Posterior Circulation Involvement Research Articles

Development and validation of a novel nomogram for predicting long-term rebleeding risk among patients with hemorrhagic moyamoya disease: a 10-year multicenter retrospective cohort study.

The aim of this study was to develop and validate a predictive nomogram model for long-term rebleeding events in patients with hemorrhagic moyamoya disease (HMMD). In total, 554 patients with HMMD from the Fifth Medical Center of the Chinese PLA General Hospital (5-PLAGH cohort) were included and randomly divided into training (390 patients) and internal validation (164 patients) sets. An independent cohort from the First Medical Center and Eighth Medical Center of Chinese PLA General Hospital (the 1-PLAGH and 8-PLAGH cohort) was used for external validation (133 patients). Univariate Cox regression analysis and least absolute shrinkage and selection operator (LASSO) regression algorithm were used to identify significant factors associated with rebleeding, which were used to develop a nomogram for predicting 5- and 10-year rebleeding. Intraventricular hemorrhage was the most common type of cerebral hemorrhage (39.0% of patients in the 5-PLAGH cohort and 42.9% of the 1-PLAGH and 8-PLAGH cohort). During the mean ± SD follow-up period of 10.4 ± 2.9 years, 91 (16.4%) patients had rebleeding events in the 5-PLAGH cohort. The rebleeding rates were 12.3% (68 patients) at 5 years and 14.8% (82 patients) at 10 years. Rebleeding events were observed in 72 patients (14.3%) in the encephaloduroarteriosynangiosis (EDAS) surgery group, whereas 19 patients (37.3%) experienced rebleeding events in the conservative treatment group. This difference was statistically significant (p < 0.001). We selected 4 predictors (age at onset, number of episodes of bleeding, posterior circulation involvement, and EDAS surgery) for nomogram development. The concordance index (C-index) values of the nomograms of the training cohort, internal validation cohort, and the external validation cohort were 0.767 (95% CI 0.704-0.830), 0.814 (95% CI 0.694-0.934), and 0.718 (95% CI 0.661-0.775), respectively. The nomogram at 5 years exhibited a sensitivity of 48.1% and specificity of 87.5%. The positive and negative predictive values were 38.2% and 91.3%, respectively. The nomogram at 10 years exhibited a sensitivity of 47.1% and specificity of 89.1%. The positive and negative predictive values were 48.5% and 88.5%, respectively. EDAS may prevent rebleeding events and improve long-term clinical outcomes in patients with HMMD. The nomogram accurately predicted rebleeding events and assisted clinicians in identifying high-risk patients and devising individual treatments. Simultaneously, comprehensive and ongoing monitoring should be implemented for specific patients with HMMD throughout their entire lifespan.

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 that is rupture-prone. Steno-occlusive changes in the posterior cerebral arteries (PCAs) may contribute to worsened outcomes in patients with MMD; however, there is little information on the incidence and natural history of posterior circulation MMD (PCMMD). We describe clinical PCMMD characteristics in a large cohort of patients with MMD. We retrospectively reviewed patients with MMD treated between 1991 and 2019 at a large academic medical center. Demographics, perioperative outcomes, and radiological phenotypes were recorded for 770 patients. PCA disease was graded as either 0 (no disease), 1 (mild), 2 (moderate), or 3 (severe or occluded) based on cerebral angiography. Patients with angiographically confirmed MMD diagnosis with at least 6 months follow-up and completion of revascularization surgery were included; patients with intracranial atherosclerosis, intracranial dissection, vasculitis, and undefined inflammatory processes were excluded. The presence of stenosis/occlusion was graded radiographically to assess for disease progression and the prevalence of risk factors related to reduced progression-free survival. In all, 686 patients met the inclusion criteria, with PCA disease identified in 282 (41.1%) patients. Of those 282 patients with PCMMD, disease severity ranged from 99 (35.1%) with mild, 72 (25.5%) with moderate, and 111 (39.4%) with severe. The total number of postoperative complications was significantly associated with PCMMD severity (P=0.0067). Additionally, PCMMD severity correlated with worse postoperative modified Rankin Scale scores (P<0.0001). At a mean follow-up of 6.0±3.9 (range, 0.1-25.0) years, a total of 60 (12.6%) patients showed new/worsening PCMMD. The overall postoperative, progression-free survival in patients with PCMMD was 95.4% at 1 year, 82.4% at 3 years, 68.8% at 5 years, and 28.3% at 10 years, with prognostic factors for progression including preoperative PCMMD status, history of tobacco use, and hypertension (P<0.0001, P<0.001, and P<0.0001, respectively). PCA disease involvement in MMD is associated with higher rates of ischemic perioperative complications and worsened functional outcomes, likely due to reduced collateral flow. Ten-year progression of PCA disease is highly likely and should be monitored throughout follow-up; future studies will assess the impact of PCA disease progression on long-term outcomes.

