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Background: Hashimoto's disease has reportedly been associated with stroke; however, cerebrovascular morphology and clinical course remain poorly documented. The present study aimed to determine the angiographic features and clinical outcomes of intracranial arterial stenosis (IAS) associated with Hashimoto's disease in a retrospective cohort., Methods: Overall, 107 adult patients with IAS were screened for anti-thyroid antibodies; of these, 26 patients tested positive. The 42 affected hemispheres were classified into subgroups according to the steno-occlusion site and the development of abnormal collateral (moyamoya) vessels. These subgroups were dichotomized into moyamoya vessels positive (MM type) and negative (non-MM type). The initial presentation, IAS progression, and vascular events during the follow-up period were compared., Results: The following sites of stenosis were identified: the bifurcation of the internal carotid artery in 11 (26.2%), M1 or A1 in 29 (69.0%), and more distal (M2-M4/A2-A4) in 2 (4.8%) hemispheres. Further, 17 hemispheres were categorized into the MM type and 25 were classified into the non-MM type. During the follow-up period (mean 2.5 years), IAS progression was identified in 8 (32%) hemispheres of the non-MM type and 0 (0%) hemispheres of the MM type (p = 0.041). Ischemic attacks occurred in 5 (20.0%) hemispheres of the non-MM type (4.6%/year) and 0 hemispheres of the MM type (p = 0.08). Further, 4 (23.5%) hemispheres of the MM type experienced intracerebral hemorrhage, whereas none of the non-MM type hemorrhaged (p = 0.012)., Conclusions: Hashimoto's disease-associated IAS exhibits various angiographic morphologies, resulting in different clinical presentations. Screening for anti-thyroid antibodies and careful management based on vascular morphology appears important in adults with IAS.

The utility of endovascular thrombectomy for acute occlusion of the distal intracranial artery (A2/A3/M2/M3/P2/P3) is unclear, and aspiration and stent thrombectomy are associated with risk of bleeding. We analyzed patients with acute occlusion of the distal intracranial artery to assess the safety and efficacy of microcatheter-based tirofiban infusion.We retrospectively reviewed data of the endovascular thrombectomy registry of our center between January 2018 and June 2019. Patients with distal intracranial artery occlusion who underwent endovascular thrombectomy with microcatheter-based infusion of tirofiban were recruited.Of 13 patients included, 1 presented with anterior cerebral artery occlusion, 2 with posterior cerebral artery occlusion, 2 with posterior inferior cerebellar artery occlusion, and 7 with middle cerebral artery M2 occlusion. The mean National Institute of Health Stroke scale score was 10.1 (3-19). Three patients (23.1%) underwent bridging treatment of intravenous thrombolysis with recombinant plasminogen activator and endovascular thrombectomy. The arithmetic mean onset-to-recanalization time was 696.3 minutes (140-1440) and average operating time was 47.1 minutes (30-80). After treatment, 10 patients (76.9%) underwent revascularization. No operative complications were observed in any case. All patients underwent angiography and were reviewed 7 to 14 days after surgery. Imaging revealed significant improvements in recanalization compared with the immediate postoperative period, with no reoccurrence of occlusion. The mean modified Rankin scale score at the 3-month follow-up was 0.54 (0-2).Microcatheter-based infusion of bolus-dose tirofiban can result in safe and effective recanalization of acute occlusion of the distal artery in the case of a relatively light thrombotic load.

Background and Purpose: Direct presentation of patients with acute ischemic stroke to a comprehensive stroke center (CSC) reduces time to endovascular treatment (EVT), but may increase time to treatment for intravenous thrombolysis (IVT). This dilemma, however, is not applicable to patients who have a contraindication for IVT. We examined the effect of direct presentation to a CSC on outcomes after EVT in patients not eligible for IVT., Methods: We used data from the MR CLEAN Registry (2014-2017). We included patients who were not treated with IVT and compared patients directly presented to a CSC to patients transferred from a primary stroke center. Outcomes included treatment times and 90-day modified Rankin Scale scores (mRS) adjusted for potential confounders., Results: Of the 3637 patients, 680 (19%) did not receive IVT and were included in the analyses. Of these, 389 (57%) were directly presented to a CSC. The most common contraindications for IVT were anticoagulation use (49%) and presentation > 4.5 h after onset (26%). Directly presented patients had lower baseline NIHSS scores (median 16 vs. 17, p = 0.015), higher onset-to-first-door times (median 105 vs. 66 min, p < 0.001), lower first-door-to-groin times (median 93 vs. 150 min; adjusted β = - 51.6, 95% CI: - 64.0 to - 39.2) and lower onset-to-groin times (median 220 vs. 230 min; adjusted β = - 44.0, 95% CI: - 65.5 to - 22.4). The 90-day mRS score did not differ between groups (adjusted OR: 1.23, 95% CI: 0.73-2.08)., Conclusions: In patients who were not eligible for IVT, treatment times for EVT were better for patients directly presented to a CSC, but without a statistically significant effect on clinical outcome.

