Posterior Circulation Involvement Research Articles

Abstract TP173: Neuroimaging Biomarkers Of Moyamoya Arteriopathy By Age In A Surgical Cohort

Introduction: Moyamoya arteriopathy (MMA) is a significant risk factor for pediatric stroke. Radiographic signs thought to indicate disease severity guide clinical decision making. We hypothesized that there exists a correlation between age and imaging biomarkers. Methods: We retrospectively identified pediatric MMA patients seen at our center that underwent surgical revascularization. Preoperative MRI/MRA imaging was reviewed by two pediatric neuroradiologists who were blinded to clinical characteristics. The following neuroimaging markers were evaluated by consensus: Suzuki staging, cerebrovascular stenosis score (CVSS), the presence of posterior circulation steno-occlusive lesions, ivy sign, and brush sign. Associations of these features with age were analyzed using simple logistic regression and Spearman correlation analyses. Results: We identified 27 patients undergoing 30 individual revascularization procedures between January 2003 and June 2021. There were 29 images of diagnostic quality for analysis. Median age at imaging was 8.5 years. Younger age was associated with posterior arterial lesions (p=0.028). There was no statistically significant association between age and ivy sign (p=0.205), brush sign (p=0.156), Suzuki stage (p=0.671), or CVSS (0.234). Conclusions: In this cohort of surgical MMA patients, younger children were more likely to have posterior circulation involvement on MRA. While not statistically significant, all analyzed radiographic signs were more prevalent among younger patients. Further studies correlating neuroimaging biomarkers with clinical outcomes in larger cohorts including non-surgical MMA patients are needed.

Clinical and functional characteristics of women after stroke, fallers and non-fallers: a longitudinal study

BACKGROUND: Occurrence of falls is among the most frequent complications presented by the elderly and individuals with neurological diseases. It is known that in the elderly, there is a greater frequency of falls in women. However, in individuals after stroke, this relationship is not established. The purpose of this study was to differentiate the clinical and functional characteristics of fallers and non-fallers women after stroke and to verify the association between functional mobility and functional capacity in these women. DESIGN AND SETTING: This is a longitudinal prospective study conducted in Salvador, Bahia, Brazil. METHODS: Women with independent gait assisted in a reference outpatient clinic were included. Sociodemographic and clinical data were collected, and the National Institutes of Health Stroke Scale, Modified Barthel Index, Timed Up and Go (TUG), and European quality of life were applied. The level of significance was set at 5%. RESULTS: A total of 68 women with a mean age of 56 (± 13.7) years were selected; 51% of the women fell in the follow-up, the latter was younger than 55 years (± 13.4), with greater severity of the stroke. Impairment in the posterior vascular territory was more evident among falling women (P = 0.009), but all falling and non-falling women presented impaired functional mobility (TUG = 15seconds). However, the total sample was classified as totally independent or with slight dependence on the activities of daily living (ADLs) (MBI = 49). The correlation between TUG time and MBI was negative and significant (R = -0.702, P ? 0.001). CONCLUSIONS: The involvement of posterior circulation is an important predictor of falls, an important indicator in the monitoring of women after stroke. It also emphasizes that the lower functional mobility impacted women's functional capacity.

Preoperative clinical symptomatology and stroke burden in pediatric moyamoya angiopathy: Defining associated risk variables.