Prevalence of Systemic Hypertension and the Effects of Cerebral Revascularization in Patients With Moyamoya Disease

Background Hypertension is often codiagnosed in patients with moyamoya disease (MMD), a progressive intracranial steno‐occlusive vasculopathy; this has principally been attributed to renal artery stenosis (up to 10%). Susceptibility MMD genes, including ring finger protein 213/mysterin and GUCY1A3 , have also been linked to extracranial vascular disease and increased systolic blood pressure. We aimed to define the prevalence of systemic hypertension in MMD patients and characterize its evolution after cerebral revascularization. Methods Patients with MMD treated with extracranial‐intracranial bypass from 2014 to 2018 were retrospectively enrolled. Blood pressure measurements and antihypertensive agent use were recorded pre‐ and postoperatively. Hypertension was defined according to the 2020 International Society of Hypertension Guidelines (adults) and 2017 American Academy of Pediatrics Guidelines (children). Multivariate logistic regression was performed for clinical and radiographic predictors of hypertension. Results A total of 242 adult and 51 pediatric patients underwent revascularization. Preoperatively, 146 adult and 20 pediatric patients met the diagnostic criteria for hypertension resulting in prevalences of 60.3% and 39.2% respectively. In adults, this was significantly associated with age (odds ratio [OR] 1.05 [95% CI, 1.02–1.09]), body mass index (OR, 1.08 [95% CI, 1.03–1.13]), hyperlipidemia (OR, 2.57 [95% CI, 1.09–6.04]), kidney disease (OR, 18.98 [95% CI, 1.80–200.47]), and symptomatic presentation (OR, 8.88 [95% CI, 1.16–68.06]). After a mean follow‐up of 34.3±18.1 months in adults (33.8±14.9 months – children), patients with hypertension decreased by 15.3% (1.9% – pediatrics) and 31.8% (17.7% – children) experienced improvement in hypertensive status with normalization of blood pressure or reduced need for antihypertensive agents. Posterior circulation involvement was a negative predictor for response of hypertensive status to revascularization (OR, 0.10 [95% CI, 0.01–0.79]). Conclusion Hypertension is prevalent among adult and pediatric patients with MMD with contributions from known vascular risk factors. Its association with symptomatic presentation and observed improvement following revascularization suggests blood pressure changes, in part, are a compensatory physiological response to increased intracranial vascular resistance.

Clinical and genetic factors associated with contralateral progression in unilateral moyamoya disease: Longitudinal and Cross-Sectional Study

ObjectiveThis study aimed to explore the long-term outcome of unilateral moyamoya disease and predict the clinical and genetic factors associated with contralateral progression in unilateral moyamoya disease. MethodsWe retrospectively recruited unilateral moyamoya disease patients with available genetic data who underwent encephaloduroarteriosynangiosis (EDAS) surgery at our institution from January 2009 to November 2017. Long-term follow-up data, including clinical outcomes, angiographic features, and genetic information, were analyzed. ResultsA total of 83 unilateral moyamoya disease patients with available genetic data were enrolled in our study. The mean duration of clinical follow-up was 7.9 ± 2.0 years. Among all patients, 19 patients demonstrated contralateral progression to bilateral disease. Heterozygous Ring Finger Protein 213 p.R4810K mutations occurred significantly more frequently in unilateral moyamoya disease patients with contralateral progression. Furthermore, patients with contralateral progression typically demonstrated an earlier age of onset than those with non-progressing unilateral moyamoya disease. In the contralateral progression group, posterior circulation involvement was observed in 11 (11/19, 57.9%) patients compared to 12 (12/64, 18.8%) in the non-contralateral progression group (P = 0.001). The time to peak of cerebral perfusion and neurological status showed significant postoperative improvement. ConclusionLong-term follow-up revealed that the EDAS procedure might provide benefits for unilateral moyamoya disease patients. Ring Finger Protein 213 p.R4810K mutations, younger age, and posterior circulation involvement might predict the contralateral progression of unilateral moyamoya disease.

