Daraspreet Kainth

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.

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.

The Emergence of Endovascular Treatment–Only Centers for Treatment of Intracranial Aneurysms in the United States

BACKGROUND Because of the availability of new technology, the spectrum of endovascular treatment for intracranial aneurysms has expanded widely. Some centers have started offering only endovascular treatment to patients with intracranial aneurysms (endovascular treatment-only centers [ETOCs]). Our objective was to identify the proportion and outcome of patients treated at ETOCs in the United States. METHODS We determined the proportion of ETOCs in the United States using Nationwide Inpatient Survey data files from 2010. We compared short-term outcomes between ETOCs and endovascular and surgical treatment centers (ESTCs). The outcomes studied were none to minimal disability, moderate to severe disability, in-hospital mortality, postprocedure complications, length of stay, and hospital charges. RESULTS Out of 85 hospitals performing endovascular treatment of unruptured aneurysms, 13 (15%) were categorized as ETOCs. Out of the 10,447 patients with unruptured aneurysms, 1245 (12%) were treated at ETOCs. ETOCs were more likely to be nonteaching hospitals (55% versus 45%, P=.02). The rates of in-hospital mortality (1.2% versus 1.8%) and none to minimal disability (88% versus 84%) were similar in patients treated at ETOCs and ESTC hospitals. The mean hospitalization charges were similar, but length of stay (4±7 days versus 6±10 days, P<.0001) was significantly shorter among patients treated at ETOCs. Only 2.7% patients required secondary neurosurgical procedures at the ETOCs compared with 5.8% in ESTCs (P=.09). CONCLUSION The recent emergence of ETOCs and provision of treatment with comparable outcomes and shorter length of stay at these hospitals may change the pattern of intracranial aneurysm treatment in the United States.

Preprocedure change in arterial occlusion in acute ischemic stroke patients undergoing endovascular treatment by computed tomographic angiography.

BACKGROUND The American Heart Association/American Stroke Association guidelines strongly recommend a noninvasive intracranial vascular study such as computed tomographic (CT) angiogram in acute stroke patient if endovascular treatment is contemplated. OBJECTIVE The objective was to determine the frequency of change in occlusion site between CT angiogram and cerebral angiogram in acute ischemic stroke patients undergoing endovascular treatment. METHODS All acute ischemic stroke patients who underwent a CT angiogram and subsequently underwent endovascular treatment were included. The CT and cerebral angiographic images were reviewed independently to determine presence and location of arterial occlusion. Severity of occlusion was classified by a previously described grading scheme. Clinical outcome at discharge was determined using modified Rankin scale. RESULTS Computed tomographic angiogram was performed in 150 patients (mean age ± SD, 64.7 ± 16 years) before endovascular treatment. The mean interval (±SD) between CT angiogram and cerebral angiogram was 193 ± 164 minutes, and 65 (43.3%) of 150 patients received intravenous recombinant tissue plasminogen activator before cerebral angiography. Recanalization between CT angiogram and cerebral angiography was seen in 28 (18.7%) patients, whereas worsening of occlusion was seen in 31 (20.7%) patients. We noticed a trend towards higher rates of improvement (60.7% vs 42.0%, P = .07) and favorable outcome at discharge (42.9% vs 28.7%, P = .1) among patients who experienced preprocedure recanalization. After adjusting for age and initial National Institutes of Health Stroke Scale score strata, preprocedure recanalization was not associated with significantly higher rate of favorable outcome (modified Rankin scale, 0-2) at discharge (odds ratio, 2.1; 95% confidence interval, 0.8-5.5). After adjusting for age and National Institutes of Health Stroke Scale score strata, preprocedure worsening was not associated with significantly lower rates of favorable outcomes at discharge (odds ratio,0.4; 95% confidence interval, 0.1-1.4). CONCLUSIONS A relatively high proportion of patients have preprocedure recanalization or worsening between CT angiogram and cerebral angiogram in acute ischemic stroke patients selected for endovascular treatment.

Contents Vol. 40, 2013

296 Regional North American Annual Meeting of the World Federation of Neurology – Research Group on Neuroepidemiology University of California at San Diego, San Diego, Calif., March 22, 2013 Guest Editors: Weisskopf, M.G. (Boston, Mass.); Leimpeter, A. (Oakland, Calif.); Van Den Eeden, S.K. (Oakland, Calif.) (available online only)

Front & Back Matter

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