To evaluate the preoperative clinical and magnetic resonance imaging and angiography (MRI-MRA) characteristics in pediatric moyamoya patients. Analysis included 100 children with moyamoya angiopathy referred to our moyamoya center for preoperative evaluation. Clinical symptoms, neurological status using Pediatric Stroke Outcome Measurement (PSOM) and degree of disability on modified Rankin scale score (mRS) were evaluated. MRI-MRA evaluation included the assessment of ischemic lesions and involvement of posterior circulation. Data were analyzed for moyamoya disease (MMD), moyamoya syndrome (MMS) and age at disease onset. Stroke was a common presentation in both MMD and MMS patients. TIAs and headaches/migraine were more frequent in MMD. There was no evidence of a difference in stroke burden on MRI as well as in PCA involvement between the two subgroups. Children <2 years had higher odds of having a stroke (OR 15.5, 95% CI 3.8-62.4, p<0.001), recurrent stroke (OR 11.8, 95%CI 2.9-46.7, p<0.001) and unfavorable mRS (≥2) (OR 4.2, 95% CI 1.3-13.7, p=0.01) when compared to those >5 years of age. There was some evidence of association of PCA involvement with recurrent strokes (OR 3.1, CI 1.0-9.6, p=0.05), a poor PSOM (OR 3.0, 95% CI 1.1-8.2, p=0.04) and mRS (OR 3.1, 95% CI 1.2-8.3, p=0.02). Stroke seems to be a common presentation in both MMD and MMS patients. Early age at symptom onset and involvement of posterior circulation seem to be important risk factors for a high stroke burden and an unfavorable PSOM and mRS.

Expanding the clinical and neuroimaging features of post-varicella arteriopathy of childhood.

Post-varicella arterial ischemic stroke (AIS) is considered an uncommon cause of pediatric stroke that is considered a self-limiting, monophasic disease. However, in a subset of patients, disease recurs; the prevalence of vasculopathy or AIS recurrence, severity of clinical outcomes, and standardized therapies have not been well characterized. Herein, we determined the clinical-neuroradiological features, long-term evolution, and relationship between acute phase treatment and vasculopathy recurrence in a pediatric population with post-varicella AIS. Clinical, laboratory, and neuroradiological features of 22 children with post-varicella AIS between 2010 and 2019 (16 males, mean age at stroke 4 years, range 1.7-10) were reviewed. Statistical analyses were performed using χ2 and Fisher exact tests. Of the 22 cases, mean time from varicella to stroke was 4.5 months with 3 cases presenting more than 12 months after rash; 21 (95%) were not vaccinated for varicella; 3 (13.6%) had posterior circulation involvement; and 5 (22.7%) had AIS or vasculopathy recurrence, of which 4 recurred 6.1 months to 2.8 years after initial clinical onset. Recurrence was associated with lack of antiviral treatment during the first episode (p = 0.02). Post-varicella AIS can occur months after rash making diagnosis challenging. Because recurrent vasculopathy was seen predominantly in cases not treated with antiviral therapy during initial presentation, it is important to rapidly diagnose post-varicella AIS through clinical criteria and/or virological testing then treat with antivirals to prevent recurrence.

Abstract P565: Influence of Thyroid Disease on Presentation and Outcomes of Moyamoya Disease

Introduction: The presence of thyroid autoantibodies may play a role in the pathogenesis of moyamoya-type angiopathy. However, the influence of thyroid disease on presenting characteristics and treatment outcomes of moyamoya disease (MMD) remains unclear. Methods: Consecutive patients with MMD prospectively treated at our institution from 2015-2018 were reviewed. Results: 264 MMD patients with MMD were identified, 33 (12.5%) of whom presented with concurrent thyroid disease (4 hyperthyroid, 28 hypothyroid, 1 unspecified). Comparing groups with and without thyroid disease, there were no differences in age of presentation (p=0.12), racial composition (p=0.38), or rates of diabetes (p=0.32), hypertension (p=1.0), and hyperlipidemia (p=0.71). There was a higher proportion of females (94% F vs. 74% F, p=0.02) and lower rates of smoking (p=0.04) and alcohol consumption (p=0.04) in the thyroid disease group. Despite lower rates of vascular risk factors, the thyroid disease group exhibited more severe angiographic MMD at presentation, with higher Suzuki grade (p=0.002), higher proportion of bilateral disease (91% vs. 67%, p=0.007), and a trend towards more posterior circulation involvement (39% vs. 24%, p=0.09). MMD patients with concurrent thyroid disease were also more likely to present with carotid occlusion as opposed to large vessel stenosis (39% vs 10%, p=0.03), resulting in increased collateral filling via the extracranial (3.5% vs. 2.8%, p=0.04) and posterior circulations (24% vs. 16%, p=0.002). Patients with thyroid disease trended towards having higher rates of ischemic complications (28% vs. 16.8%, p=0.06) perioperatively; however, there were no differences in rates of post-operative hemorrhage (p=1.0) or transient neurologic events (p=0.85). Conclusions: Patients with concurrent moyamoya and thyroid disease present with more advanced angiographic intracranial disease, which may be associated with higher rates of ischemic complications in the perioperative period.

