial carotid artery calcifications (ICAC) are associated with impaired vascular physiology, increased arterial stiffness and pulse pressure. Their presence might therefore be associated with increased risk of intracerebral hemorrhage (ICH) expansion, according to the avalanche model. We explored the association between ICAC presence and pattern and hematoma expansion (HE). Retrospective analysis of a monocentric, prospectively collected cohort of ICH patients admitted between June 2017 and October 2023. ICAC pattern was determined by Kockelkoren's rating scale on admission CT; medial ICAC were defined with a >6 points cutoff. A follow-up CT scan was performed within 72 h. HE was analyzed as a dichotomous (≥6 mL and/or ≥33%) and as a categorical (none/mild/moderate/severe) variable, and its predictors were explored with logistic and ordinal regression respectively, accounting for baseline volume, onset-to-CT time, and anticoagulation. All the analyses were stratified by ICH location (supratentorial deep vs lobar ICH). A total of 201 patients were included (median age 78, 42% females, 59% deep ICH). Medial ICAC were significantly more common in deep ICH with HE compared with non-expanders (72% vs 49%, p = 0.03), whereas there was no association between ICAC and HE in lobar ICH (53% vs 52%, p = 0.85). This association between medial ICAC and HE in deep ICH remained significant in logistic (aOR 3.11, 95% CI [1.19-9.06], p = 0.03) and ordinal regression (acOR 2.42, 95% CI [1.19-4.99], p = 0.01). Ipsilateral medial ICAC are associated with higher odds of HE in deep ICH. Our findings are best interpreted as hypothesis generating, requiring prospective validation and further research to characterize the underlying biological mechanisms.

A computational method to predict cerebral perfusion flow after endovascular treatment based on invasive pressure and resistance

Background and objectivePredicting post-operative flow is essential for assessing the risk of adverse events in cerebrovascular stenosis patients following endovascular treatment (EVT). This study aimed to evaluate the accuracy of the CFD simulation model in predicting post-operative velocity, flow and pressure distal to a stenosis, based on cerebrovascular microcirculatory resistance. MethodsThe patient-specific models of the extracranial and intracranial arteries were reconstructed. The cerebrovascular microcirculatory resistance was applied to estimate post-operative blood velocity and flow rates. Pearson correlation and Bland-Altman analyses were used to evaluate the correlation and agreement between CFD calculations and transcranial Doppler (TCD) measurements. ResultsThere was a strong correlation between CFD- and TCD-based mean velocities (r=0.7733; P=0.0002), with volume flow measured by both methods also showing robust correlation (r=0.8621; P<0.0001). Additionally, agreement was found between mean velocities determined by CFD simulation and those estimated by TCD (P=0.2446, mean difference −4.2089; limits of agreement -11.5764 to 3.1586). However, agreement between volume flow from CFD simulations and TCD was less consistent (P=0.0387, mean difference -0.3272, limits of agreement -0.9276 to 0.2731). ConclusionsThe computational method used in this study enables the prediction of hemodynamic changes and offers valuable support in tailoring treatment strategies for cerebrovascular stenosis lesions.

Hypertension may associate with cerebral small vessel disease and infarcts through the pathway of intracranial atherosclerosis

Hypertension, a major modifiable risk factor for cardiovascular diseases, is linked to late-life neurocognitive disorders such as vascular dementia and Alzheimer's disease (AD). This study explores the associations between hypertension, intracranial atherosclerotic disease (ICAD), cerebral small vessel disease (cSVD), and Alzheimer's disease neuropathologic change (ADNC) in a large community-based autopsy study.This cross-sectional study used data from the Biobank for Aging Studies of the University of São Paulo Medical School. Sociodemographic and clinical information was gathered from a reliable next-of-kin informant. Neurofibrillary tangles, neuritic plaques, lacunar infarcts, hyaline arteriolosclerosis, and cerebral amyloid angiopathy were evaluated. Causal mediation analyses with natural effect models were performed to examine indirect associations of hypertension with cerebrovascular pathologies and ADNC through morphometric measurements of intracranial artery lumen obstruction.Hypertensive participants (n = 354) presented a higher rate of stenosed arteries (obstruction ≥ 50%), critically stenosed arteries (obstruction ≥ 70%), and more severe ICAD, shown by higher maximum and mean obstruction indexes compared to nonhypertensive participants (n = 166). These measurements of atherosclerosis were associated with neurofibrillary tangles and cSVD lesions. Hypertension was indirectly associated with hyaline arteriolosclerosis and lacunar infarcts through the pathway of ICAD. Presenting hypertension indirectly increased the odds of displaying hyaline arteriolosclerosis by 26% (95% CI: 1.08, 1.45, p = 0.002) and lacunar infarcts by 17% (95% CI: 1.01, 1.35, p = 0.029). Cognitive and APOE ε4 carrier status did not alter the investigated associations. In this community sample, hypertension was indirectly associated with cSVD through ICAD.

