Stroke is the leading cause of long term disability in developed countries and one of the top causes of mortality worldwide. The past decade has seen substantial advances in the diagnostic and treatment options available to minimize the impact of acute ischemic stroke. The key first step in stroke care is early identification of patients with stroke and triage to centers capable of delivering the appropriate treatment, as fast as possible. Here, we review the data supporting pre-hospital and emergency stroke care, including use of emergency medical services protocols for identification of patients with stroke, intravenous thrombolysis in acute ischemic stroke including updates to recommended patient eligibility criteria and treatment time windows, and advanced imaging techniques with automated interpretation to identify patients with large areas of brain at risk but without large completed infarcts who are likely to benefit from endovascular thrombectomy in extended time windows from symptom onset. We also review protocols for management of patient physiologic parameters to minimize infarct volumes and recent updates in secondary prevention recommendations including short term use of dual antiplatelet therapy to prevent recurrent stroke in the high risk period immediately after stroke. Finally, we discuss emerging therapies and questions for future research.

Patients with the same c.-32-13T-->G haplotype (c.q. GAA genotype) may manifest first symptoms at different ages, indicating that secondary factors may substantially influence the clinical course of patients with this mutation.

Background and Purpose-Risk-standardized hospital readmission rates are used as publicly reported measures reflecting quality of care. Valid risk-standardized models adjust for differences in patient-level factors across hospitals. We conducted a systematic review of peer-reviewed literature to identify models that compare hospital-level poststroke readmission rates, evaluate patient-level risk scores predicting readmission, or describe patient and process-of-care predictors of readmission after stroke. Methods-Relevant studies in English published from January 1989 to July 2010 were identified using MEDLINE, PubMed, Scopus, PsycINFO, and all Ovid Evidence-Based Medicine Reviews. Authors of eligible publications reported readmission within 1 year after stroke hospitalization and identified Ն1 predictors of readmission in risk-adjusted statistical models. Publications were excluded if they lacked primary data or quantitative outcomes, reported only composite outcomes, or had Ͻ100 patients. Results-Of 374 identified publications, 16 met the inclusion criteria for this review. No model was specifically designed to compare risk-adjusted readmission rates at the hospital level or calculate scores predicting a patient's risk of readmission. The studies providing multivariable models of patient-level and/or process-of-care factors associated with readmission varied in stroke definitions, data sources, outcomes (all-cause and/or stroke-related readmission), durations of follow-up, and model covariates. Few characteristics were consistently associated with readmission. Conclusions-This review identified no risk-standardized models for comparing hospital readmission performance or predicting readmission risk after stroke. Patient-level and system-level factors associated with readmission were inconsistent across studies. The current literature provides little guidance for the development of risk-standardized models suitable for the public reporting of hospital-level stroke readmission performance. (Stroke. 2010;41:2525-2533.)

Pompe disease was named after the Dutch pathologist Dr JC Pompe who reported about a deceased infant with idiopathic hypertrophy of the heart. The clinical findings were failure to thrive, generalized muscle weakness and cardio-respiratory failure. The key pathologic finding was massive storage of glycogen in heart, skeletal muscle and many other tissues. The disease was classified as glycogen storage disease type II and decades later shown to be a lysosomal disorder caused by acid α-glucosidase deficiency. The clinical spectrum of Pompe disease appeared much broader than originally recognized. Adults with the same enzyme deficiency, alternatively named acid maltase deficiency, were reported to have slowly progressive skeletal muscle weakness and respiratory problems, but no cardiac involvement. The clinical heterogeneity is largely explained by the kind and severity of mutations in the acid α-glucosidase gene (GAA), but secondary factors, as yet unknown, have a substantial impact. The Pompe disease mutation database aims to list all GAA sequence variations and describe their effect. This update with 107 sequence variations (95 being novel) brings the number of published variations to 289, the number of non-pathogenic mutations to 67 and the number of proven pathogenic mutations to 197. Further, this article introduces a tool to rate the various mutations by severity, which will improve understanding of the genotypephenotype correlation and facilitate the diagnosis and prognosis in Pompe disease.

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We found a strong dose-response relationship between the number of cigarettes smoked daily and ischemic stroke among young men. Although complete smoking cessation is the goal, even smoking fewer cigarettes may reduce the risk of ischemic stroke in young men.

The findings suggest that acetylcholinesterase inhibitors such as donepezil have at best selective, modest effects on cognition in patients with PSP. In light of its deleterious effects on ADL/mobility, donepezil is not recommended for this patient population.

The authors utilized repetitive transcranial magnetic stimulation (rTMS) in 16 normal volunteers to investigate the role of the left dorsolateral prefrontal cortex (PFC) in analogic reasoning. rTMS over the left and right PFC, over the left motor cortex, and sham stimulation over the left PFC were administered during memory and analogic reasoning conditions. rTMS over the left PFC led to a significant reduction in response times only in the analogy condition without affecting accuracy. These results indicate that the left PFC is relevant for analogic reasoning and that rTMS applied to the PFC can speed up solution time.