Abstract MP47: Clinicoradiological Outcomes of Surgical Revascularization for Moyamoya Disease With Minimum 10-Year Follow-Up

Introduction: The long-term progression of disease and recurrence rate of symptomatic events after surgical revascularization for moyamoya disease (MMD) are unknown. Method: Patients with MMD operated on between 1991 and 2009 were prospectively enrolled into the departmental database. Results: 230 revascularization (87% combined direct and indirect and 13% indirect alone) procedures were performed on 129 patients for the treatment of MMD with a long-term bypass patency rate of 98%. Unilateral presentation was identified in 39 (30.2%) compared to 90 (69.8%) with bilateral MMD. Posterior circulation (PC) involvement was identified in 34 (26.4%) patients. Over a median follow-up period of 13 years (range 10-26 years), 15 (38.5%) patients experienced radiographic progression of unilateral disease and 14 (10.9%) experienced PC radiographic progression. Delayed surgical revascularization of the untreated contralateral anterior circulation was performed on 11 (28.2%) patients due to symptomatic progression of disease. Progression of PC disease occurred only in patients with PC involvement upon presentation. Aside from those with unilateral progression, fourteen additional patients (10.9%) experienced delayed cerebrovascular accidents (CVAs), 8 of which were ischemic and 6 were hemorrhagic strokes. Twelve delayed CVAs occurred in a previously revascularized hemisphere while one occurred in an un-diseased hemisphere from a micro pseudoaneurysm. Repeat revascularization was performed in 6 patients while 4 were managed conservatively and 4 were deceased. Relative to baseline presenting mRS, 92.2% of patients were improved or unchanged at 10 year follow-up. On multivariate regression, progression of the PC predicted the development of delayed CVA (Odds ratio 8.1, 95% CI 1.1-59.2) while pre-operative stroke (Odds ratio 3.4, 95% CI 1.2-9.8) presentation and progression of disease was predictive of 10-year outcome (Odds ratio 4.0, 95% CI 1.5-10.6). Conclusion: Good functional outcomes over a median follow-up period of 13 years support the strategy of revascularization for MMD patients. Disease progression of the posterior circulation is common and may reduce pial-pial collaterals resulting in delayed CVAs.

Abstract MP9: Incidence and Outcomes of Posterior Circulation Involvement in Moyamoya Disease

Moyamoya disease (MMD) is a progressive, occlusive disease of the internal carotid arteries and their proximal branches, with the subsequent development of an abnormal vascular network of small, weak blood vessels that are prone to rupture. Steno-occlusive changes in the posterior cerebral arteries (PCA) may contribute to worse outcomes in MMD patients, however, there is a paucity of information on the incidence and natural history of MMD patients with PCA disease. We retrospectively reviewed the charts of patients with MMD treated between 1987 and 2019. Demographics, peri-operative outcomes, and radiological phenotypes were recorded for 450 patients. PCA disease was scored as either 0 (no disease), 1 (mild), 2 (moderate), or 3 (severe, or occluded). Out of 450 patients, 164 (34.4%) had concurrent PCA disease. In patients with PCA disease, the extent of occlussion was mild in 56 (34%), moderate in 41 (25%), and severe in 67 (40.9%) patients. In total, there were 319 females (70.9%), with a higher proportion of female MMD patients having severe or moderate PCA disease (p=0.038). Patients with severe and moderate PCA disease had higher Suzuki grades (p&lt;0.0001), more extensive collateral angiopathy (p&lt;0.0001), more frequent cerebrovascular accidents on presentation (p=0.012), higher hemodynamic scores (p=0.022), and a greater frequency of post-operative ischemic complications (p=0.019). Peak modified Rankin Scale (mRS) scores were higher in patients with any severity of PCA disease (p=0.0006). Over a mean follow-up of 6.94±4.16 years, 28 patients (8.86%) developed new or progressive PCA disease. Bilateral disease was present in 66 patients (14.7%) and was associated with higher Suzuki grades (p&lt;0.0001), more extensive collateral angiopathy (p&lt;0.0001), and greater post-operative ischemic complications (p=0.011). Peak mRS scores were higher in patients with bilateral disease compared to patients with no PCA disease (p&lt;0.0001). PCA disease involvement is associated with higher rates of ischemic peri-operative complications and poor functional outcomes likely due to reduced collateral flow. Progression of PCA disease is not uncommon. Future studies are needed to assess the impact of PCA disease progression on long-term outcomes.

