Comparison of mid-age-onset and late-onset Huntington's disease in Finnish patients

The phenotype of juvenile Huntington's disease (HD) differs clearly from that of adult-onset HD, but information about differences between mid-age-onset HD and late-onset HD (LOHD) is scarce. A national cohort of 206 patients with adult-onset HD was identified using national registries and patient records. LOHD was defined as age >= 60 years at HD diagnosis. Genetic disease burden was assessed using CAG age product (CAP) score. LOHD comprised 25% of the adult-onset HD cohort giving a point prevalence of 2.38/100,000 in the Finnish population at least 60 years of age. The proportion of LOHD out of new HD diagnoses increased from 21% in 1991-2000 to 33% in 2001-2010. At the time of diagnosis, patients with LOHD had 10.4 units (95% CI 4.8-15.9; p = 0.0003) higher CAP scores, more severe motor impairment and slightly more severe functional impairment than that in patients with mid-age-onset HD. There was no difference in the rate of disease progression or survival between LOHD and mid-age-onset patients. The lifespans of deceased patients were shorter in mid-age-onset HD (p < 0.001) and LOHD (p = 0.002) than their life expectancies. Causes of death differed between the two patient groups (p = 0.025). LOHD comprises a quarter of Finnish HD patients and the proportion appears to be increasing. Our results did not reveal differences in the phenotype between mid-age-onset HD and LOHD, but prospective studies are needed

Seasonality of stroke in Finland

INTRODUCTION: The burden of stroke is increasing globally. Reports on seasonal variations in stroke occurrence are conflicting and long-term data are absent.METHODS: A retrospective cohort study using discharge registry data of all acute stroke admissions in Finland during 2004-2014 for patients ≥18 years age. A total of 97,018 admissions for ischemic stroke (IS) were included, 18,252 admissions for intracerebral hemorrhage (ICH) and 11,271 admissions for subarachnoid hemorrhage (SAH).RESULTS: The rate of IS admissions increased (p = 0.025) while SAH admission rate decreased (p < 0.0001), and ICH admission rate remained stable during the study period. The lowest seasonal admission rates were detected in summer and the highest in autumn for all stroke subtypes. Seasonal variation of IS was more pronounced in men (p = 0.020), while no sex difference was detected in ICH or SAH. The seasonal patterns of in-hospital mortality and length of stay (LOS) differed markedly by stroke subtype. Diagnoses of hypertension, atrial fibrillation, or diabetes showed no seasonality.CONCLUSIONS: All major stroke subtypes occurred most commonly in autumn and most infrequently in summer. Seasonality of in-hospital mortality and length of hospital stay appears to vary by stroke subtype. The seasonal pattern of ischemic stroke occurrence appears to have changed during the past decades. Key messages All major stroke subtypes (ischemic stroke, intracerebral hemorrhage, subarachnoid hemorrhage) occurred most frequently in autumn and least frequently in summer. Seasonal patterns of in-hospital mortality and length of stay differed markedly by stroke subtype. The seasonal pattern of ischemic stroke occurrence in Finland seems to have changed compared to 1982-1992.</div

Middle age self-report risk score predicts cognitive functioning and dementia in 20–40 years

Introduction: On the basis of the proxy measures of cognitive reserve, we created a middle age self-report risk score for early prediction of dementia.Methods: We used a longitudinal population-based study of 2602 individuals with a replication sample (N = 1011). Risk score at a mean age of 47 years was based on questions on educational and occupational attainments. Cognitive status at a mean age of 74 was determined via two validated telephone instruments.Results: The prevalence of dementia was 10% after a mean follow-up of 28 years. Risk score was a good predictor of dementia: area under the curve = 0.77 (95% confidence interval, 0.74–0.80). The risk of dementia decreased as a function of risk score from 36% to 0%. The risk score was significantly associated with cognition after a mean follow-up of 39 years in the replication sample.Discussion: Self-report risk score predicted cognitive functioning and dementia risk 20–40 years later.</h4

Middle age self-report risk score predicts cognitive functioning and dementia in 20-40 years

Peer reviewe

Arpp19 Promotes Myc and Cip2a Expression and Associates with Patient Relapse in Acute Myeloid Leukemia