Association of renal biomarkers with fast progressor phenotype and related outcomes in anterior circulation large vessel occlusion stroke.

Renal dysfunction is a known predictor of long-term functional dependency after anterior circulation large vessel occlusion (ACLVO) stroke. However, the impact of renal dysfunction on early infarct growth rate (IGR) has not been previously demonstrated. The objective of this study was to define the association of creatinine-based renal biomarkers with fast or slow progressor phenotypes and related clinical outcomes in ACLVO stroke. This retrospective study examined patients with acute intracranial internal carotid artery or middle cerebral artery-M1 occlusions admitted between 2014 and 2019. Patients were included if they received baseline CT perfusion (CTP) or MRI on presentation within 24 h of estimated stroke onset. Infarct growth rate (IGR) was determined by ischemic core volume on CTP or MRI divided by time from stroke onset to imaging. IGR was used to stratify fast progressor (IGR ≥10 mL/h) and slow progressor (IGR < 10 mL/h) status. Renal dysfunction was assessed based on serum creatinine and estimated glomerular filtration rate (eGFR) on presenting laboratories. Logistic regression models, adjusted for significant covariates, identified independent associations between renal dysfunction biomarkers, progressor status, and clinical outcomes based on modified Rankin Scale (mRS) at 90 days. Among 230 patients with ACLVO, 29% were fast progressors, with median serum creatinine levels higher than slow progressors (1.1 vs. 0.9 mg/dL, p < 0.05) and lower median eGFR (66.2 vs. 69.0 mL/min/1.73m2, p < 0.05). Elevated creatinine (≥1.2 mg/dL) was independently associated with fast progressor status (adjusted OR 2.37, 95% CI 1.18-4.77), worse 90-day mRS (adjusted OR 1.88, 95% CI 1.01-3.51) and mortality (adjusted OR 2.57, 95% CI 1.14-5.79). Reduced eGFR (<60 mL/min/1.73m2) was independently associated with fast progressor status (adjusted OR 2.38, 95% CI 1.14-4.94), but not with 90-day mRS or mortality. Serum creatinine-based biomarkers of renal dysfunction were associated with fast progressor phenotype of ACLVO stroke, and worse clinical outcomes, which may help identify such patients earlier during emergency evaluation for expedited access to EVT. Future prospective studies are warranted to confirm and test implementation of these findings.

Endovascular therapy in patients with acute intracranial non-terminal internal carotid artery occlusion (ICA-I).

Acute intracranial occlusion of the internal carotid artery (ICA) can be distinguished into (a) occlusion of the terminal ICA, involving the proximal segments of the middle or anterior cerebral artery (ICA-L/-T) and (b) non-terminal intracranial occlusions of the ICA with patent circle of Willis (ICA-I). While patients with ICA-L/-T occlusion were included in all randomized controlled trials on endovascular therapy (EVT) in anterior large vessel occlusion, data on EVT in ICA-I occlusion is scarce. We thus aimed to evaluate effectiveness and safety of EVT in ICA-I occlusions in comparison to ICA-L/-T occlusions. A large international multicentre cohort was searched for patients with intracranial ICA occlusion treated with EVT between 2014 and 2023. Patients were stratified by ICA occlusion pattern, differentiating ICA-I and ICA-L/-T occlusions. Baseline factors, technical (modified thrombolysis in cerebral infarction (mTICI) scale) and functional outcomes (modified Rankin scale [mRS] at 3 months) as well as rates of (symptomatic) intracranial hemorrhage ([s]ICH) were analyzed. Of 13,453 patients, 1825 (13.6%) had isolated ICA occlusion. ICA-occlusion pattern was ICA-I in 559 (4.2%) and ICA-L/-T in 1266 (9.4%) patients. Age (years: 74 vs 73), sex (female: 45.8% vs 49.0%) and pre-stroke functional independency (pre-mRS ⩽ 2: 89.9% vs 92.2%) did not differ between the groups. Stroke severity was lower in ICA-I patients (NIHSS at admission: 14 [7-19] vs 17 [13-21] points). EVT was similarly successful with respect to technical (mTICI2b/3: 76.1% (ICA-I) vs 76.6% (ICA-L/-T); aOR 1.01 [0.76-1.35]) and functional outcome (mRS ordinal shift cOR 1.01 [0.83-1.23] in adjusted analyses. Rates of ICH (18.9% vs 34.5%; aOR 0.47 [0.36-0.62] and sICH (4.7% vs 7.3%; aOR 0.58 [0.35-0.97] were lower in ICA-I patients. EVT might be performed safely and similarly successful in patients with ICA-I occlusions as in patients with ICA-L/-T occlusions.

