Fareed Suri

Prevalence and characteristics of concurrent down syndrome in patients with moyamoya disease.

BACKGROUND An association between moyamoya disease and Down syndrome appears to exist on the basis of reported anecdotal cases in the literature. OBJECTIVE To determine the prevalence of Down syndrome associated with moyamoya disease in inpatients and to identify the demographic and clinical features of moyamoya disease that may be unique when associated with Down syndrome. METHODS In this observational study, we analyzed data from the Nationwide Inpatient Sample between 2002 and 2009 using International Classification of Diseases codes for moyamoya disease and Down syndrome for patient identification. Data including patient age, sex, race/ethnicity, secondary diagnosis, procedures, hospital costs, and patient outcomes were obtained. RESULTS From 2002 to 2009, an estimated 518 patients (mean ± SD age, 16.2 ± 1.68 years) with coexisting moyamoya disease and Down syndrome were admitted. The estimated prevalence was 3.8% (3760 per 100,000) among patients admitted with moyamoya disease and 9.5% (9540 per 100,000) among moyamoya patients < 15 years of age. Patients admitted with moyamoya disease and Down syndrome were most frequently white and Hispanic (P = .02). They were more likely to present with ischemic stroke and less commonly with hemorrhagic stroke (15.3% and 2.7%, respectively; P < .05). CONCLUSION This is the first study to estimate the prevalence of Down syndrome in patients with moyamoya disease. The 26-fold-greater prevalence of Down syndrome in patients with coexisting moyamoya disease compared with the prevalence of Down syndrome among live births (145 per 100,000) highlights the need for a better understanding of the common pathophysiology of the 2 conditions.

Neurointerventional Procedural Volume per Hospital in United States Implications for Comprehensive Stroke Center Designation

Background and Purpose— Availability of neurointerventional procedures is recommended as a necessary component of a comprehensive stroke center by various regulatory guidelines that also emphasize adequate procedural volumes. We studied the volumes of neurointerventional procedures performed in various hospitals across the United States with subsequent comparisons with rates of minimum procedural volumes recommended by various professional bodies or used in clinical trials to ensure adequate operator experience. Methods— We reviewed the Nationwide Inpatient Sample database in the United States for the years 2005 to 2008. Using International Classification of Disease—Clinical Modification, 9th revision, and Medicare severity diagnosis-related group codes, we identified among hospitals that admit stroke patients those that met the minimum criteria for overall and individual procedural volumes specified in various guidelines. We then compared the characteristics between the high-volume hospitals that performed at least 100 cervicocerebral angiograms and met ≥1 other procedural criterion (n=79) and low-volume hospitals (n=958). Results— Proportions of hospitals that met individual procedural volume criteria over the 4-year period according to procedure were: cervicocerebral angiography (7.0%–7.8%); endovascular acute ischemic stroke treatments (0.4%–2.6%); carotid angioplasty/stent placement (3.0%–5.3%); intracranial angioplasty/stent placement (0.3%–1.3%); and aneurysm embolization (1.3%–2.6%). There were significant trends for increasing numbers of all the endovascular procedures except intracranial angioplasty/stent placement over the course of 4 years. The high-volume hospitals were more likely to be urban teaching hospitals (70.9% versus 13.1%; P<0.001), had larger bed size (79.7% versus 26.9%; P<0.001), and had significantly higher rates of hemorrhagic stroke admissions and lower rates of transient ischemic attack admissions. Urban teaching location/status (OR, 8.92; CI, 4.3–18.2; P<0.001) and large bed size (OR, 4.40; CI, 2.0–9.5; P<0.001) remained as independent predictors of a high-volume hospital when adjusted for age, gender, risk factors, and stroke subtype. Conclusions— There are very few hospitals in the United States that meet all the neurointerventional procedural volume criteria for all endovascular procedures recommended to ensure adequate operator experience. Our results support the creation of specialized regional centers for ensuring adequate procedural volume within treating hospitals.

Epidemiological and clinical features of moyamoya disease in the USA

Background: An increasing number of cases of Moyamoya disease have been reported in the Japanese and US literature. We performed this study to quantify the rise in the prevalence of Moyamoya disease and to study the unique epidemiological and clinical features in the USA that may explain a change in incidence. Methods: We analyzed data derived from patients entered in the Nationwide Inpatient Sample between 2005 and 2008, using ICD-9 codes for Moyamoya disease. Data including patient age, gender, ethnicity, secondary diagnosis, medical complications, and hospital costs were obtained. Results: From 2005 to 2008 in the USA, there were an estimated 7,473 patients admitted with a primary or secondary diagnosis of Moyamoya disease. Patients admitted with Moyamoya disease were most frequently women and Caucasian. Overall, ischemic stroke was the most common reason for admission. Hemorrhagic stroke was more frequent in adults compared with children, 18.1 versus 1.5% (p < 0.05). Conclusion: The number of patients identified and admitted with Moyamoya disease has risen dramatically in the last decade. This study can lead to a better understanding of the disease pattern and healthcare consequences in the USA and suggests that pathophysiologic differences in Moyamoya disease may exist.

