Spinocerebellar ataxia types 1, 2, 3, and 6: disease severity and nonataxia symptoms.

OBJECTIVE: To identify factors that determine disease severity and clinical phenotype of the most common spinocerebellar ataxias (SCAs), we studied 526 patients with SCA1, SCA2, SCA3. or SCA6. METHODS: To measure the severity of ataxia we used the Scale for the Assessment and Rating of Ataxia (SARA). In addition, nonataxia symptoms were assessed with the Inventory of Non-Ataxia Symptoms (INAS). The INAS count denotes the number of nonataxia symptoms in each patient. RESULTS: An analysis of covariance with SARA score as dependent variable and repeat lengths of the expanded and normal allele, age at onset, and disease duration as independent variables led to multivariate models that explained 60.4% of the SARA score variance in SCA1, 45.4% in SCA2, 46.8% in SCA3, and 33.7% in SCA6. In SCA1, SCA2, and SCA3, SARA was mainly determined by repeat length of the expanded allele, age at onset, and disease duration. The only factors determining the SARA score in SCA6 were age at onset and disease duration. The INAS count was 5.0 +/- 2.3 in SCA1, 4.6 +/- 2.2 in SCA2, 5.2 +/- 2.5 in SCA3, and 2.0 +/- 1.7 in SCA6. In SCA1, SCA2, and SCA3, SARA score and disease duration were the strongest predictors of the INAS count. In SCA6, only age at onset and disease duration had an effect on the INAS count. CONCLUSIONS: Our study suggests that spinocerebellar ataxia (SCA) 1, SCA2, and SCA3 share a number of common biologic properties, whereas SCA6 is distinct in that its phenotype is more determined by age than by disease-related factors

Functional impairment in patients with myotonic dystrophy type 1 can be assessed by an ataxia rating scale (SARA)

Myotonic dystrophy type 1 (DM1) is not characterised by ataxia per se; however, DM1 and ataxia patients show similar disturbances in movement coordination often experiencing walking and balance difficulties, although caused by different underlying pathologies. This study aims to investigate the use of a scale previously described for the assessment and rating of ataxia (SARA) with the hypothesis that it could have utility in DM1 patients as a measure of disease severity and risk of falling. Data from 54 DM1 patients were pulled from the PHENO-DM1 natural history study for analysis. Mean SARA score in the DM1 population was 5.45 relative to the maximum score of eight. A flooring effect (score 0) was observed in mild cases within the sample. Inter-rater and test–retest reliability was high with intraclass coefficients (ICC) of 0.983 and 1.00, respectively. Internal consistency was acceptable as indicated by a Cronbach’s alpha of 0.761. Component analysis revealed two principle components. SARA correlated with: (1) all measures of muscle function tested, including quantitative muscle testing of ankle dorsiflexion (r = −0.584*), the 6 min walk test (r = −0.739*), 10 m walk test (r = 0.741*), and the nine hole peg test (r = 0.602*) and (2) measures of disease severity/burden, such as MIRS (r = 0.718*), MDHI (r = 0.483*), and DM1-Activ (r = −0.749*) (*p < 0.001). The SARA score was predicted by an interaction between modal CTG repeat length and age at sampling (r = 0.678, p = 0.003). A score of eight or above predicted the use of a walking aid with a sensitivity of 100% and a specificity of 85.7%. We suggest that further research is warranted to ascertain whether SARA or components of SARA are useful outcome measures for clinical trials in DM1. As a tool, it can be used for gathering information about disease severity/burden and helping to identify patients in need of a walking aid, and can potentially be applied in both research and healthcare settings

The glocalised telenovela as a space for possible identifications for diaspora girls in Northern Belgium: an audience cum content analysis of Sara

Because research on glocalised telenovelas in Western Europe is absent in literature and telenovelas seem highly popular among diaspora girls from Moroccan descents living in Northern Belgium, this paper studies the embedded themes and identification possibilities of telenovelas and explores its thematic interest and meanings for diaspora girls. By means of an audience cum content analysis on the case study Sara, text and audience are combined. Sara is predominantly a ‘Cinderella story’ with a clear ‘love’ and ‘class and social mobility’ discourse where emotional identification is triggered through different parameters. Belgian girls from Moroccan descent mainly watch the Sara for reasons of entertainment and escapism. They negotiate between lived and telenovela-created experiences and consequently formulate aspirations and dreams for future partners, gender roles, careers and (family) life