Neurovascular considerations in patients with Down syndrome and moyamoya syndrome.

In this article, we describe a rare and complex case of moyamoya syndrome in a 7-year-old boy with Down syndrome and atlantoaxial subluxation. The patient presented with an ischemic stroke in the left hemisphere and cervical cord compression with increased cord edema. Diagnostic digital subtraction angiography revealed unique patterns of vascular involvement, with retrograde flow through the anterior spinal artery, ascending cervical artery, occipital artery, and multiple leptomeningeal arteries compensating for bilateral vertebral artery occlusion.This case underscores the underreported phenomenon of upward retrograde flow through the anterior spinal artery in bilateral vertebral artery occlusion. We address the rare manifestation of posterior circulation involvement in moyamoya syndrome, highlighting the importance of considering atlantoaxial instability as a contributing factor, as the absence of atlantoaxial stability is a risk factor for vertebral artery dissection. This study contributes valuable insights into the intricate relationship of moyamoya syndrome, Down syndrome, and atlantoaxial instability, urging clinicians to consider multifaceted approaches in diagnosis and treatment. It also emphasizes the potential significance of the anterior spinal artery as a compensatory pathway in complex vascular scenarios.

Predictors of congruency between clinical and radiographic Oxfordshire Community Stroke Project Classification subtypes

BackgroundThe Oxfordshire Community Stroke Project (OCSP) classification has been widely used to assess ischemic stroke patients based on clinical characteristics alone. However, the correlation between the clinical presentation evaluated using OCSP and imaging findings is yet to be determined. Our study aimed to describe the baseline characteristics of the OCSP subtypes of patients admitted with ischemic stroke and evaluate the predictors of the relationship between clinical and neuroimaging findings. MethodsPatients with a confirmed diagnosis of ischemic stroke admitted to a comprehensive stroke center in Brazil between February 2015 and October 2017 were eligible for the study. All participants underwent computed tomography (CT) at admission and follow-up neuroimaging within seven days, per the institutional protocol. Trained staff classified patients according to the OCSP at hospital admission. The radiographic OCSP classification was retrospectively assessed based on the last follow-up neuroimaging by investigators unaware of the clinical classification. ResultsThe overall agreement rates ranged from 65.5% to 88.7%. Lower NIHSS scores, absent hyperdense MCA sign, higher ASPECTS, and absent brainstem symptoms were related to a higher risk of misclassification. Treatment with intravenous tPA was associated with reclassification in patients with total anterior circulation syndrome. For predicting radiographic posterior circulation involvement, vertigo (OR 2.9, 95% CI 1.7–5.1, p < 0.001) and brainstem symptoms (OR 35, 95% CI 20.5–60.2, p < 0.001) were directly associated with correct classification, but motor and higher cerebral function were not correlated. ConclusionThe clinical OCSP classification presented good congruency rates with the neuroimaging findings. However, patients with lacunar syndromes are often misclassified when radiological criteria are considered.

Substance use disorder in young adults with stroke: clinical characteristics and outcome.