Ischemic and hemorrhagic stroke can occur in the setting of pediatric trauma, particularly those with head or neck injuries. The risk of stroke appears highest within the first two weeks after trauma. Stroke diagnosis may be challenging due to lack of awareness or concurrent injuries limiting detailed neurological assessment. Other injuries may also complicate stroke management, with competing priorities for blood pressure, ventilator management, or antithrombotic timing. Here we review epidemiology, clinical presentation, and diagnostic approach to blunt arterial injuries including dissection, cerebral sinovenous thrombosis, mineralizing angiopathy, stroke from abusive head trauma, and traumatic hemorrhagic stroke. Owing to the complexities and heterogeneity of concomitant injuries in stroke related to trauma, a single pathway for stroke management is impractical. Therefore providers must understand the goals and possible costs or consequences of stroke management decisions to individualize patient care. We discuss the physiological principles of cerebral perfusion and oxygen delivery, considerations for ventilator strategy when stroke and lung injury are present, and current available evidence of the risks and benefits of anticoagulation to provide a framework for multidisciplinary discussions of cerebrovascular injury management in pediatric patients with trauma., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Mechanical thrombectomy (MT) failure is associated with very poor prognosis. Permanent intracranial stenting (PIS) may be useful in such refractory occlusions. However, this strategy requires an aggressive antithrombotic regimen that may be harmful in extended strokes. The aim of this study was to compare clinical outcomes between patients with refractory acute large vessel occlusions (LVOs) treated by PIS versus patients for whom the procedure was stopped without recanalization., Methods: We conducted a systematic review by searching for articles in PubMed, the Cochrane Library, and ClinicalTrials.gov from January 2015 to September 2019. Two reviewers independently selected studies comparing PIS after failed MT in addition to usual care versus usual care only. A comparative meta-analysis was performed using random-effects models to estimate odds ratios of favorable clinical outcome at 90 days, defined as a modified Rankin scale 0-2, mortality and symptomatic intracranial hemorrhage (SICH)., Results: Four comparative studies were included for a total of 352 patients: 149 in the PIS group versus 203 in the control group. PIS was associated with significantly higher rates of 90-day favorable clinical outcome (odds ratio [OR], 2.87 [95% confidence interval (95% CI), 1.77-4.66]; p < 0.001; I 2 , 0%) and lower mortality (OR, 0.39 [0.16-0.93]; p = 0.03; I 2 , 43%), whereas SICH rates did not significantly differ (OR, 0.68 [0.37-1.27]; p = 0.23; I 2 , 0%)., Conclusion: From observational study results, attempting PIS after failed MT seems to improve clinical outcomes without increasing the risk of intracranial bleeding. Randomized trials are needed to confirm these results.

Acute ischemic stroke is a severe and life-threatening disease, particularly when caused by a large-vessel occlusion. The only available 2 treatment options are intravenous alteplase and endovascular therapy (mechanical clot removal), both of which are highly time-dependent. Thus, rapid patient transfer, diagnosis, and treatment are crucial, and time-consuming imaging methods and overly selective treatment selection criteria should be avoided. A combined endovascular therapy approach using stent-retrievers and aspiration is the most effective way to achieve fast first-pass complete reperfusion and should thus be used. To diagnose and treat patients as fast as possible, the organization of existing systems of care, and particularly pre-hospital transfer systems, have to be changed. Several different transport models are currently in use because the optimal patient transfer paradigm is highly dependent on local geography and hospital efficiency., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Chronic subdural hematoma (CSDH) remains a neurosurgical condition with high recurrence rate after surgical treatment. The primary pathological mechanism is considered to be repeated microbleedings from fragile neo-vessels within the outer hematoma membrane. The neo-vessels are supplied from peripheral branches of the middle meningeal artery, and embolization of MMA (eMMA) has been performed to prevent re-bleeding episodes and thereby CSDH recurrence., Objective: To evaluate the published evidence for the effect of eMMA in patients with recurrent CSDH. Secondarily, to investigate the effect of eMMA as an alternative to surgery for primary treatment of CSDH., Method: A systematic review of the literature on eMMA in patients with recurrent CSDH was conducted. PubMed, Embase, and Cochrane databases were reviewed using the search terms: Embolization, Medial Meningeal Artery, Chronic Subdural Haematoma, and Recurrence. Furthermore, the following mesh terms were used: Chronic Subdural Haematoma AND embolization AND medial meningeal artery AND recurrence. Eighteen papers were found and included. No papers were excluded. The number of patients with primary CSDH and the number of patients with recurrent CSDH treated with eMMA were listed. Furthermore, the number of recurrences in both categories was registered., Results: Eighteen papers with a total of 191 included patients diagnosed with CSDH treated with eMMA for primary and recurrent CSDH were identified. Recurrence rate for patients treated with eMMA for recurrent CSDH was found to be 2.4%, 95% CI (0.5%; 11.0%), whereas the recurrence rate for patients treated with eMMA for primary CSDH was 4.1%, 95% CI (1.4%; 11.4%)., Conclusion: eMMA is a minimally invasive procedure for treatment of CSDH. Although this study is limited by publication bias, it seems that this procedure may reduce recurrence rates compared with burr hole craniostomy for both primary and recurrent hematomas. A controlled study is warranted.