Essential thrombocythemia complicated by multivessel intracranial dolichoectasia with subarachnoid hemorrhage and recurrent cerebral infarction

BackgroundPatients with essential thrombocythemia (ET) can develop systemic thrombotic and hemorrhagic vascular complications due to increased and dysfunctional platelets.Case presentationWe encountered a 49-year-old Japanese woman with a history of essential ET, cerebral infarction, hypertension, and hyperlipidemia. She had been taking anagrelide (2.5 mg/day) and aspirin (100 mg/day) and had vomited at home. At the time of admission to our hospital, she was in a deep stupor. We thought these symptoms were caused by intracranial hypertension. Head computed tomography showed severe subarachnoid hemorrhage (SAH) at the interhemispheric fissure, hematoma at the corpus callosum, and intraventricular hemorrhages. Digital subtraction angiography demonstrated multiple dilated, stenotic, tortuous intracranial arteries, and a dissecting aneurysm at the right anterior cerebral artery (ACA). The dissecting aneurysm measured 7.7 × 15.5 mm, and a bleb was observed. Our diagnosis was ruptured ACA dissecting aneurysm and diffuse intracranial arterial dolichoectasia (DIADE). We performed stent-assisted coil embolization of the aneurysm. She remained apathetic and manifested unilateral apraxia and alien limb syndrome due to a partial callosal disconnection. Two months thereafter her symptoms gradually improved, her consciousness level and limb function improved, and her modified Rankin scale (mRS) score was 2. However, when we tapered her antiplatelet therapy, she again developed an acute cerebral infarct.ConclusionsThis is the first presentation of a patient with ET complicated by DIADE, both of which can result in ischemic and hemorrhagic vascular complications including SAH. As antithrombotic therapy in patients with DIADE and ET may be associated with higher hemorrhage rates, its effects must be monitored carefully.

Posterior short ciliary arteries ischemia following embolization of anterior ethmoid artery to treat dural arteriovenous fistulas: a case report

BackgroundThe Onyx™ Liquid Embolic System is a non-adhesive liquid embolic agent, which has been proved by the US FDA for embolization of lesions in the peripheral and neurovasculature since 2005. We reported a case of ischemic optic neuropathy after using Onyx-18 to embolize the anterior ethmoid arteries that feeding dural arteriovenous fistulas (DAVF).Case presentationA 57-year-old Asian male presented with anterior cranial fossa DAVF underwent embolotherapy by delivering Onyx-18 through a microcatheter into the anterior ethmoid arteries under angiography guidance. The interventional procedure was successful and no clear evidence was found pointing to untargeted occlusive embolus. But after the surgery the patient experienced delayed painless vision loss in the right eye (RE). The fundoscopy showed unilateral papilledema with pale optic disc in RE, accompanied by significant edema and thickening in the retinal nerve fiber layer (RNFL) of macula. The fundus fluorescence angiography showed that most of the optic disc in RE had postponed or absent fluorescence filling. Visual evoked potential (VEP) confirmed that the amplitude of the P100 component was decreased in RE without significant prolongation of the latency. The patient was diagnosed with anterior ischemic optic neuropathy, but immediate pulse steroid therapy failed to rescue his vision.ConclusionPreoperative evaluation of the patient’s hemodynamic status and fundus examination are essential for assessing the risk of ischemic ocular complications, and the non-adhesive liquid embolic agent Onyx-18 should be used cautiously during endovascular embolization of intracranial artery.

