Movement Disorders in Prionopathies: A Systematic Review

Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed prionopathies. Timing of symptom and movement disorder onset were documented. Continuous variables were compared between two groups using the Wilcoxon rank sum test and between multiple groups using Kruskal–Wallis test. Categorical variables were compared using Fisher’s exact test. Results: A total of 324 cases were included in this analysis. Movement disorders were a common feature at the onset of symptoms in most prionopathies. Gait ataxia was present in more than half of cases in all types of prionopathies. The prevalence of limb ataxia (20%) and myoclonus (24%) was lower in Gerstmann–Sträussler–Scheinker disease compared to other prionopathies (p ≤ 0.004). Myoclonus was common but often a later feature in sporadic Creutzfeldt–Jakob disease (2 months before death). Chorea was uncommon but disproportionately prevalent in variant Creutzfeldt–Jakob disease (30% of cases; p < 0.001). In genetic Creutzfeldt–Jakob disease, E200K PRNP carriers exhibited gait and limb ataxia more often when compared to other mutation carriers. Discussion: Movement disorders are differentially present in the course of the various prionopathies. The movement phenomenology and appearance are associated with the type of prion disease and the PRNP genotype and likely reflect the underlying pattern of neurodegeneration. Reliance on myoclonus as a diagnostic feature of sporadic Creutzfeldt–Jakob disease may delay its recognition given its relatively late appearance in the disease course

Get With The Guidelines®–Stroke performance indicators in patients with transient ischemic attack

ABSTRACT Get With The Guidelines®–Stroke is an in-hospital program for improving stroke care by promoting adherence to scientific guidelines. Of the patients with transient ischemic attack (TIA), 10-15% have a stroke within three months, and many patients do not receive the recommended interventions to prevent this outcome. Objective: The goal of this study was to assess the adherence to stroke quality indicators in patients with TIA. Methods: This retrospective observational study evaluated consecutive patients admitted to a primary stroke center with TIA or acute ischemic stroke (AIS) from August 2008 to December 2013. Six quality indicators applicable to both TIA and AIS were analyzed and compared between groups. Results: A total of 357 patients with TIA and 787 patients with AIS were evaluated. Antithrombotic medication use within 48 hours of admission, discharge use of anticoagulation for atrial fibrillation and counseling for smoking cessation were similar between groups. In the TIA group, discharge use of antithrombotic medication (95% versus 98%; p = 0.01), lipid-lowering treatment (57.7% versus 64.1%; p < 0.01) and stroke education (56.5% versus 74.5%; p < 0.01) were all less frequently observed compared with patients with AIS. Conclusions: The adherence to some of the Get With The Guidelines®–Stroke quality indicators was lower in patients with TIA than in patients with AIS. Measures should be undertaken to reinforce the importance of such clinical interventions in patients with TIA