Intracranial artery dissection (IAD) is an underdiagnosed, non-atherosclerotic cause of stroke with various clinical manifestations. To identify all the potential studies investigating the epidemiology, risk factors, symptoms, radiology findings, and treatment methods of IAD, we conducted a literature search screening PubMed, SCOPUS, EMBASE, and BIREME. According to the results of several studies, IAD is the major cause of ischemic stroke in at least one-third of the cervical-cranial artery dissection (CCAD) cases presenting with ischemic stroke. Mechanical causes are associated with cervical artery dissections (CAD) in up to 40 % of the cases. However, the risk factors for IAD are still not completely understood. Antithrombotic therapy with either antiplatelet or classic anticoagulants is the mainstay of treatment for preventing further thromboembolic complication after a stroke. Endovascular or surgical treatment options can be considered when medical therapies are not effective or when there is a high rate of recurrence or increased risk of bleeding. The observational studies have shown that these methods are very effective in preventing recurrence and significantly improving morbidity and mortality in patients with ruptured dissections. Clinical trials are required to establish the best option for each mechanism of ischemic lesion., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: Approved alternatives in the guidelines for acute ischemic stroke patients who have failed intracranial thrombectomy are lacking. Primary permanent intracranial stenting was initially used in the era before thrombectomy and might still be a useful rescue treatment in acute stroke patients suffering from ongoing large vessel occlusion refractory to thrombectomy., Methods: The prospectively collected registry of patients with acute stroke caused by large vessel occlusions and treated with the emboTrap® device in Karolinska Hospital from October 2013 through March 2017 were retrospectively reviewed. Clinical outcome of non-recanalized patients with a thrombolysis in cerebral infarction (TICI) score of 0-1 after failed thrombectomy were compared with those who were treated with permanent intracranial stenting as rescue therapy. Favorable outcome was defined as modified Rankin scale 0-2., Results: The emboTrap® device was used in 201 patients. Persistent re-occlusions on withdrawal of the thrombectomy device were seen in 26 patients (13%) and of those, 12 individuals (46%) were treated with intracranial stenting. Baseline National Institutes of Health stroke scale (NIHSS), occlusion site, and onset-to-puncture time did not differ between the stenting group and the non-recanalized group. During the procedure half dose (5/12 patients) or full dose abciximab (6/12 patients), or aspirin (1/12 patient) was given intravenously immediately after stent placement. In 2 patients (17%) multiple stents were implanted. The stenting group had better functional outcomes at 3 months compared to the non-stenting group with 8/12 (66%) vs. 3/14 (21.4%, p < 0.05). Of the patients 5 (36%) in the non-stented group had died at 3 months follow-up, whereas mortality in the stenting cohort was 0% (p < 0.05) and no symptomatic intracranial hemorrhage (ICH) occurred in either group., Conclusion: Intracranial stenting after failure of recanalization with thrombectomy led to a better rate of clinical outcome than leaving the patient non-recanalized. The required antiplatelet therapy, predominantly abciximab, did not lead to additional ICH.

The term vascular cognitive disorder (VCD) refers to a heterogeneous group of disorders in which the primary feature is cognitive impairment attributable to cerebrovascular disease (CVD). This includes not only vascular dementia (VaD) but also cognitive impairment of insufficient severity to meet diagnostic criteria for dementia. VCD is recognized as the second most common cause of dementia after Alzheimer's disease (AD), but prevalence rates vary widely according to the diagnostic criteria employed. There have been recent attempts to standardize diagnostic criteria. VCD incorporates a range of neuropathological mechanisms including poststroke impairment, small and large vessel disease, and cases of mixed-pathology, with CVD interacting with AD and other neuropathologies. Recent neuroimaging data have improved our understanding of the etiology of VCD. Symptomatic treatments for VaD have modest benefit and there is increased focus on the primary and secondary preventative benefits of vascular risk factor control., Competing Interests: None., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Sickle cell disease (SCD) is one of the most common autosomal recessive diseases in humans, occurring at a frequency of 1 in 365 African-American and 1 in 50 sub-Saharan African births. Despite progress in managing complications of SCD, these remain a major health burden worldwide. Stroke is a common and serious complication of SCD, most often associated with steno-occlusive cerebral arteriopathy, but little is known about its pathogenesis. Transcranial Doppler ultrasonography is currently the only predictive test for future development of stroke in patients with sickle cell anemia and is used to guide preventative treatment. However, transcranial Doppler ultrasonography does not identify all patients at increased risk for stroke, and progressive arteriopathy may occur despite preventative treatment. While sibling studies have shown a strong genetic contribution to the development of steno-occlusive arteriopathy (SOA) in SCD, the only genome-wide association study compared a relatively small cohort of 177 patients with stroke to 335 patients with no history of stroke. This single study detected variants in only 2 genes, ENPP1 and GOLGB1, and only one of these was confirmed in a subsequent independent study. Thus, the underlying genes and pathogenesis of SOA in SCD remain poorly understood, greatly limiting the ability to develop more effective preventive therapies. Dissecting the molecular causes of stroke in SCD will provide valuable information that can be used to better prevent stroke, stratify risk of SOA, and optimize personalized medicine approaches., (Copyright © 2018. Published by Elsevier Inc.)