Stroke incidence among young adults has risen in the last decade. This research attempts to determine the effect of substance use disorder (SUD) on the clinical characteristics of stroke, mortality, outcome after IV thrombolysis, and functional dependency after 1 month among young adults. Through a retrospective study, data were extracted from the electronic medical records of stroke in young adults admitted to intensive care units in Kasr Al-Ainy Hospital (February 2018-January 2021). The National Institute of Health Stroke Scale (NIHSS) and the Modified Rankin Scale were documented at the onset and after 1 month. The study included 225 young adults with stroke (median age of 40, IQR: 34-44). Only 93 young adults (41%) met the criteria of SUD. Anabolic steroid use disorder was significantly associated with cerebral venous thrombosis (P-value = 0.02), while heroin use disorder was significantly associated with a hemorrhagic stroke (P-value = 0.01). Patients with tramadol, cannabis, and cocaine use disorders had significantly more frequent strokes in the posterior than the anterior circulation. Patients with heroin use disorders had significantly higher mortality than those without heroin use disorders (P-value = 0.01). The risk of poor outcomes was doubled by alcohol or heroin use disorder, while it was tripled by cocaine use disorder (P-value = 0.01 for each). Forty-one percent of young adults diagnosed with a stroke had SUD, with a relatively higher posterior circulation involvement. Increased mortality was associated with heroin use disorder more than other substances. Poor stroke outcome was associated with alcohol, heroin, and cocaine use disorders.

Association between DIAPH1 variant and posterior circulation involvement with Moyamoya disease

Moyamoya disease (MMD) is a chronic and progressive cerebrovascular stenosis or occlusive disease that occurs near Willis blood vessels. The aim of this study was to investigate the mutation of DIAPH1 in Asian population, and to compare the angiographic features of MMD patients with and without the mutation of the DIAPH1 gene. Blood samples of 50 patients with MMD were collected, and DIAPH1 gene mutation was detected. The angiographic involvement of the posterior cerebral artery was compared between the mutant group and the non-mutant group. The independent risk factors of posterior cerebral artery involvement were determined by multivariate logistic regression analysis. DIAPH1 gene mutation was detected in 9 (18%) of 50 patients, including 7 synonymous mutations and 2 missense mutations. However, the incidence of posterior cerebral artery involvement in mutation positive group was very higher than that in mutation negative group (77.8% versus 12%; p = 0.001). There is an association between DIAPH1 mutation and PCA involvement (odds ratio 29.483, 95% confidence interval 3.920–221.736; p = 0.001). DIAPH1 gene mutation is not a major genetic risk gene for Asian patients with moyamoya disease but may play an important role in the involvement of posterior cerebral artery.

Childhood posterior circulation arterial ischemic stroke – An unusual case with a possible COVID-19-related arteriopathy

Viral infections are known to increase predilection to stroke and coronavirus disease 2019 (COVID-19) has proven these concerns true. We are presenting the case of a 6-year-old previously normal male child diagnosed with posterior circulation stroke who had all etiological workups negative except for the COVID antibody. Imaging showed thrombi over the V3 segment of the vertebral artery at C1–C2 level causing near complete occlusion, with embolic infarct in the bilateral posterior inferior cerebellar artery, bilateral posterior cerebral artery, bilateral anterior inferior cerebellar artery, and the left superior cerebellar artery. The child was managed with methylprednisolone, anticoagulation, and supportive care and was able to restore near-normal neurological status within months. This case is unique in terms of the involvement of posterior circulation which is rare in the pediatric population. A possibility of inflammation-related arteriopathy secondary to infection should be considered in the etiological workup of stroke. Anti-inflammatory measures to control cytokine storm along with supportive care will ensure a good outcome.

Transcranial Doppler Screening for Patent Foramen Ovale Closure in Cryptogenic Strokes in Young: A Single Center Experience from South India.