More severe cerebral small vessel disease associated with poor leptomeningeal collaterals in symptomatic intracranial atherosclerotic stenosis.

We investigated the association between cerebral small vessel disease (CSVD) and ipsilateral leptomeningeal collateral (LMC) status in patients with symptomatic intracranial atherosclerotic stenosis (sICAS). In 108 patients with 50-99% symptomatic intracranial internal carotid artery or M1 middle cerebral artery stenosis, 4 CSVD imaging markers (lacunes, cerebral microbleeds, enlarged perivascular spaces [EPVSs], and white matter hyperintensities [WMHs]) were assessed in MRI. Score of 0 or 1 was assigned to each marker and added up as a summary CSVD score (ranging 0-4) to reflect an overall CSVD burden. Ipsilateral LMC status was assessed by determining the laterality of distal vessels in anterior and posterior cerebral artery territories on CT angiography. Moderate-to-severe EPVSs (adjusted odds ratio [aOR] = 4.15; p = 0.031) and WMHs (aOR = 5.90; p = 0.010), and higher summary CSVD score (aOR = 1.66; p = 0.030) were independently associated with poor LMCs. There was significant interaction between stenosis percentage in sICAS and summary CSVD score on poor LMCs (p = 0.022 for interaction), when higher CSVD score was significantly associated with poor LMCs in patients with severe sICAS (aOR = 2.84; p = 0.011) but not in those with moderate sICAS. The findings indicated possibly adverse effect of CSVD on the recruitment or development of LMCs in sICAS patients, especially in patients with severe sICAS.

Site-specific m6A-miR-494-3p, not unmethylated miR-494-3p, compromises blood brain barrier by targeting tight junction protein 1 in intracranial atherosclerosis.

Intracranial atherosclerosis is one of the most common causes of ischaemic stroke. However, there is a substantial knowledge gap on the development of intracranial atherosclerosis. Intracranial arteries are characterized by an upregulation of tight junctions between endothelial cells, which control endothelial permeability. We investigated the role of N6-methyladenosine (m6A), a common RNA modification, on endothelial integrity, focusing on the pro-atherogenic microRNA miR-494-3p and tight junction proteins TJP1 and PECAM1. We assessed the m6A landscape, along with the expression of miR-494-3p, TJP1 and PECAM1 in postmortem human vertebral arteries (VA), internal carotid arteries (ICA), and middle cerebral arteries (MCA) with various stages of intimal thickening and plaque formation. The interactions between m6A-modified miR-494-3p mimics, TJP1 and PECAM1, were investigated in vitro using primary human (brain) endothelial cells. Increased m6A expression was observed in the luminal lining of atherosclerosis-affected VAs, accompanied by reduced TJP1 and PECAM1, but not VE-cadherin, expression. Colocalization of m6A and miR-494-3p in the luminal lining of VA plaques was confirmed, indicating m6A methylation of miR-494-3p in intracranial atherosclerosis. Moreover, site-specific m6A-modification of miR-494-3p led to repression specifically of TJP1 protein expression at cell-cell junctions of brain microvascular endothelial cells, while unmodified miR-494-3p showed no effect. This study highlights increasing m6A levels during intracranial atherogenesis. Increases in m6A-miR-494-3p contribute to the observed decreased TJP1 expression in endothelial cell-cell junctions. This is likely to have a negative effect on endothelial integrity and may thus accelerate intracranial atherosclerosis progression.

Prognostic impact of intracranial arteriosclerosis subtype after endovascular treatment for acute ischaemic stroke.