Background: The effectiveness of thrombectomy for acute ischemic stroke has been established, and earlier treatment produces better outcomes. If possible to identify large-vessel occlusion (LVO) at the prehospital phase, eligible patients can be shipped directly to a hospital that can perform thrombectomy. The purpose of this study was to determine factors that are specific to LVO and can be known before hospital arrival., Methods: The subjects were stroke patients during the period between July 2014 and June 2016, who had a National Institutes of Health Stroke Scale (NIHSS) score of 8 or higher and came to our hospital within 6 hours of onset. These patients were divided into an LVO group and a non-LVO group, and background factors, mode of onset, individual NIHSS item scores, and blood pressure at the time of the visit were retrospectively investigated. The selected factors were compared with LVO prediction scales reported in the past., Results: There were 196 stroke patients who had NIHSS scores of 8 or higher and arrived at the hospital within 6 hours. Of these 196 patients, 56 had LVO. This LVO group included a significantly higher number of patients with the 2 items of atrial fibrillation (odds ratio [OR], 11.5: 95% confidence interval [CI], 4.04-32.9; P < .0001) and systolic blood pressure of 170 mm Hg or lower (OR, 2.99: 95% CI, 1.33-6.71, P = .008). These 2 items predicted LVO equally to existing LVO prediction scales., Conclusions: The 2 items of atrial fibrillation and systolic blood pressure of 170 mm Hg or lower were significantly correlated with LVO., (Copyright © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Purpose of Review: We aimed to summarize recent findings in cervical (CeAD) and intracranial artery dissection (IAD) research., Recent Findings: Considered a disease of the young- and middle-aged, an analysis on the largest CeAD-population to date (n = 2391) revealed that about 1 of 14 CeAD-patients was aged ≥60 years. Distinct genetic variants were associated with CeAD. However, in clinical practice, genetic investigations are not helpful due to the small effect size. Despite the paucity of data from randomized-controlled trials in CeAD-stroke patients, both intravenous thrombolysis and endovascular treatment should be considered as acute treatment in such patients. Future research is needed to clarify which patients benefit most from each treatment modality. Whether to use antiplatelets or anticoagulants in stroke prevention in CeAD-patients is still a matter of debate. One randomized-controlled feasibility trial has been published, and another trial designed to show non-inferiority of aspirin to vitamin-K-antagonists is underway and will be terminated in late 2018. Non-vitamin-K-oral anticoagulants should not be used in CeAD outside a properly designed trial, as experience with these drugs in CeAD-patients is limited. With many IAD patients developing intracranial hemorrhage, antithrombotic therapy should be used with caution. Knowledge about CeAD and IAD has advanced substantially. Nevertheless, further research is mandatory, in particular regarding pathophysiology, acute treatment, and stroke-preventive therapy, as well as long-term outcome and prognosis.

Background and Purpose: The purpose of this study was to investigate the frequency of symptomatic in-stent restenosis (ISR) and its contribution to nonprocedural symptomatic infarction in the SAMMPRIS trial (Stenting and Aggressive Medical Management for the Prevention of Recurrent Stroke in Intracranial Stenosis)., Methods: Patients without a periprocedural primary end point were followed up to determine the occurrence of any of the following events: ischemic stroke, cerebral infarct with temporary signs, or transient ischemic attack in the territory of the stented artery. Vascular imaging performed after these events was reviewed for ISR. Annual rates for symptomatic ISR were calculated using Kaplan-Meier estimates., Results: Of 183 patients in the stenting group without a periprocedural primary end point, 27 (14.8%) had a symptomatic infarction (stroke or cerebral infarct with temporary signs) and 16 (8.7%) had transient ischemic attack alone in the territory during a median follow-up of 35.0 months. Of the 27 patients with infarctions, 17 (9.3%) had an ischemic stroke and 10 (5.5%) had a cerebral infarct with temporary signs alone. Adequate vascular imaging to evaluate ISR was available in 24 patients with infarctions (showing ISR in 16 [66.7%]) and in 10 patients with transient ischemic attack alone (showing ISR in 8 [80%]). The 1-, 2-, and 3-year rates (with 95% confidence limits) for symptomatic ISR in the SAMMPRIS stent cohort were 9.6% (6.1%-14.9%), 11.3% (7.5%-17.0%), and 14.0% (9.6%-20.2%), respectively., Conclusions: Symptomatic ISR occurred in at least 1 of 7 patients in SAMMPRIS by 3 years of follow-up and was likely responsible for the majority of nonprocedural cerebral infarctions., Clinical Trial Registration: URL: https://clinicaltrials.gov. Unique identifier: NCT00576693., (© 2017 American Heart Association, Inc.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Objectives: Severe symptomatic intracranial stenosis is an important cause of stroke. Intracranial stenting is alternatively applied to treat intracranial atherosclerotic disease. However, Stenting versus Aggressive Medical Therapy for Intracranial Arterial Stenosis trial (SAMMPRIS) and Vitesse Stent Ischemic Therapy trial (VISSIT) both demonstrated intracranial stenting were inferior to aggressive medical treatment. But careful patient selection probably can improve the outcome of stenting in intracranial artery stenosis. Therefore, the validation of risk factors associated with serious adverse events (SAEs) after intracranial stenting may contribute to identify patients who are at high risk of stenting therapy and benefit patient selection for stenting., Patients and Methods: Patients who underwent intracranial stenting with symptom attributable to severe (>70%) intracranial stenosis were included in our institution. In-hospital SAEs after procedure were reviewed. Risk factors associated with SAEs were analyzed using multivariable logistic regression analysis., Result: Thirty serious adverse events (5.1%) occurred among a total of 583 patients, with a mean age of 58.1±9.7, including 13 ischemic strokes, 12 brain hemorrhages and 5 deaths. Bivariate analysis and multivariable logistic regression analysis showed age (OR=0.94, 95% CI:0.900-0.983), history of DM (OR=2.439, 95% CI:1.107-5.371), preprocedural mRS score (OR=3.076, 95% CI:1.290-7.336) and lesion site in BA (OR=9.056, 95% CI:1.147-71.524) were risk factors associated with SAEs., Conclusion: History of DM and lesion site in BA were risk factors associated with postprocedural in-hospital SAEs after stenting of severe symptomatic intracranial stenosis. But considering of the limitation of this retrospective study, further studies are necessary to confirm our results., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Background: Fractional flow reserve (FFR)-guided revascularization strategy is popular in coronary intervention. However, the feasibility of assessing stenotic severity in intracranial large arteries using pressure gradient measurements still remains unclear., Methods: Between March 2013 and May 2014, 12 consecutive patients with intracranial large artery stenosis (including intracranial internal carotid artery, middle cerebral M1 segment, intracranial vertebral artery, and basilar artery) were enrolled in this study. The trans-stenotic pressure gradient was measured before and/or after percutaneous transluminal angioplasty and stenting (PTAS), and was then compared with percent diameter stenosis. A Pd /Pa cut-off of ≤0.70 was used to guide stenting of hemodynamically significant stenoses. The device-related and procedure-related serious adverse events and recurrent cerebral ischemic events were recorded., Results: The target vessel could be reached in all cases. No technical complications occurred due to the specific study protocol. Excellent pressure signals were obtained in all patients. For seven patients who performed PTAS, the mean pre-procedural pressure gradient decreased from 59.0 ± 17.2 to 13.3 ± 13.6 mm Hg after the procedure (P < 0.01). Only one patient who refused stenting experienced a TIA event in the ipsilateral MCA territory. No recurrent ischemic event was observed in other patients., Conclusion: Mean trans-stenotic pressure gradients can be safely and easily measured with a 0.014-inch fluid-filled guide wire in intracranial large arteries. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Background: An unfused or twiglike middle cerebral artery (MCA) is a congenital anomaly related to the abnormal development of the MCA. An unfused or twiglike MCA can cause both ischemic and hemorrhagic strokes. Previous reports have shown that an unfused or twiglike MCA with coexisting aneurysms can cause subarachnoid hemorrhage (SAH)., Cases: Two patients presented with nonaneurysmal SAH due to an unfused or twiglike MCA. Both patients had stenosis of the proximal M1 segment of the left MCA and abnormal vascular nets distal to the stenotic lesion. Vasculature distal to the abnormal vascular nets remained grossly normal. These findings were not evident in computed tomography or magnetic resonance angiography., Conclusion: An unfused or twiglike MCA can cause nonaneurysmal SAH. Digital subtraction angiography is necessary for accurate diagnosis., (Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Atherosclerosis is not the only cause of intracranial arterial disease. Arterial dissection, moyamoya disease, vascular inflammatory disease, vasospasm and immunologic disorders are important non-atherosclerotic intracranial arterial diseases. Identification of the correct etiology is important in establishing treatment strategies and assessing prognosis. Careful history taking and appropriate laboratory testing are essential. Although catheter angiography is the most important diagnostic tool to examine various intracranial arterial diseases, other diagnostic modalities such as CT angiography and MR angiography are nowadays widely used. High resolution vessel wall MRI also can assist in making the correct diagnosis as this can yield information regarding vessel wall pathology. Certain diseases such as infectious vasculopathies and moyamoya disease are more prevalent in certain parts of the world, and physicians practicing in these regions should be mindful of these disorders. In this chapter, these non-atherosclerotic intracranial arterial diseases are discussed. Moyamoya disease will be described in another chapter., (© 2016 S. Karger AG, Basel.)