When cultural modifications are taken into account: Ibsen's a doll's house on Iranian screen

How cross-cultural communications terminate in imperfect understanding can arguably be rendered by critical awareness about culturally differentiating conceptions in the interacting communities. This study aims to shed light on how an awakening message of a Western literary work can achieve a functional realization in an Eastern society via domestication of the cultural mnemonics. To this end, the present paper explored how Henrik Ibsen's A Doll's House was introduced to Persian culture as a movie entitled Sara by Dariush Mehrjui mediated by apprehending the intended function of the original work and cultural capital of the target community. Meticulous comparative analysis of A Doll's House and Sara yielded that the given successful 'transmission' was materialized through two levels of macro contextual and micro cultural domestications. Generic and thematic transformations at the former level and various strata of cultural turns at the latter have conceivably lent verisimilitude to Sara and its proximity to the cultural schema of Iranian audience, through which Nora in Norway could be 'translated' to Sara in Iran to enlighten both audience. It is hoped the arguments of this research can offer some critical points for both proponents and opponents of the debates on cultural turn in generic translation. DOI: 10.5901/mjss.2015.v6n5s2p21

Population structures in the SARA and SARB reference collections of Salmonella enterica according to MLST, MLEE and microarray hybridization

In the 1980's and 1990's, population genetic analyses based on Multilocus Enzyme Electrophoresis (MLEE) provided an initial overview of the genetic diversity of multiple bacterial species, including Salmonella enterica. The genetic diversity within S. enterica subspecies enterica according to MLEE is represented by the SARA and SARB reference collections, each consisting of 72 isolates, which have been extensively used for comparative analyses. MLEE has subsequently been replaced by Multilocus Sequence Typing (MLST). Our initial MLST results indicated that some strains within the SARB collection differed from their published descriptions. We therefore performed MLST on four versions of the SARB collection from different sources and one collection of SARA, and found that multiple isolates in SARB and SARA differ in serovar from their original description, and other SARB isolates differed between different sources. Comparisons with a global MLST database allowed a plausible reconstruction of the serovars of the original collection. MLEE, MLST and microarrays were largely concordant at recognizing closely related strains. MLST was particularly effective at recognizing discrete population genetic groupings while the two other methods provided hints of higher order relationships. However, quantitative pair-wise phylogenetic distances differed considerably between all three methods. Our results provide a translation dictionary from MLEE to MLST for the extant SARA and SARB collections which can facilitate genomic comparisons based on archival insights from MLEE

UNBALANCED BEHAVIOR OF THE CHARACTER REBECCA IN THE FILM THE ROOMMATE

The Roommate adalah film yang disutradarai oleh Christian E.Christiansen.The Roommate menceritakan tentang seorang mahasiswi bernama Sara Mathew yang memiliki teman sekamar yang obsesif bernama Rebecca. Kedekatan Rebecca dengan Sara semakin erat yang kemudian berubah menjadi sebuah obsesi dan membuat Rebecca melakukan hal-hal yang buruk , bahkan melakukan kekerasan fisik terhadap orang-orang disekitar Sara. Dalam penelitian ini penulis akan mendeskripsikan unsur intrinsik dan ekstrinsik dari film The Roommate. Tujuan dari penilitian ini adalah masalah psikologi yang dialami oleh karakter Rebecca dalam film The Roommate menggunakan Teori Psikoanalisis: Id, Ego, and Superego dari Sigmund Freud. Penulis akan menggunakan pendekatan eksponensial untuk mendeskripsikan unsur intrinsik dan pendekatan psikologi untuk mendeskripsikan unsur ekstrinsik film. Metode yang digunakan untuk mengumpulkan data ada penelitian pustaka. Hasil dari penelitian ini adalah bahwa tiga unsur Id, Ego, dan Superego dalam diri Rebecca berkembang secara tidak seimbang. Ketidakseimbangan organisasi dari Id, Ego, dan Superego menghasilkan perilaku yang tidak seimbang yang mengindikasikan bahwa Rebecca memiliki masalah psikologi

The Classroom

Postcard from Sara Peterson, during the Linfield College Semester Abroad Program at Telemark University in Bø, Norwa

One Month In

Postcard from Sara Schurter, during the Linfield College Semester Abroad Program at James Cook University in Cairns, Australi

Caravan At The Edge Of The World

During a windswept caravan holiday with Dean’s parents Sara has to decide between true love and her place at Universit