Incidence and Progression of Posterior Circulation Involvement in Moyamoya Disease

Online date: November 16, 2020 This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)

Moyamoya: An Update and Review.

This article is a clinical review of Moyamoya disease (MMD) and Moyamoya syndrome (MMS). We review the incidence, epidemiology, pathology, historical context, clinical and radiographic findings, diagnostic imaging modalities, radiographic grading systems, the effectiveness of medical, interventional, and surgical treatment, and some of the nuances of surgical treatment options. This article will help pediatricians, neurologists, neurosurgeons, and other clinical practitioners who are involved in caring for patients with this rare clinical entity.MMD is an intrinsic primary disease process that causes bilateral progressive stenosis of the anterior intracranial circulation with the involvement of the proximal portions of the intracranial internal carotid artery (ICA) extending to involve the proximal portions of the anterior cerebral artery (ACA) and middle cerebral artery (MCA); posterior circulation involvement is very rare. This causes a compensatory response where large numbers of smaller vessels such as the lenticulostriate arteries begin to enlarge and proliferate, which gives the angiographic appearance of a "Puff of Smoke", which is translated into Japanese as "Moyamoya”. MMS is a secondary process that occurs in response to another underlying pathological process that causes stenosis of intracranial blood vessels, such as radiation. For example, an external source of radiation causes stenosis of the ICA with a compensatory response of smaller blood vessels, which then enlarge and proliferate in response and has the same "Puff of Smoke" appearance on the diagnostic cerebral angiogram (DCA).Histological findings include an irregular internal elastic lamina with luminal narrowing, hyperplasia of the tunica media, and intimal thickening with vacuolar degeneration in smooth muscle cells in the tunica media. Compensation for diminishing blood supply occurs through angiogenesis, which causes the proliferation and enlargement of smaller collateral blood vessels to increase blood supply to under-perfused areas of the brain.MMD is rare in the United States, with just 0.086 newly diagnosed cases per 100,000 individuals per year, which is approximately one per million new cases annually. Risk factors for MMD include Eastern Asian ancestry and predisposing conditions such as neurofibromatosis and Down's syndrome.Clinically, patients often present with stroke signs and symptoms from cerebral ischemia. The proliferation of collateral blood vessels within the basal ganglia can produce movement disorders. Catheter-based DCA is the current gold standard for obtaining a diagnosis. CT perfusion allows preoperative identification of ischemic vascular territories, which may be amenable to surgical intervention. MRI enables rapid detection of acute ischemic stroke using diffusion-weighted Imaging (DWI) and apparent diffusion coefficient (ADC) sequences to assess for any diffusion restriction. Non-contrast CT of the head is used to rule out acute hemorrhage in the presentation of a progressive neurological deficit.The treatment option for Moyamoya is generally surgical; medical treatment has failed to halt disease progression and neuro-interventional techniques such as attempted stenting of stenosed vessels have failed. Surgical options include direct and indirect cerebrovascular bypass.

Pressing Issues in COVID-19: Probable Cause to Seize SARS-CoV-2 for Its Preferential Involvement of Posterior Circulation Manifesting as Severe Posterior Reversible Encephalopathy Syndrome and Posterior Strokes.