Up to 12% of patients with stroke have intracranial arterial dolichoectasia (IADE) and the basilar artery is affected in 80% of these cases. Diagnostic criteria and prognosis studies of IADE are based on basilar artery diameter, which is a good quantitative marker for the severity of the disease. The pathophysiology is largely unknown, but IADE can be viewed as a common final pathway of arterial wall response or damage in the tunica media due to various mechanisms, such as matrix metalloproteinase dysfunction or muscle cell or elastic fibre injury. No randomised controlled trials have been undertaken in IADE and thus little high-level evidence is available on which to base treatment guidelines. IADE management depends on clinical presentation and disease severity, and includes blood pressure control, antithrombotic treatments, endovascular procedures, and surgery. Further studies are needed to better define IADE in the general population, to establish its prevalence and pathophysiology, to identify subgroups at risk of life-threatening complications, and to offer effective treatment options., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Spontaneous intracranial artery dissection is an uncommon and probably underdiagnosed cause of stroke that is defined by the occurrence of a haematoma in the wall of an intracranial artery. Patients can present with headache, ischaemic stroke, subarachnoid haemorrhage, or symptoms associated with mass effect, mostly on the brainstem. Although intracranial artery dissection is less common than cervical artery dissection in adults of European ethnic origin, intracranial artery dissection is reportedly more common in children and in Asian populations. Risk factors and mechanisms are poorly understood, and diagnosis is challenging because characteristic imaging features can be difficult to detect in view of the small size of intracranial arteries. Therefore, multimodal follow-up imaging is often needed to confirm the diagnosis. Treatment of intracranial artery dissections is empirical in the absence of data from randomised controlled trials. Most patients with subarachnoid haemorrhage undergo surgical or endovascular treatment to prevent rebleeding, whereas patients with intracranial artery dissection and cerebral ischaemia are treated with antithrombotics. Prognosis seems worse in patients with subarachnoid haemorrhage than in those without., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

The aim of this narrative review is to evaluate the pathogenesis, clinical features, diagnosis, treatment and prognosis of intracranial artery dissection (IAD). IAD is a rare and often unrecognized cause of stroke or subarachnoid haemorrhage (SAH), especially in young adults. Two types of IAD can be identified: a subintimal or subadventitial dissection. It is suggested that a subintimal dissection results in luminal stenosis, thromboembolism and subsequently cerebral ischaemia, whilst a subadventitial IAD could result in the formation of a pseudo-aneurysm and compression on brainstem or cranial nerves. Rupture of such a dissecting aneurysm causes SAH. The exact cause of IAD remains unknown but several factors are associated with its development. Diagnosis is based on clinical presentation and specific features seen on multimodal neuroimaging. The management of IAD depends on the clinical presentation. In the case of cerebral ischaemia, anticoagulants or antiplatelet agents are used, whilst in the case of SAH endovascular treatment is primarily advocated. Prognosis depends on clinical presentation. Presentation with SAH has a worse prognosis., (© 2014 The Author(s) European Journal of Neurology © 2014 EAN.)