Disease relapse from standard chemotherapy in acute myeloid leukemia (AML) is poorly understood. The importance of protein phosphatase 2A (PP2A) as an AML tumor suppressor is emerging. Therefore, here, we examined the potential role of endogenous PP2A inhibitor proteins as biomarkers predicting AML relapse in a standard patient population by using three independent patient materials: cohort1 (n = 80), cohort2 (n = 48) and The Cancer Genome Atlas Acute Myeloid Leukemia (TCGA LAML) dataset (n = 160). Out of the examined PP2A inhibitors (CIP2A, SET, PME1, ARPP19 and TIPRL), expression of ARPP19 mRNA was found to be independent of the current AML risk classification. Functionally, ARPP19 promoted AML cell viability and expression of oncoproteins MYC, CDK1, and CIP2A. Clinically, ARPP19 mRNA expression was significantly lower at diagnosis (p = 0.035) in patients whose disease did not relapse after standard chemotherapy. ARPP19 was an independent predictor for relapse both in univariable (p = 0.007) and in multivariable analyses (p = 0.0001) and gave additive information to EVI1 expression and risk group status (additive effect, p = 0.005). Low ARPP19 expression was also associated with better patient outcome in the TCGA LAML cohort (p = 0.019). In addition, in matched patient samples from diagnosis, remission and relapse phases, ARPP19 expression was associated with disease activity (p = 0.034), indicating its potential usefulness as a minimal residual disease (MRD) marker. Together, these data demonstrate the oncogenic function of ARPP19 in AML and its risk group independent role in predicting AML patient relapse tendency

Cross cultural adaptation and psychometric properties of the Finnish version of Western Ontario shoulder instability index (WOSI)

Background: Western Ontario shoulder instability index (WOSI) is a widely used disease-specific self-assessment measurement tool for patients with shoulder instability. The main aim of this study was to translate and cross culturally adapt the WOSI into Finnish language and to test its measurement properties. Methods: WOSI was translated in Finnish and adapted into an electronic user interface. 62 male patients with traumatic anteroinferior shoulder instability, programmed for stabilizing shoulder surgery, answered the questionnaire twice preoperatively (2 and 0 weeks), and twice postoperatively (3 and 12 months). Additional scoring tools, such as satisfaction to treatment outcome, subjective shoulder value (SSV), Oxford shoulder instability index (OSIS) and Constant score (CS), were used as comparators. The reliability, validity and responsiveness of WOSI were investigated through statistical analysis. Results: Preoperative test-retest results were available for 49 patients, and 54 patients were available at final follow up. The mean WOSI was 57.8 (SD 20.3), 70.4 (SD 18.9), and 85.9 (SD 15.5), at baseline, 3, and 12 months, respectively. There was a statistically significant mean improvement of 28.8 (SD 24.5) in WOSI between baseline and 12 months (p < 0.0001). The intraclass correlation coefficient for the preoperative WOSI was excellent 0.91. At 12 months WOSI had an excellent Pearson’s correlation coefficient both with SSV (0.69), OSIS (-0.81), and poor with CS (0.25) scores, confirming our a priori hypothesis. There were no detected floor nor ceiling effects for WOSI pre- or postoperatively. The calculated minimal detectable change was 9.2 and the estimated minimal clinically important difference 13.4 to 18.1. Conclusion: Finnish version of WOSI is a reliable and valid tool for assessing health state and improvement after operative treatment of shoulder instability in young male patients.publishedVersionPeer reviewe

Cross cultural adaptation and psychometric properties of the Finnish version of Western Ontario shoulder instability index (WOSI)

Background: Western Ontario shoulder instability index (WOSI) is a widely used disease-specific self-assessment measurement tool for patients with shoulder instability. The main aim of this study was to translate and cross culturally adapt the WOSI into Finnish language and to test its measurement properties.Methods: WOSI was translated in Finnish and adapted into an electronic user interface. 62 male patients with traumatic anteroinferior shoulder instability, programmed for stabilizing shoulder surgery, answered the questionnaire twice preoperatively (2 and 0 weeks), and twice postoperatively (3 and 12 months). Additional scoring tools, such as satisfaction to treatment outcome, subjective shoulder value (SSV), Oxford shoulder instability index (OSIS) and Constant score (CS), were used as comparators. The reliability, validity and responsiveness of WOSI were investigated through statistical analysis.Results: Preoperative test-retest results were available for 49 patients, and 54 patients were available at final follow up. The mean WOSI was 57.8 (SD 20.3), 70.4 (SD 18.9), and 85.9 (SD 15.5), at baseline, 3, and 12 months, respectively. There was a statistically significant mean improvement of 28.8 (SD 24.5) in WOSI between baseline and 12 months (p Conclusion: Finnish version of WOSI is a reliable and valid tool for assessing health state and improvement after operative treatment of shoulder instability in young male patients.</p

The governors of school markets? : Local education authorities, school choice and equity in Finland and Sweden

As one of the key elements of the Nordic welfare model, education systems are based on the idea of providing equal educational opportunities, regardless of gender, social class and geographic origin. Since the 1990s, Nordic welfare states have undergone a gradual but wide-ranging transformation towards a more market-based mode of public service delivery. Along this trajectory, the advent of school choice policy and the growing variation in the between-school achievement results have diversified the previously homogenous Nordic education systems. The aim of our paper is to analyse how Finnish and Swedish local education authorities comprehend and respond to the intertwinement of the market logic of school choice and the ideology of equality. The data consist of two sets of in-depth thematic interviews with staff from the local providers of education, municipal education authorities. The analysis discloses the ways in which national legislation has authorized municipal authorities to govern the provision of education.Peer reviewe