MR imaging measures of intracranial atherosclerosis in a population-based study

Purpose To implement a magnetic resonance (MR) imaging protocol to measure intracranial atherosclerotic disease (ICAD) in a population-based multicenter study and report examination and reader reliability of these MR imaging measurements and descriptive statistics representative of the general population. Materials and Methods This prospective study was approved by the institutional review boards and compliant with HIPAA. Atherosclerosis Risk in Communities (ARIC) study participants (n = 1980) underwent brain MR imaging from 2011 to 2013 at four ARIC sites. Imaging included three-dimensional black-blood MR imaging and time-of-flight MR angiography. One hundred two participants returned for repeat MR imaging to estimate examination and reader variability. Plaque presence according to vessel segment was recorded. Quantitative measurements included lumen size and degree of stenosis, wall and/or plaque thickness, area and volume, and normalized wall index for each vessel segment. Reliability was assessed with percentage agreement, κ statistics, and intraclass correlation coefficients. Results Of the 1980 participants, 1755 (mean age, 77.6 years; 1026 women [59%]; 1234 white [70%]) completed examinations with adequate to excellent image quality. The weighted ICAD prevalence was 34.4% (637 of 1755 participants) and was higher in men than women (38.5% [302 of 729 participants] vs 31.7% [335 of 1026 participants], respectively; P = .012) and in African Americans compared with whites (41.1% [215 of 518 participants] vs 32.4% [422 of 1234 participants], respectively; P = .002). Percentage agreement of plaque identification per participant was 87.0% (interreader estimate), 89.2% (intrareader estimate), and 89.9% (examination estimate). Examination and reader reliability ranged from fair to good (κ, 0.50-0.78) for plaque presence and from good to excellent (intraclass correlation coefficient, 0.69-0.99) for quantitative vessel wall measurements. Conclusion Vessel wall MR imaging is a reliable tool for identifying and measuring ICAD and provided insight into ICAD distribution across a U.S. community-based population. (©) RSNA, 2016 Online supplemental material is available for this article.

Racial differences in prevalence and risk for intracranial atherosclerosis in a us community-based population

Importance Intracranial atherosclerotic disease (ICAD) is an important cause of stroke; however, little is known about racial differences in ICAD prevalence and its risk factors. Objective To determine racial differences in ICAD prevalence and the risk factors (both midlife and concurrent) associated with its development in a large, US community-based cohort. Design, Setting, and Participants Analysis of 1752 black and white participants recruited from the Atherosclerosis Risk in Communities (ARIC) cohort study who underwent 3-dimensional intracranial vessel wall magnetic resonance imaging from October 18, 2011 to December 30, 2013; data analysis was performed from October 18, 2011 to May 13, 2015. Exposures Midlife and concurrent cardiovascular risk factors. Main Outcomes and Measures Intracranial plaque presence, size (maximum normalized wall index) and number were assessed by vessel wall magnetic resonance imaging. Midlife and concurrent vascular risk factor associations were determined by Poisson regression (plaque presence), negative binominal regression (plaque number), and linear regression (plaque size), and compared between races. Results Of the 1752 study participants (mean [SD] age, 77.6 [5.3] years; range, 67-90 years), 1023 (58.4%) were women and 518 (29.6%) were black. Black men had the highest prevalence (50.9% vs 35.9% for black women, 35.5% for white men, and 30.2% for white women; P < .001) and the highest frequency (22.4% vs 12.1% for black women, 10.7% for white men, and 8.7% for white women; P < .01) of multiple plaques. Prevalence increased with age, reaching 50% before ages 68, 84, and 88 years in black men, white men, and white women, respectively (ICAD prevalence remained <50% in black women). Midlife hypertension and hyperlipidemia were associated with 29% (prevalence ratio [PR], 1.29; 95% CI, 1.08-1.55) and 18% (PR, 1.18; 95% CI, 0.98-1.42), respectively, increased ICAD prevalence with no significant differences between races. Midlife hypertension was also associated with larger plaques (1.11 higher mean maximum normalized wall index; 95% CI, 0.21-2.01). Midlife smoking and diabetes were associated with increased risk of ICAD in black individuals (102% [PR, 2.02; 95% CI, 1.12-3.63] and 57% [PR, 1.57; 95% CI, 1.13- 2.19], respectively), but not in white participants (P < .05 interaction by race). Conclusions and Relevance The prevalence of ICAD was highest in black men. Midlife smoking and diabetes were strongly associated with late-life ICAD in blacks only, whereas midlife hypertension and hyperlipidemia were associated with late-life ICAD in both races. These associations may help to explain racial differences in US stroke rates and offer insight into preventive risk-factor management strategies.