Cryptogenic strokes are common in young adults. Patent foramen ovale (PFO) is an important cause of cryptogenic ischemic strokes. Transcranial Doppler (TCD) with bubble contrast is a noninvasive bedside tool in screening for PFO and other right to left shunt (R-L shunt). Percutaneous PFO closure in selected patients with a high risk for paradoxical emboli is beneficial. Data on PFO in young cryptogenic strokes from India are limited. To determine the utility of screening for R-L shunt using TCD in young patients with cryptogenic strokes and to identify clinical predictors of an R-L shunt. This was a hospital-based prospective study conducted between January 2013 and December 2019 in a tertiary hospital in South India. All consecutive patients with ischemic stroke and ages between 18 and 45 years were included. TCD with bubble contrast study was performed on all patients. Those who were TCD bubble contrast study positive and had features of an embolic stroke of undetermined source (ESUS) underwent transesophageal echocardiography (TEE) to confirm a PFO and to look for its high-risk features. Selected ESUS patients with PFO and associated high-risk features as identified on TEE underwent percutaneous PFO device closure. All patients were followed up in the stroke and cardiology clinics. During the study period, 6,197 patients with ischemic strokes were screened for eligibility of which 304 (4.9%) were between the age of 18 and 45 years. Of these, 300 patients with ischemic stroke in young underwent the TCD bubble contrast study. R-L shunt was found in 121 (40.3%) patients. Based on an extensive etiological evaluation, 72 patients were identified to have an ESUS and underwent TEE for confirming PFO. Of these, 65 patients had PFO, four were negative, and three were found to have extracardiac shunts. Based on clinical findings, imaging features, and high-risk features on TEE, 29 patients underwent PFO closure. Activity at the time of stroke onset equivalent to a Valsalva maneuver (p ≤ 0.01), isolated cortical infarction (p = 0.027), and posterior circulation involvement (p = 0.0135) were significantly associated with the presence of an R-L shunt. The patients who had a higher modified anatomical-functional risk of paradoxical embolism (AF-RoPE) score, a high-grade shunt on the TCD bubble contrast study had a longer length of the tunnel and had the presence of an interatrial septal aneurysm (p = 0.012) were referred for PFO device closure. R-L shunt is common in cryptogenic ischemic strokes in young. TCD with bubble contrast study is a noninvasive and feasible bedside tool to detect them. Applying the ESUS criteria in these cryptogenic strokes with a positive TCD bubble contrast study can be then used for selecting patients for more invasive tests like TEE. High-risk PFOs picked up with TEE can be then considered for PFO closure for secondary stroke prevention. The history of Valsalva maneuver-like activity (such as lifting heavy weights or straining) at the time of stroke onset can be a clinical predictor for the presence of an R-L shunt. In addition to isolated cortical infarction, the presence of posterior circulation infarct in ESUS can predict the presence of an R-L shunt.

Mechanical thrombectomy in a Chilean public hospital. Experience in 149 patients

Mechanical thrombectomy is the accepted treatment for acute ischemic stroke in Large Vessel Occlusion. The Barros Luco Trudeau hospital developed endovenous thrombolysis in 2010, and since 2012, implemented endovascular management, becoming the neurovascular center in the southern area of the metropolitan region. To describe endovascular management of acute ischemic stroke in a Chilean public hospital. Analysis of patients with acute ischemic stroke that were treated with mechanical throm-bectomy from 2012 to 2019 in the Barros Luco Hospital. In the study period, a mechanical thrombectomy was carried out in 149 patients aged 61 ± 15 years (46% females). The average National institute of Health Stroke Scale (NIHSS) at presentation was 19 ± 4-5. Anterior or posterior circulation involvement was present in 89.9 and 10.1 % of patients. Twenty-five percent of patients were referred from other public centers. The mean lapse between onset of symptoms and thrombectomy was 266 ± 178 in. Ninety days after the procedure, 58% of patients had minimal or absent disability (Modified Ranson score of 0-2), and 19,2% died. Mechanical thrombectomy, according to this experience, has favorable clinical outcomes in patients with high NIHSS scores at entry.

