Background: Hereditary cerebellar ataxias (HCA) and hereditary spastic paraplegias (HSP) are two groups of neurodegenerative disorders that usually present with progressive gait impairment, often leading to permanent disability. Advances in genetic research in the last decades have improved their diagnosis and brought new possibilities for prevention and future treatments. Still, there is great uncertainty regarding their global epidemiology. Summary: Our objective was to assess the global distribution and prevalence of HCA and HSP by a systematic review and meta-analysis of prevalence studies. The MEDLINE, ISI Web of Science and Scopus databases were searched (1983-2013) for studies performed in well-defined populations and geographical regions. Two independent reviewers assessed the studies and extracted data and predefined methodological parameters. Overall, 22 studies were included, reporting on 14,539 patients from 16 countries. Multisource population-based studies yielded higher prevalence values than studies based primarily on hospitals or genetic centres. The prevalence range of dominant HCA was 0.0-5.6/10⁵, with an average of 2.7/10⁵ (1.5-4.0/10⁵). Spinocerebellar ataxia type 3 (SCA3)/Machado-Joseph disease was the most common dominant ataxia, followed by SCA2 and SCA6. The autosomal recessive (AR) HCA (AR-HCA) prevalence range was 0.0-7.2/10⁵, the average being 3.3/10⁵ (1.8-4.9/10⁵). Friedreich ataxia was the most frequent AR-HCA, followed by ataxia with oculomotor apraxia or ataxia-telangiectasia. The prevalence of autosomal dominant (AD) HSP (AD-HSP) ranged from 0.5 to 5.5/10⁵ and that of AR-HSP from 0.0 to 5.3/10⁵, with pooled averages of 1.8/10⁵ (95% CI: 1.0-2.7/10⁵) and 1.8/10⁵ (95% CI: 1.0-2.6/10⁵), respectively. The most common AD-HSP form in every population was spastic paraplegia, autosomal dominant, type 4 (SPG4), followed by SPG3A, while SPG11 was the most frequent AR-HSP, followed by SPG15. In population-based studies, the number of families without genetic diagnosis after systematic testing ranged from 33 to 92% in the AD-HCA group, and was 40-46% in the AR-HCA, 45-67% in the AD-HSP and 71-82% in the AR-HSP groups. Key Messages: Highly variable prevalence values for HCA and HSP are reported across the world. This variation reflects the different genetic make-up of the populations, but also methodological heterogeneity. Large areas of the world remain without prevalence studies. From the available data, we estimated that around 1:10,000 people are affected by HCA or HSP. In spite of advances in genetic research, most families in population-based series remain without identified genetic mutation after extensive testing.

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Prospective treatment of aortic stenosis with rosuvastatin by targeting serum LDL slowed the hemodynamic progression of aortic stenosis. This is the first prospective study that shows a positive effect of statin therapy for this disease process. (Rosuvastatin Affecting Aortic Valve Endothelium; http://www.clinicaltrials.gov/ct/show/NCT00114491?order = 1; NCT0014491).

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MULTIPLE SCLEROSIS MSJ JOURNAL1258 journals.sagepub.com/home/msj BackgroundCognitive impairment (CI) is known to be present in all stages of multiple sclerosis (MS); however, the prevalence estimates vary considerably between studies, ranging from 40% to 65%. 1 The profile of CI in the overall MS population is now relatively well known, involving mainly complex attention, information processing speed, episodic memory, and executive functions. 1,2 Therefore, brief neuropsychological batteries for MS 3 and newly developed assessment tools 4 mainly focus on the assessment of these functions. However, few studies investigated the differences in the prevalence and profile of CI between the different MS disease subtypes, providing heterogeneous results. [5][6][7][8][9] Many of these studies included small clinical samples and focused mainly on relapsing remitting (RR) or progressive forms. Moreover, the association of CI with several clinical features, such as physical disability, sex, and disease duration, is not well established, since inconsistent results have been reported in the literature. [10][11][12][13] The heterogeneity of the published literature could be, at least in part, attributable to small sample size and dissimilarities in the clinical characteristics of the studies' samples. Exploring the independent effects of age, physical disability, disease duration, and disease subtype could prove central to provide a better understanding of the potential role and interaction of cognitive reserve, brain aging, and disease severity for determining CI in MS. The severity of impairment and the number of involved domains were significantly higher in SP and primary progressive multiple sclerosis (PPMS) than in CIS and RR. In multivariable logistic regression analysis, the presence of CI was significantly associated with higher Expanded Disability Status Scale (EDSS) and older age. Conclusion: CI is present in all MS subtypes since the clinical onset and its frequency is increased in the progressive forms, but these differences seem to be more associated with patient age and physical disability than to disease subtype per se.