Clinical features and therapeutic strategies of cervicocranial arterial dissection (CCAD) are still unclear. A retrospective review was conducted on 71 CCAD patients. Diagnosed by DSA and outcome evaluation was through mRS scores follow-up 12 months. All patients were allocated into three groups according to clinical situation: 1) subarachnoid hemorrhage (SAH), 2) ischemic symptoms and 3) mass effect. CCAD with anterior circulation arterial dissection (ACAD) had higher ischemia than that with posterior circulation arterial dissection (PCAD) (p=0.023). The non-aneurysmal dissection (NAD) patients were susceptible to ischemia (p=0.00) and patients with aneurismal dissection (AD) were more susceptible to SAH (p=0.001); The outcome of patients with SAH was significantly worse than patients with other manifestations (p=0.012). Following up one year, the outcome of CCAD involving posterior inferior cerebellar artery (PICA) was significantly worse than the other area (p=0.035). There was no statistically significant difference in mRS scores between endovascular treatment and conservative treatment (p=0.052) at one year follow-up. Patients suffering from SAH that received endovascular treatment experienced improved outcomes than patients with conservative treatment (p=0.033). The patients in the ACAD, NAD and extracranial CCAD groups were more likely to suffer from ischemia, while patients in the AD group were susceptible to SAH. CCAD with SAH or involving PICA had poor prognoses. The therapeutic efficacy of conservative treatment is nearly equal to endovascular treatment in CCAD patients follow up 12 months; however, endovascular treatment may decrease the risk of mortality for the patient with SAH.

Advances in medical devices technology now allow the endovascular treatment of stenotic lesions of the intracranial arteries with the help of the implantation of microstents. The technical development in stent, catheter and guidewire technology now allows the application of these devices in the intracranial arteries, where access issues due to difficult anatomy and length of the access pathway pose far more hurdles to accurately, efficiently and safely navigate these devices as compared with the treatment of lesions of the peripheral arteries or the coronary system. Although the efficacy and safety of these procedures show a continuous improvement with decreased periprocedural complication rates, the development of a significant intraluminal stenotic lesion as a result of neointimal hyperplasia induced within the implants remains a serious delayed complication. In this review, the authors give an overview of the factors affecting the development of neointimal hyperplasia and review the currently available possibilities of the prevention as well as the treatment of the already existing intraimplant stenotic lesions.

The objective is to present the management of patients with aneurysms and other intracranial vascular malformations by interventional neuroradiology (INR) techniques. Interventional neuroradiology is dedicated to the treatment of patients who present with pathologies of the brain, the face, and the spinal cord that are treatable by direct percutaneous or endovascular approaches. Indications of INR have dramatically increased over the last 20 years thanks to innovations in radiological equipments (angio suite, 3D images, etc.) and embolic materials. The endovascular treatment alone is generally curative but sometimes it will be performed before surgery to decrease its risks. Interventional neuroradiology is thus part of several multidisciplinary teams including departments of neurosurgery, neurology, intensive care, anesthesiology, vascular surgery, ENT, etc. We will present the main indication of INR that is the treatment of patients with aneurysms and other intracranial vascular malformations. In conclusion, interventional neuroradiology plays nowadays the main role for the management of patients with neurovascular diseases. Interventional neuroradiology belongs to several multidisciplinary teams and allows to diagnose, to treat, and to follow-up these patients.

We describe the risk factors and treatment recommendations of intracerebral haemorrhage, arterial ischaemic stroke, and cerebral sinovenous thrombosis in childhood. The aetiology in children differ a lot from the aetiology in adults, hence guidelines specifically for children have been written. These guidelines build mainly on expert opinions, since only few evidence-based studies exist. Paediatric stroke is one of the major causes of lifelong disability, and the high costs to the families and the society make further research very important.

Numerous techniques have been described to treat intracranial vessel perforation during endovascular interventions. We describe a novel application of Onyx-18 for the treatment of intracranial catheter perforations by sealing the vessel from the outside while retracting the catheter into the arterial lumen.

Background: Concomitant intracranial and visceral artery aneurysms are a rare occurrence. We report the case of a patient who underwent surgical repair of a ruptured intracranial aneurysm but subsequently experienced a ruptured hepatic artery aneurysm in the immediate postoperative period., Methods: A 59-year-old woman presented with a ruptured posterior communicating artery aneurysm and was treated with coil occlusion. On postoperative day 3, she became hypotensive with a rigid abdomen and was found to have a ruptured hepatic artery aneurysm. Rapid transfusion of packed red blood cells was begun, and the patient was taken to the angiography suite where the hepatic artery aneurysm was identified and successfully occluded using detachable coils to prevent further hemorrhage., Results: Recovery was complicated by an ileus and mild pulmonary edema. Over several weeks, neurological status and third nerve paresis improved, with eventual discharge to a subacute rehabilitation center. Subsequent follow-up is notable for recurrence of the intracranial aneurysm, and multiple strictures of right intrahepatic arteries and hepatic ducts. The patient is currently being evaluated for liver transplant, but continues to improve neurologically., Conclusion: Prior to this report, there have only been 2 documented cases of concomitant intracranial and visceral artery aneurysms. These reports serve to remind the clinician that intracranial aneurysms may be only part of a systemic pathology, which should be taken into account when unexpected complications arise postoperatively.