Since December 2019, a novel Severe Acute Respiratory Syndrome coronavirus 2 from China has rapidly spread worldwide. Although respiratory involvement is the mainstay of coronavirus disease 2019 (COVID-19), systemic involvement has recently drawn more attention. In particular, a number of recent articles have shed light on the nervous system as one of the possible targets. At our institution, we observed 15 patients with acute brain vascular manifestations; most interesting, we had a higher prevalence of the posterior circulation acute impairment. In our series, 7 patients had acute posterior cerebral injury: 1, hemorrhagic posterior reversible encephalopathy syndrome; 5, posterior circulation ischemic stroke; and 1, parieto-occipital hemorrhagic stroke. On the basis of our evidence and previous basic science reports, we believe a common etiopathogenetic thread may connect ischemic/hemorrhagic events of the posterior circulation and posterior reversible encephalopathy syndrome in the setting of COVID-19.

Involvement of the intracranial circulation in giant cell arteritis

ObjectiveGiant cell arteritis (GCA) is the most common primary vasculitis affecting the elderly population. GCA preferentially involves the extracranial branches of the carotid artery; intracranial vasculitis is thought to be a rare occurrence. This study determined the prevalence of intracranial vasculitis in a large series of patients evaluated for GCA and describes the clinical presentation of such cases. DesignRetrospective chart review using a prospective database. When possible, subjects underwent high-resolution 3T contrast-enhanced magnetic resonance imaging (MRI) and MR angiography (MRA) of the scalp and intracranial arteries. ParticipantsPatients presenting with suspected GCA between January 2015 and December 2018. Four additional, non-database cases of GCA with intracranial involvement are also described. ResultsOf 197 patients, 168 had a contrast-enhanced MRI of the head and 51 had imaging findings suggestive of vasculitis. Five patients showed probable or definitive involvement of both the anterior and posterior intracranial circulation with isolated posterior intracranial circulation involvement in one additional patient. One of these patients showed evidence of acute posterior circulation ischemia and presented with vertigo but no evidence of ischemic optic neuropathy or ophthalmic artery enhancement. Of the 51 patients, 14 had abnormal enhancement of the ophthalmic arteries, including 1 with arteritic ischemic anterior optic neuropathy and vertebral arteritis and 1 patient with involvement of the internal carotid and posterior cerebral arteries but no reported vision changes. ConclusionAlthough uncommon, clinicians should be aware that GCA can directly involve the intracranial circulation with both the anterior and posterior circulation affected in most of our cases.

Postoperative hemorrhage during the acute phase after direct or combined revascularization for moyamoya disease: risk factors, prognosis, and literature review.

Postoperative hemorrhage during the acute phase is rarely observed after revascularization surgery for moyamoya disease (MMD) but can have severe complications. Its risk factors and outcomes are still unclear. The aim of this study was to investigate the predictors of postoperative hemorrhage during the acute phase in MMD and examine the outcomes of the hemorrhage. The authors reviewed the preoperative clinical characteristics and radiographic features of 465 consecutive MMD cases (518 procedures) that had undergone direct or combined bypass surgery at their institution between 2009 and 2015. Patients with postoperative intracerebral hemorrhage (ICH) or ICH plus intraventricular hemorrhage (IVH) during the acute phase were screened, and then the incidence, location, and risk factors of hemorrhage in these patients were analyzed. Short-term and long-term outcomes (modified Rankin Scale scores) for these patients were also collected. Outcomes were compared between patients with and those without postoperative ICH using propensity score analysis to reduce the between-group differences in baseline characteristics. Postoperative hemorrhage occurred in 11 (2.1%; ICH = 9, IVH = 2) of 518 procedures (mean patient age 39.82 ± 8.8 years). Hemorrhage occurred in the first 24 hours after the operation in 8 cases (72.7%). In the ICH group, most of the hemorrhage sites (77.8%) were located beneath the anastomosed area, and the mean hematoma volume was 16.98 ± 22.45 ml (range 3-57 ml). One case from the ICH group required hematoma evacuation. Among the adult patients (463 procedures [89.4%]), preoperative hypertension (p = 0.008), CT perfusion (CTP) stage > III (p = 0.013), and posterior circulation involvement (p = 0.022) were significantly associated with postoperative ICH. No significant differences between the postoperative ICH group and the no-hemorrhage group were detected in terms of postoperative neurofunctional status at discharge (p = 0.569) or at the last follow-up (p = 1.000). Neither was there a significant difference in future stroke risk (p = 0.538) between these two groups. Preoperative hypertension, CTP stage > III, and posterior circulation involvement are independent risk factors for postoperative ICH after direct or combined revascularization for MMD. After appropriate perioperative management, postoperative ICH has no significant correlations with the postoperative short-term and long-term neurofunctional status.