The influence of intracranial arteriosclerosis (ICAR) on acute ischaemic stroke (AIS) prognosis is unclear. This study explored its impact, focusing on ICAR subtypes categorized by intracranial carotid artery calcification (ICAC) patterns: intimal or atherosclerotic versus internal elastic lamina calcification or non-atherosclerotic. The aim was to determine their effect on AIS prognosis in patients undergoing endovascular treatment (EVT). This prospective cohort study included consecutive AIS patients with anterior circulation large vessel occlusion undergoing EVT. ICAC, the hallmark of ICAR, was assessed using non-contrast computed tomography to quantify volume and establish the predominant ICAR subtype. The primary outcome was long-term functional outcome, measured by the 90-day modified Rankin Scale score. Secondary outcomes included first-pass effect, revascularization degree, symptomatic intracranial haemorrhage and 24-h infarct volume. Multivariate-adjusted linear and logistic regression models were used to assess the association of ICAC volume and subtype with these outcomes. From January 2021 to February 2022, 181 patients were included, of whom 172 (95%) had ICAC. Internal elastic lamina calcification was the predominant subtype in 103 (57%), intimal in 52 (29%) and mixed in 17 (9%). The intimal or atherosclerotic ICAC pattern was linked to poorer functional outcomes (adjusted odds ratio 2.12, 95% confidence interval [CI] 1.10-4.09), decreased first-pass effect probability (adjusted odds ratio 0.42, 95% CI 0.21-0.84) and higher infarct volume (adjusted β value 22.11, 95% CI 0.55-43.67). A predominant intimal ICAC subtype, linked to underlying atherosclerosis, correlated with larger infarct volume and poorer 90-day functional outcomes in EVT-treated AIS patients. Intracranial atherosclerosis appears to be a relevant factor hampering clinical benefits post-EVT.

Superior cervical ganglionectomy attenuates vascular remodeling in spontaneously hypertensive rats.

Sympathetic hyperactivity contributes to the pathogenesis of hypertension. However, it is unclear whether the excessive sympathetic activity is an independent and crucial factor for vascular remodeling in hypertension. This study focused on the effect of local sympathetic denervation with superior cervical ganglionectomy (SCGx) on vascular remodeling. Surgical bilateral SCGx was performed in 9-week-old male Wistar-Kyoto rats (WKY) and spontaneously hypertensive rats (SHR). Control rats received sham-operation without SCGx. All measurements were made 4 weeks after the surgery. The effectiveness of SCGx was confirmed by the eye features of Horner syndrome, greatly reduced tyrosine hydroxylase (TH) contents in the superior cervical ganglion (SCG)-innervated arteries in the head. Although SCGx had no significant effects on blood pressure and heart rate in WKY and SHR, it attenuated vascular remodeling of facial artery and superficial temporal artery in SHR, two representative SCG-innervated extracranial arteries, without significant effects on non-SCG-innervated thoracic aorta and mesenteric artery. SCGx-treated SHR had more auricular blood flow and retina microvasculature than sham-operated SHR. However, SCGx had only a mild effect in attenuating the vascular remodeling of basilar artery and middle cerebral artery, two representative SCG-innervated intracranial arteries, in SHR. SCGx-treated SHR exhibited upregulation of α-smooth muscle actin, downregulation of proliferating cell nuclear antigen, and attenuation of oxidative stress and inflammation in facial artery and superficial temporal artery. Sympathetic denervation by SCGx in SHR attenuated local vascular remodeling, suggesting that sympathetic overactivity is a crucial pathogenic factor of vascular remodeling in SHR.

Results of Using Different Brain Reperfusion Methods in Patients with Acute Ischemic Stroke

Acute ischemic stroke (AIS) occupies one of the leading places in the structure of disability and mortality in adult population throughout the world, which causes a significant social and economic burden of this pathology. Recent advances in AIS diagnosis and treatment have significantly reduced mortality and improved functional outcomes in patients. However, categories of patients with unsatisfactory results and complications of reperfusion therapy still remain. In some of them, mortality may exceed the overall rate. To this day, there are controversial aspects in determining indications and choosing the preferred method of cerebral reperfusion. The presented cases describe the results of using thrombolytic therapy and thromboextraction in AIS patients of different age groups. The importance of various neuroimaging methods (multislice computed tomography, computed angiography, duplex ultrasound of extra- and intracranial arteries) for timely diagnosis and determination of optimal revascularization tactics was noted.

Vascular Abnormalities and Neurofibromatosis Type 1: A Paediatric Case Series.