Clinical features and outcomes of paediatric moyamoya vasculopathy in Malaysia

Background: Moyamoya vasculopathy (MMV) is a chronic progressive occlusive cerebrovascular disease causing recurrent strokes in children. We describe the clinical features, treatment and outcomes of childhood MMV in Malaysia; and compared their neurological outcome with the published literature. Methods: A retrospective study between 2005–2020 of Malaysian children with MMV seen at 4 tertiary Malaysian hospitals. Their post-stroke outcomes were assessed using the modified Rankin scale (mRS) and Pediatric Stroke Outcome Measure (PSOM). Results: Twenty one cases were included with a median age of presentation at 5.8 years and a median follow-up period of 4.6 years. The female-to-male-ratio was 2:1. All patients had acute ischaemic cerebrovascular accidents. The anterior cerebral circulation was affected in all cases with additional three having also posterior circulation involvement. Among 17 (81%) patients neuroimaging showed bilateral moyamoya. Eight (30%) had preceding febrile illness and the commonest presenting symptom was hemiparesis seen in 8 (38%). Seven (35%) had surgical revascularisation whilst the remainder opted for conservative management. Nineteen (90%) achieved a good stroke outcome but 2 had a poor PSOM outcome and 1 also had a poor mRS outcome. Both these patients declined surgical revascularisation. Conclusion: Our cohort demonstrated the following MMV features: young age at the first presentation, female and Chinese preponderance; the main type of MMV was moyamoya disease; and ischaemic stroke as the commonest presentation. The overall neurological and functional outcomes were good despite the parental preference on medical therapy over surgical revascularization.

Homonymous Hemianopia Secondary to A Long Fusiform Aneurysm of Posterior Cerebral Artery in A Patient with Connective Tissue Disease

Highlight: Fusiform aneurysms are uncommon, accounting for only 1% of total intracranial aneurysms, and posterior circulation involvement is only 3-13% of cases of intracranial aneurysms. Connective tissue diseases are very rare as the cause of this vasculopathy. Homonymous hemianopia due to the mass effect or dilated blood vessels compressing the left optic tract. ABSTRACT Introduction: Fusiform aneurysms are uncommon, accounting for 1% of all intracranial aneurysms. Dissection and atherosclerosis are the main causes of this vasculopathy, but connective tissue disease is a very uncommon cause. Ehlers-Danlos Syndrome is the most common connective tissue disease, accounting for 11% of all cases. Symptoms depend on the location and size of the aneurysm, including headaches, blurred or double vision, and focal neurological deficits. Case: A 36-year-old man suddenly experienced blurred vision in both eyes on the right, starting with a chronic left-sided headache and no history of cardiovascular disease. In the confrontation test, Humphrey gave the right homonymous hemianopia. A head CT scan showed a lobulated lesion which showed enhancement in the left suprasellar region, and cerebral digital subtraction angiography (DSA) gave the impression of a long fusiform aneurysm L PCA. Clinically, the patient’s skin on the left side of his face was darker than on the right, his skin was more elastic, and his blood vessels were wider and more prominent on the side of the fusiform aneurysm. Conclusion: Posterior circulation involvement is only 3-13% of cases of intracranial aneurysms. Many cases of intracranial aneurysms are not detected before rupture, resulting in delays in treatment. Surgical or endovascular surgery can be performed if the size is &gt;10 mm and causes clinical symptoms. Symptoms of ischemia are managed with antiplatelets or anticoagulants. Incidentally detected unruptured aneurysms are generally managed conservatively because of the highly friable nature of the blood vessels in patients with connective tissue diseases.

Pediatric Moyamoya Disease and Syndrome in Italy: A Multicenter Cohort.