Diffusion-Weighted Imaging Patterns According to the Right-to-Left Shunt Amount in Cryptogenic Stroke

Background: Although patent foramen ovale (PFO) is considered to be the main cause of cryptogenic stroke, it is difficult to define “true” PFO-related stroke. Objective: In this study, we evaluated comprehensive diffusion-weighted imaging (DWI) findings in patients with cryptogenic stroke according to the right-to-left shunt (RLS) amounts on transcranial Doppler (TCD) sonography. Methods: We assessed consecutive patients with cryptogenic stroke between October 2010 and 2018. The RLS amount on TCD was assessed according to the International Consensus Criteria (ICC). Massive RLS was defined as the highest category of ICC (Curtain pattern). We assessed DWI findings, including the location of lesions, involved vascular territory, and DWI lesion patterns. Results: A total of 100 consecutive patients with cryptogenic stroke were assessed, and PFO was found in 59 patients. In multivariable analyses, massive RLS was noted to be positively associated with the presence of cortical lesion (adjusted OR [aOR] 15.75, 95% CI 1.94–127.71, p = 0.010), multiple territory involvement (aOR 5.24, 95% CI 1.57–17.53, p = 0.007), and number of DWI lesions (beta 0.713, 95% CI 0.245 to 1.181, p = 0.003) after adjusting for confounders. Conversely, massive RLS showed inverse correlations with posterior circulation involvement (aOR 0.22, 95% CI 0.06–0.87, p= 0.031) and number of large DWI lesions (beta –0.328, 95% CI –0.629 to –0.026, p = 0.034). Conclusions: We demonstrated that massive RLS on TCD was associated with multiple, small-scattered cortical lesion in patients with cryptogenic stroke. These DWI pattern is highly suggestive of PFO-related stroke.

Acute Ischemic Stroke: A Review of Imaging, Patient Selection, and Management in the Endovascular Era. Part I: Initial Management and Imaging

AbstractTill recently, the mainstay of management of acute ischemic stroke (AIS) has been intravenous thrombolysis. However, response to treatment and outcomes in the presence of a large vessel occlusion (LVO) were largely suboptimal. Endovascular thrombectomy techniques with stentrievers and aspiration catheters have revolutionized stroke treatment significantly, improving outcomes in this once untreatable disease. The interventional radiologist must play an active role in the stroke team in streamlining imaging as well as endovascular management. The focus of this review article is on initial management and imaging. Initial measures consist of patient resuscitation, basic investigations and assessment of stroke severity using the National Institutes of Health Stroke Scale (NIHSS), all of which have therapeutic and prognostic implications to be considered by the neurointerventionist. Imaging must aim to be swift and efficient. Choice of a modality must be based on available infrastructure as well as clinical-radiologic factors such as the time since ictus or posterior circulation involvement. Computed tomography (CT) is the preferred modality for its speed, whereas magnetic resonance imaging (MRI) remains the gold standard problem solving technique for detection of stroke. Exclusion of hemorrhagic stroke and other stroke mimics is the first objective. Thereafter, imaging is targeted toward assessing the parenchyma and vasculature. Defining the core and penumbra is the most important goal of parenchymal imaging. The core may be defined by the presence of early ischemic changes on CT, CT angiographic source images, or diffusion restriction on MRI. The penumbra is approximated by collateral status or perfusion methods. The prime directive of vascular imaging, either CT or magnetic resonance angiography (MRA) is to establish the presence of an LVO. Once confirmed, the decision for thrombolysis and/or thrombectomy is based on clinical and imaging criteria, the most ideal being that of a moderately severe stroke with a small core and LVO on imaging.

Posterior circulation involvement and collateral flow pattern in moyamoya disease with the RNF213 polymorphism.

Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive stenosis of the circle of Willis with a compensatory collateral vessel network. Recent studies have identified the ring finger protein 213 gene (RNF213) as the unique susceptibility gene for moyamoya disease. The purpose of this study was to compare clinical features of moyamoya disease, especially angiographic findings, between patients with and without the RNF213 mutation. Blood samples from 35 patients with moyamoya disease were obtained between May 2016 and May 2017. Information on age at the time of diagnosis, sex, and initial symptom were obtained via retrospective chart review. Angiographic records were evaluated. RNF213 variants were detected in the 28 of 35 patients (80%), including all pediatric patients (100%) and 18 of 25 adult patients (72%) in our cohort. Leptomeningeal collateral flow from posterior to anterior circulation was more frequent in the RNF213-negative group than in the RNF213-positive group (100% versus 38.9%; p = 0.020). Posterior cerebral arterial territorial involvement was more frequently observed in RNF213-positive patients than in RNF213-negative patients (50% versus 0%; p = 0.027). RNF213 may play a significant role in the development of collateral anastomoses.

Blood Pressure Elevation and Risk of Moyamoya Syndrome in Patients With Trisomy 21.

Individuals with Down syndrome (DS) are at risk for the development of moyamoya syndrome (MMS); MMS is often recognized only after a resulting stroke has occurred. Our goal with this study was to determine if elevations in blood pressure (BP) precede acute presentation of MMS in individuals with DS. A single-center, retrospective case-control study was performed. Thirty patients with MMS and DS and 116 patients with DS only were identified retrospectively. Three BP recordings were evaluated at set intervals (18-24 months, 12-18 months, and 6-12 months before diagnosis of MMS). These were then compared against control averages from patients with DS only. To assess changes over the time, we used general linear model repeated measures analysis of variance. To identify independent predictors of MMS and DS, we used a multivariable analysis using generalized estimating equations accounting for repeated measures of BP. BP in patients with MMS and DS rose significantly over the 24-month period preceding presentation (34th, 42nd, and 70th percentiles at the 18-24-month, 12-18-month, and 6-12-month periods, respectively). BPs in the patients with both MMS and DS were significantly higher than in the DS-only controls in the 6 to 12 (P < .001) and 12 to 18 months before presentation (P = .016). Higher Suzuki scores, bilateral disease, and posterior circulation involvement were also predictive of BP elevation before presentation. Elevations in BP may foreshadow presentation of MMS in individuals with DS. This simple, low-cost screening measure may lead to early identification of at-risk patients in the medical home and prevent irreversible neurologic injury.

Clinical features, course, and outcomes of a UK cohort of pediatric moyamoya.

To describe characteristics and course of a large UK cohort of children with moyamoya from multiple centers and examine prognostic predictors. Retrospective review of case notes/radiology, with use of logistic regression to explore predictors of outcome. Eighty-eight children (median presentation age 5.1 years) were included. Thirty-six presented with arterial ischemic stroke (AIS) and 29 with TIA. Eighty had bilateral and 8 unilateral carotid circulation disease; 29 patients had posterior circulation involvement. Acute infarction was present in 36/176 hemispheres and chronic infarction in 86/176 hemispheres at the index presentation. Sixty-two of 82 with symptomatic presentation had at least one clinical recurrence. Fifty-five patients were treated surgically, with 37 experiencing fewer recurrences after surgery. Outcome was categorized as good using the Recovery and Recurrence Questionnaire in 39/85 patients. On multivariable analysis, presentation with TIA (odds ratio [OR] 0.09, 95% confidence interval [CI] 0.02-0.35), headache (OR 0.10, 95% CI 0.02-0.58), or no symptoms (OR 0.08, 95% CI 0.01-0.68) was less likely to predict poor outcome than AIS presentation. Posterior circulation involvement predicted poor outcome (OR 4.22, 95% CI 1.23-15.53). Surgical revascularization was not a significant predictor of outcome. Moyamoya is associated with multiple recurrences, progressive arteriopathy, and poor outcome in half of patients, especially with AIS presentation and posterior circulation involvement. Recurrent AIS is rare after surgery. Surgery was not a determinant of overall outcome, likely reflecting surgical case selection and presentation clinical status.