Object: Noninvasive neuroimaging techniques are increasingly identifying unruptured intracranial arterial dissections (IADs) at examination for headache or ischemic symptoms. Approximately 3% of cases of aneurysmal subarachnoid hemorrhage (SAH) are caused by IADs in Japan, but the natural history of unruptured IADs is not known., Methods: Clinical data obtained in 190 patients with 206 IADs were retrospectively analyzed on the basis of long-time follow-up of geometry and clinical event. The IADs were divided into an unruptured group and SAH group depending on the patient's clinical status at the initial diagnosis. Day 0 was defined as the day preceding diagnosis of IAD-that is, the day of symptom onset. This was retrospectively determined from the clinical history., Results: The 206 IADs included 98 unruptured lesions and 108 SAH. In both groups, the vertebral artery was the most frequent site. In the unruptured group, 93 IADs were followed for a mean of 3.44 years. The mean interval between symptom onset (Day 0) and neuroimaging diagnosis was 9.8 days. Subsequent geometry change was seen in 78 (83.9%) of 93 IADs. Major change was almost completed within 2 months, and complete normalization was seen on neuroimaging in 17 (18.3%) of 93 IADs, with the earliest on Day 15. Rupture of the IAD in the unruptured group occurred in only 1 patient on Day 11. In the SAH group, 84 of the 108 patients complained of preceding headache before onset of SAH. In 81 (96.4%) of the 84 patients, SAH occurred on Day 0-3 with the latest on Day 11. In all patients in the unruptured and SAH groups, the latest day of SAH from the onset of preceding headache was Day 11., Conclusions: Most IADs causing SAH bleed within a few days of occurrence. Most IADs that are unruptured already have little risk for bleeding at diagnosis because of the repair process. Intracranial arterial dissections may be much more common than previously thought, and the majority may occur and heal without symptom manifestation.

To investigate the risk factors and treatment outcomes for ischemic stroke in children, we reviewed the charts of 93 children with ischemic stroke seen at our hospital between 1997 and 2006. Age at stroke, sex, medical history, family history, clinical findings upon admission, history of seizure, and radiological findings were recorded. Mean age at onset of the initial stroke was 56.6+/-46.9 months, ranging from 1 month to 14 years. The male:female ratio was 1.6:1. Cardiac and infectious disease were the most common risk factors (37.7%). There were five children (5.4%) who had recurrent stroke and three (3.2%) who had multiple risk factors. Cardiac and infectious causes appeared to be the most important risk factors for ischemic stroke in children in the Adana region of Turkey., (Crown Copyright 2010. Published by Elsevier Ltd. All rights reserved.)

Patients who have had a stroke or transitory ischemic attack (TIA) attributed to stenosis (50-99%) of a major intracranial artery face a 12-14% risk of subsequent stroke during the 2-year period after the initial ischemic event, despite treatment with antithrombotic medications. Most of this risk accrues during the first year. Some patients are at substantially greater risk, particularly those with a severe (70-99%) stenosis, those who have recently had an ischemic event, and women. Patients may also be at high risk if they had an initial stroke rather than TIA or if they have symptoms precipitated by hemodynamic maneuvers. The annual risk of subsequent stroke may exceed 20% in these high-risk groups.

The prevalence and natural history of asymptomatic intracranial arterial stenosis are not very well characterized. Existing data suggest that incidentally discovered asymptomatic intracranial stenosis presents a fairly low risk of stroke, though substantial uncertainty remains. Patients may be at greater risk if there are tandem stenoses. Methods to stratify the risk of stroke with asymptomatic intracranial atherosclerotic lesions have yet to be established and validated. In general, aggressive intervention for an asymptomatic intracranial stenosis is not currently recommended.

Purpose: To access the safety of low-dose heparin in comparison to a high-dose regimen in patients undergoing intracranial stent-assisted angioplasty., Methods: Sixty-four consecutive patients (53 men; mean age 54 years) undergoing stent-assisted angioplasty of 70 intracranial arterial stenoses were randomized to receive either low-dose (2000-U bolus+500 U/h) or high-dose (3000-U bolus+800 U/h) intravenous heparin during the procedure. The activated clotting time (ACT) was measured. The groups were compared for the following primary endpoints until hospital discharge: target lesion acute thrombosis, intracranial hemorrhage (ICH), and death., Results: The overall angioplasty success rate was 93% (65/70 lesions). Stents were placed in 94.7% (36/38) and 90.6% (29/32) of patients in the low-dose and high-dose groups, respectively (p = 0.65). The primary endpoint occurred in 6% (2/33) of patients in the low-dose group versus 16% (5/31) of patients in the high-dose group (p = 0.25). Two patients, 1 patient in each group, experienced acute target lesion thrombosis during the procedure (p = NS); ICH occurred in 5 patients: 1 in the low-dose group and 4 in high-dose group (3.0% versus 12.9%, p = 0.19)., Conclusion: The use of a low-dose heparin regimen did not increase the incidence of target lesion thrombosis or ICH in this small pilot trial. Intraoperative low-dose heparin seems to be safe for patients undergoing intracranial stent-assisted angioplasty, but these data should be confirmed in a larger trial.

Childhood stroke syndromes are an important cause of mortality and morbidity. This paper focuses on the recent advances in arterial ischaemic stroke beyond the neonatal period. Vascular risk factors are identified in the majority of children and guide both acute and longer term treatments, as well as determining prognosis. Contrary to popular belief many children have residual impairments encompassing a wide range of domains. National and international collaborations are facilitating an increase in the understanding of childhood stroke and have the eventual aim of conducting trials of potential therapeutic interventions.