BackgroundMoyamoya is a rare progressive cerebral arteriopathy, occurring as an isolated phenomenon (moyamoya disease, MMD) or associated with other conditions (moyamoya syndrome, MMS), responsible for 6–10% of all childhood strokes and transient ischemic attacks (TIAs).MethodsWe conducted a retrospective multicenter study on pediatric-onset MMD/MMS in Italy in order to characterize disease presentation, course, management, neuroradiology, and outcome in a European country.ResultsA total of 65 patients (34/65 women) with MMD (27/65) or MMS (38/65) were included. About 18% (12/65) of patients were asymptomatic and diagnosed incidentally during investigations performed for an underlying condition (incMMS), whereas 82% (53/65) of patients with MMD or MMS were diagnosed due to the presence of neurological symptoms (symptMMD/MMS). Of these latter, before diagnosis, 66% (43/65) of patients suffered from cerebrovascular events with or without other manifestations (ischemic stroke 42%, 27/65; TIA 32%, 21/65; and no hemorrhagic strokes), 18% (12/65) of them reported headache (in 4/12 headache was not associated with any other manifestation), and 26% (17/65) of them experienced multiple phenotypes (≥2 among: stroke/TIA/seizures/headache/others). Neuroradiology disclosed ≥1 ischemic lesion in 67% (39/58) of patients and posterior circulation involvement in 51% (30/58) of them. About 73% (47/64) of patients underwent surgery, and 69% (45/65) of them received aspirin, but after diagnosis, further stroke events occurred in 20% (12/61) of them, including operated patients (11%, 5/47). Between symptom onset and last follow-up, the overall patient/year incidence of stroke was 10.26% (IC 95% 7.58–13.88%). At last follow-up (median 4 years after diagnosis, range 0.5–15), 43% (26/61) of patients had motor deficits, 31% (19/61) of them had intellectual disability, 13% (8/61) of them had epilepsy, 11% (7/61) of them had behavioral problems, and 25% (13/52) of them had mRS > 2. The proportion of final mRS > 2 was significantly higher in patients with symptMMD/MMS than in patients with incMMS (p = 0.021). Onset age <4 years and stroke before diagnosis were significantly associated with increased risk of intellectual disability (p = 0.0010 and p = 0.0071, respectively) and mRS > 2 at follow-up (p = 0.0106 and p = 0.0009, respectively).ConclusionsMoyamoya is a severe condition that may affect young children and frequently cause cerebrovascular events throughout the disease course, but may also manifest with multiple and non-cerebrovascular clinical phenotypes including headache (isolated or associated with other manifestations), seizures, and movement disorder. Younger onset age and stroke before diagnosis may associate with increased risk of worse outcome (final mRS > 2).

Epidemiology of Moyamoya Angiopathy in Eastern India.

IntroductionMoyamoya angiopathy (MMA) is a chronic, progressive intracranial vasculopathy with variation in prevalence and clinical manifestations across different populations. This study was aimed to estimate the frequency of MMA as an etiology of stroke and its epidemiological features in the largest cohort of MMA patients in India.MethodA single-centered cross-sectional observational study over a period of 5 years (2016–2021) was undertaken among consecutive stroke and transient ischemic attack (TIA) patients to look for the presence of MMA angiographically. Each patient with angiographically proven MMA was further evaluated for demographic, clinical, and radiological characteristics.ResultsAmong 10,250 consecutive stroke and TIA patients (ischemic = 78%, hemorrhagic = 22%), frequency of MMA was 1.56% (n = 160); 15.3% among children. Female preponderance (Male:Female = 1:1.4) was noted among 160 MMA patients, with bimodal age distribution, first peak at 3–8 years, and a shorter second peak at 41–47 years. Childhood-onset MMA was seen in 75 (46.9%) with commonest initial neurological symptom of fixed-motor-weakness (44.0%), followed by TIA (26.7%); while 85 (53.1%) had adult-onset MMA with fixed-motor-weakness (50.6%) followed by headache (24.7%) as the predominant initial neurological symptom; seizure significantly higher in children (p < 0.001) and headache in adults (p = 0.012). Transient and fixed neurological manifestations constituted 87.5 and 69.4% respectively, of symptoms throughout the disease course. Cerebral infarction (45.0%) and TIA (21.9%) were the commonest types of MMA. On brain imaging, infarction was noted in 80.6%, hemorrhage in 11.3%, significantly higher among adults (p < 0.001). Cortical infarct and Gyral pattern were commoner in children (p = 0.004), subcortical infarcts in adults (p = 0.018). Frequent Suzuki staging observed was stage 4 (31.3%), followed by stage 3 (30.0%). Involvement of posterior circulation was detected in 55.6%, brain atrophy at the time of diagnosis was seen in 65.0%.ConclusionMMA is an important etiological consideration in patients with stroke, especially in children. It can present with a myriad of transient neurological symptoms, frequently overlooked, leading to delayed diagnosis, and contributing to socio-economic burden. Indian MMA showed aberrations in its gender predisposition, age distribution, frequency of familial cases, disease manifestation, and type of stroke, in comparison to its Japanese and Caucasian counterparts pointing to the inter- and intra-continent differences of MMA phenotype. Future development of the Indian MMA national registry is of essence.

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.