Neurofibromatosis type 1 (NF1) is a multisystemic neurocutaneous disease caused by a heterozygous mutation of the NF1 gene that encodes neurofibromin. Complications include vascular and neurologic abnormalities such as moyamoya syndrome, a cerebrovascular disorder with progressive occlusion of the large intracranial arteries, leading to ischemic events and the formation of abnormal vascular networks. Stenosis of the renal artery is another frequent complication of neurofibromatosis type 1, and it represents the most common cause of secondary hypertension in these patients. The purpose of the article is to describe the clinical manifestations of neurofibromatosis type 1 vasculopathy in 4 patients presenting with a wide range of neurologic and reno-vascular manifestations, as well as to examine current diagnostic management and follow-up, current therapeutic options, and to discuss further perspectives in terms of screening, diagnosis, and treatment.

Angioinvasive Rhino-Orbital-Cerebral Mucormycosis in a Patient with Type 2 Diabetes Mellitus: A Complex and Lethal Cause of Stroke

Rhino-orbital-cerebral mucormycosis is a rapidly progressive and often fatal fungal infection caused by molds of the order Mucorales, particularly affecting immunocompromised individuals. This infection is notorious for its angioinvasive properties, enabling the fungi to invade blood vessels and leading to tissue necrosis. We report the clinical course of a 59-year-old Caucasian man with poorly controlled type 2 diabetes (HbA1c 16.8%) who presented with unilateral headache, left-sided facial numbness, and incomplete left ocular motor paresis. Initial presentation raised suspicion of orbital phlegmon, leading to antibiotic and later corticosteroid pulse therapy, which worsened the patient’s condition. Subsequent imaging demonstrated extensive inflammatory changes, including wall irregularities of the left intracranial internal carotid artery, accompanied by ocular protrusion and periorbital enhancement. New palatal lesions indicated mucormycosis, which was confirmed by molecular analysis of a palatal biopsy, leading to Amphotericin B treatment. Pre-surgery imaging revealed a malignant middle cerebral artery infarction, and the patient died 16 days after symptom onset and 12 days after initial presentation under palliative care due to a poor prognosis. This case of angioinvasive mucormycosis underscores the severe and often fatal course of rhino-orbital-cerebral mucormycosis in an immunocompromised individual. The rapid progression from initially vague and unspecific symptoms to extensive vascular involvement and stroke highlights the critical need for early and accurate diagnosis, as well as prompt intervention to prevent further disease progression. Additionally, this case also illustrates the potential risks associated with corticosteroid therapy in the presence of undiagnosed fungal infections, which can exacerbate the condition and lead to serious complications. Clinicians should maintain a high index of suspicion for mucormycosis in similar clinical scenarios, prioritizing adequate antifungal treatment and careful monitoring to improve patient outcomes. Early interdisciplinary collaboration is essential for the effective management of such complex cases.

Tyrosine Kinase Inhibitor-Induced Intracranial Artery Stenosis - A Rare Cause of TIA in a Patient of Chronic Myeloid Leukemia.

Tyrosine Kinase Inhibitor-Induced Intracranial Artery Stenosis - A Rare Cause of TIA in a Patient of Chronic Myeloid Leukemia.

Treatment-Related Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but significant cause of intracranial arteriopathy and stroke in young adults. The syndrome encompasses a spectrum of disorders radiologically characterized by reversible narrowing and dilation of intracranial arteries, often triggered by vasoactive drugs or the postpartum period. The hallmark clinical feature of RCVS is thunderclap headache with or without other neurological signs. Though endothelial dysfunction and sympathetic hyperactivation are hypothesized to be key mechanisms, the exact pathogenesis of RCVS is still unclear. RCVS’s diagnosis could be challenging, since vasospasm proceeds centripetally, initially involving distal small pial and cortical arteries, and angiographic studies, especially brain magnetic resonance angiography (MRA) and computed tomography angiography (CTA), may miss it in the early phase of the disease, while early signs such as vascular hyperintensities may be visible on T2/FLAIR sequences before vasospasm onset. Catheter angiography is the gold standard and it could be used to assess vasospasm reversibility post-intra-arterial vasodilator administration. Treatment is mainly symptomatic, and nimodipine is the most commonly administered therapy, given orally or intra-arterially in severe cases. Since many aspects of RCVS remain partially known, further research is needed to better understand the complex pathophysiology of this unique clinical condition and to optimize specific management strategies.