CARAT10 has high internal consistency and good concurrent validity, making it useful to compare groups in clinical studies.

Purposes To perform a comprehensive systematic review of prospective studies about frequency of adverse drug reactions (ADRs) occurring during hospitalization (ADRIn), including a thorough study quality assessment, meta‐analysis and heterogeneity evaluation. Methods Systematic review of several databases: Pubmed, EMBASE, CINAHL, Cochrane, ISI, International Pharmaceutical Abstracts, Scirus, NHS economic, and others, as well as manual search. Inclusion criteria were: prospective studies (assessing all patients before discharge, by a specialized team, at least once a week); with data about ADRs occurring during hospitalization, using WHO's or similar definition of ADR. Two independent reviewers assessed eligibility criteria, extracted data, and evaluated risk of bias. Results From 4139 studies initially found, 22 were included. Meta‐analysis indicate that ADRs may occur in 16.88% (CI95%: 13.56,20.21%) of patients during hospitalization; however, this estimate has to be viewed with caution because there was significant heterogeneity (I2 = 99%). The most significant moderators of heterogeneity were risk of bias, population, ward, and methodology for ADR identification. Low risk of bias studies adjusted for population (pediatric versus adult) had I2 = 0%. Conclusions These data are useful as a broad characterization of in‐hospital ADRs and their frequency. However, due to heterogeneity, our estimates are crude indicators. The wide variation in methodologies was one of the most important moderators of heterogeneity (even among studies using intensive monitoring). We suggest criteria to standardize methodologies and reduce the risk of bias. Copyright © 2012 John Wiley & Sons, Ltd.

BackgroundTen years after the first proposal, a consensus definition of healthcare-associated infection (HCAI) has not been reached, preventing the development of specific treatment recommendations. A systematic review of all definitions of HCAI used in clinical studies is made.MethodsThe search strategy focused on an HCAI definition. MEDLINE, SCOPUS and ISI Web of Knowledge were searched for articles published from earliest achievable data until November 2012. Abstracts from scientific meetings were searched for relevant abstracts along with a manual search of references from reports, earlier reviews and retrieved studies.ResultsThe search retrieved 49,405 references: 15,311 were duplicates and 33,828 were excluded based on title and abstract. Of the remaining 266, 43 met the inclusion criteria. The definition more frequently used was the initial proposed in 2002 - an infection present at hospital admission or within 48 hours of admission in patients that fulfilled any of the following criteria: received intravenous therapy at home, wound care or specialized nursing care in the previous 30 days; attended a hospital or hemodialysis clinic or received intravenous chemotherapy in the previous 30 days; were hospitalized in an acute care hospital for ≥2 days in the previous 90 days, resided in a nursing home or long-term care facility. Additional criteria founded in other studies were: immunosuppression, active or metastatic cancer, previous radiation therapy, transfer from another care facility, elderly or physically disabled persons who need healthcare, previous submission to invasive procedures, surgery performed in the last 180 days, family member with a multi-drug resistant microorganism and recent treatment with antibiotics.ConclusionsBased on the evidence gathered we conclude that the definition initially proposed is widely accepted. In a future revision, recent invasive procedures, hospitalization in the last year or previous antibiotic treatment should be considered for inclusion in the definition. The role of immunosuppression in the definition of HCAI still requires ongoing discussion.