Objective: Acute ischemic stroke has reached epidemic proportions in the United States, affecting approximately 700,000 people annually. With the recent technological advancements in endovascular devices, clinicians now have tools capable of recanalizing acute intracranial occlusions. The combination of pharmacological thrombolysis and mechanical clot perturbation may result in increased rates of angiographic recanalization, which may lead to improvement in patient outcomes after acute stroke., Methods: In this article, the various intra-arterial pharmacological and mechanical therapies used by interventionists to treat acute stroke are described. Strategies for using combinations of these therapies are discussed, as are preliminary radiographic and clinical outcomes. Techniques for complex mechanical stroke interventions are discussed in detail., Results: Several advances in endovascular stroke technologies are becoming increasingly available., Conclusion: With proper patient selection, these therapies may lead to increased recanalization rates and better patient outcomes.

Introduction: Independent predictors of outcome for ischemic stroke include age and initial stroke severity. Intracranial large-vessel occlusion would be expected to predict poor outcome. Because large-vessel occlusion and stroke severity are likely correlated, it is unclear if largevessel occlusion independently predicts outcome or is simply a marker for stroke severity., Methods: A consecutive series of patients with suspected stroke or transient ischemic attack were imaged acutely with computed tomography angiography (CTA). CTAs were reviewed for intracranial large-vessel occlusion as the cause of the stroke. Baseline National Institutes of Health Stroke Scale (NIHSS) score, discharge modified Rankin score, and patient demographics were abstracted from hospital records. Poor neurological outcome was defined as modified Rankin score exceeding 2., Results: Seventy-two consecutive patients with acute ischemic stroke were imaged with CTA. The median (range) time from stroke symptom onset to CT imaging was 183 minutes (25 minutes to 4 days). Median NIHSS score was 6 (1-32) and intracranial large-vessel occlusion was found in 28 (38.9%) patients. Fifty-six percent of patients had a good neurological outcome. In multivariate logistic regression analysis, two variables predicted poor neurological outcome: baseline NIHSS score (OR 1.21, 95% CI [1.07-1.37]) and presence of intracranial large-vessel occlusion (OR 4.48, 95% CI [1.19-16.9]). The predictive value of large-vessel occlusion on outcome was similar to an 8-point increase in NIHSS score., Conclusion: In patients presenting with acute brain ischemia, intracranial large-vessel occlusion independently predicts poor neurological outcome at hospital discharge, as does the presence of a high NIHSS score. Performing routine intracranial vascular imaging on acute stroke patients may allow for more accurate determination of prognosis and may also guide therapy.

Our aim was to determine whether CT angiography is suitable for the evaluation of in-stent restenoses in small vessel stents for intracranial angioplasty. Therefore, we simulated stenoses with degrees of 25, 50, 75 and 90% in a total of 12 stents with different designs (MEDTRONIC AVE; ABBOT BioDivYsio, GUIDANT Neurolink, TERUMO Tsunami, COOK V-Flex Plus) and sizes (3.0 mm, 4.0 mm). For each stenosis, the apparent stenotic degree (ASD) was measured by CT angiography. Subjective (viewing at the CT images) and objective (acquisition of a density profile) evaluations were made after the stents were filled with a solution of 0.9% NaCl and with a diluted contrast medium. It was not possible to visualize the patent lumen in any of the stenotic stent segments by viewing at the CT images. After objective evaluation, the degree of the stenoses was generally overestimated. In the group with the 3.0-mm stents, ASD ranged from 73.6 to 100% in 25% degree stenoses. With the exception of one stent, stenoses with a degree of more than 25% appeared as vessel obstruction (ASD = 100%) in the 3.0-mm group. In the 4.0-mm group, the mean ASD was 60% for 25% degree stenoses, 76% for 50% degree stenoses, 91% for 75% degree stenoses and 96% for 95% degree stenoses. The minimum diameter of stents for differentiation between in-stent restenosis and vessel occlusion using CT angiography is 4.0 mm. In CT angiography, the degrees of in-stent stenoses are generally overestimated. The evaluation of in-stent restenoses only seems to be possible when CT angiographic images before and after contrast application are evaluated objectively by density profiles.

Background: Superior cerebellar artery aneurysms are rare. We present a clinical series of twelve of these aneurysms that were treated exclusively with endovascular coils., Method: A retrospective analysis of a prospectively collected database of cerebral aneurysms treated with coil embolization was performed. Clinical notes and radiological images were reviewed., Results: Twelve superior cerebellar artery aneurysms were treated in eleven patients between 1992 and 2001. Seven patients presented with subarachnoid hemorrhage, two with neurologic deficit, and two had asymptomatic aneurysms. Coiling resulted in complete aneurysm obliteration in six patients and incomplete obliteration in the other six. No subsequent hemorrhage occurred with follow-up between 6 and 119 months (mean follow-up 50 months). Procedural morbidity was one superior cerebellar artery infarct with good recovery. Management morbidity was one middle cerebral artery embolus during a follow-up angiogram that required thrombolysis with a good clinical result. Nine out of 11 patients on follow-up were performing at Glasgow Outcome Scale (GOS) 5. One patient with GOS 3 presented with a poor grade subarachnoid hemorrhage and the other patient with GOS 4 presented with a parenchymal hemorrhage due to an arteriovenous malformation., Conclusion: Endovascular treatment of superior cerebellar artery aneurysms is an effective treatment strategy with low morbidity.