Posterior circulation involvement in pediatric and adult patients with moyamoya disease: a single center experience in 574 patients.

The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries. Clinical and radiographic features were compared between those with and without PCA involvement, also between pediatric and adult patients. A total of 574 angiograms (140 pediatrics and 434 adults) were reviewed. The prevalence of PCA steno-occlusion did not differ significantly between pediatric patients and adult patients (35.0% vs. 30.4%, P=0.347). Pediatric patients had more advanced PCA stages compared to adult patients (P=0.045). There was a significant correlation of the PCA angiographic stages with the ipsilateral internal carotid artery (ICA) stages, both in pediatrics and in adults (both P<0.001). The frequency of ipsilateral cerebral infarction positively correlated with the advancement of PCA stages in adult patients (P<0.001), but not significant in pediatric patients (P=0.106). Pediatric patients tend to have more advanced PCA lesions than adult patients. The degree of PCA steno-occlusion positively correlates with the ipsilateral ICA stage, both in pediatrics and in adults.

Headache in stroke according to National Acute Stroke Israeli Survey.

Analysis of headache occurrence in a national cohort of patients with acute stroke. The data were based on the triennial 2-month period of the National Acute Stroke Israeli (NASIS) Registry. A total of 2166 patients with acute stroke were retrieved: 2001 (92.4%) had ischemic stroke (IS), 150 (6.9%) had intracerebral hemorrhage (ICH), 4 (0.2%) had sinus vein thrombosis (SVT), where as in 11 (0.5) the stroke type was undetermined. Two hundred and six (9.5%) patients reported headache: in SVT headache was reported by 50% of patients, in ICH by 21.3% while 8.4% of patients with IS (28% with transient ischemic attack [TIA]) complained about headache. In 18.1% of patients with headache, the stroke type was undetermined. Female gender, younger age, previous headache, cerebral hemorrhage, and dizziness/unsteadiness predisposed for headache. The incidence of headache was higher in patients with ICH than in patients with IS regardless of the history of previous headaches. In IS, headache decreased with stroke severity and was more common in the posterior than in the anterior circulation. Patients with lacunar stroke suffered less frequently from headache than patients with non-lacunar stroke. Significantly more patients with carotid dissection presented with headache than without. Intracerebral hemorrhage, younger age, female gender, posterior circulation involvement, and headache history are predictors for headache occurrence in acute stroke. Headache incidence in ICH correlates with stroke severity as opposed to IS. Headache in TIA is not unusual. Lacunar strokes are generally not accompanied by headaches. Headache remains the main complaint in SVT and carotid dissection.

Posterior Reversible Encephalopathy Syndrome: Clinical Differences in Patients with Exclusive Involvement of Posterior Circulation Compared to Anterior or Global Involvement

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic syndrome not yet fully understood and characterized by transient neurologic symptoms in addition to typical radiological findings. There are only a few articles that describe the clinical differences between patients with PRES that involve carotid and vertebrobasilar circulations. Our study aims to further evaluate the differences between predominantly anterior and posterior circulation PRES. We review 54 patients who had received the diagnosis of PRES from 2009 to 2015. The patients were divided into 2 groups: (1) exclusively in posterior zones; and (2) anterior plus posterior zones or exclusively anterior zones. Several clinical characteristics were evaluated, including the following: age, sex, previous diseases, the neurologic manifestations, the highest blood pressure in the first 48 hours of presentation, highest creatinine level during symptoms, and the neuroimaging alterations in brain magnetic resonance imaging. Mean age at diagnosis was 28.5 years old (9 men and 45 women) and mean systolic blood pressure among patients with lesions only in posterior zones was 162.1 mmHg compared to 179.2 mmHg in the anterior circulation. The most common symptoms in the 2 groups were headache and visual disturbances. PRES may have several radiological features. A higher blood pressure seems to be 1 of the factors responsible for developing widespread PRES, with involvement of carotid vascular territory. This clinical-radiological difference probably occurs because of the larger number of autonomic receptors in the carotid artery in comparison to the vertebral-basilar system.