Extension of 3DVAR to 4DVAR: Implementation of 4DVAR at the Meteorological Service of Canada

On 15 March 2005, the Meteorological Service of Canada (MSC) proceeded to the implementation of a four-dimensional variational data assimilation (4DVAR) system, which led to significant improvements in the quality of global forecasts. This paper describes the different elements of MSC’s 4DVAR assimilation system, discusses some issues encountered during the development, and reports on the overall results from the 4DVAR implementation tests. The 4DVAR system adopted an incremental approach with two outer iterations. The simplified model used in the minimization has a horizontal resolution of 170 km and its simplified physics includes vertical diffusion, surface drag, orographic blocking, stratiform condensation, and convection. One important element of the design is its modularity, which has permitted continued progress on the three-dimensional variational data assimilation (3DVAR) component (e.g., addition of new observation types) and the model (e.g., computational and numerical changes). This paper discusses some numerical problems that occur in the vicinity of the Poles where the semi-Lagrangian scheme becomes unstable when there is a simultaneous occurrence of converging meridians and strong wind gradients. These could be removed by filtering the winds in the zonal direction before they are used to estimate the upstream position in the semi-Lagrangian scheme. The results show improvements in all aspects of the forecasts over all regions. The impact is particularly significant in the Southern Hemisphere where 4DVAR is able to extract more information from satellite data. In the Northern Hemisphere, 4DVAR accepts more asynoptic data, in particular coming from profilers and aircrafts. The impact noted is also positive and the short-term forecasts are particularly improved over the west coast of North America. Finally, the dynamical consistency of the 4DVAR global analyses leads to a significant impact on regional forecasts. Experimentation has shown that regional forecasts initiated directly from a 4DVAR global analysis are improved with respect to the regional forecasts resulting from the regional 3DVAR analysis

Spinocerebellar ataxia 27B: episodic symptoms and acetazolamide response in 34 patients

Ashton C et al report a retrospective multi-centre cohort of 34 patients from Canada, France, Austria and Australia with spinocerebellar ataxia 27B, describing the common feature of episodic ataxia and other episodic features, as well as the inefficacy of acetazolamide in these patients

Intronic FGF14 GAA repeat expansions are a common cause of ataxia syndromes with neuropathy and bilateral vestibulopathy

BACKGROUND: Intronic GAA repeat expansions in the fibroblast growth factor 14 gene (FGF14) have recently been identified as a common cause of ataxia with potential phenotypic overlap with RFC1-related cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS). Our objective was to report on the frequency of intronic FGF14 GAA repeat expansions in patients with an unexplained CANVAS-like phenotype. METHODS: We recruited 45 patients negative for biallelic RFC1 repeat expansions with a combination of cerebellar ataxia plus peripheral neuropathy and/or bilateral vestibulopathy (BVP), and genotyped the FGF14 repeat locus. Phenotypic features of GAA-FGF14-positive versus GAA-FGF14-negative patients were compared. RESULTS: Frequency of FGF14 GAA repeat expansions was 38% (17/45) in the entire cohort, 38% (5/13) in the subgroup with cerebellar ataxia plus polyneuropathy, 43% (9/21) in the subgroup with cerebellar ataxia plus BVP and 27% (3/11) in patients with all three features. BVP was observed in 75% (12/16) of GAA-FGF14-positive patients. Polyneuropathy was at most mild and of mixed sensorimotor type in six of eight GAA-FGF14-positive patients. Family history of ataxia (59% vs 15%; p=0.007) was significantly more frequent and permanent cerebellar dysarthria (12% vs 54%; p=0.009) significantly less frequent in GAA-FGF14-positive than in GAA-FGF14-negative patients. Age at onset was inversely correlated to the size of the repeat expansion (Pearson's r, -0.67; R2=0.45; p=0.0031). CONCLUSIONS: GAA-FGF14-related disease is a common cause of cerebellar ataxia with polyneuropathy and/or BVP, and should be included in the differential diagnosis of RFC1 CANVAS and disease spectrum

Frequency of GAA-FGF14 Ataxia in a Large Cohort of Brazilian Patients With Unsolved Adult-Onset Cerebellar Ataxia

OBJECTIVES: Intronic FGF14 GAA repeat expansions have recently been found to be a common cause of hereditary ataxia (GAA-FGF14 ataxia; SCA27B). The global epidemiology and regional prevalence of this newly reported disorder remain to be established. In this study, we investigated the frequency of GAA-FGF14 ataxia in a large cohort of Brazilian patients with unsolved adult-onset ataxia. METHODS: We recruited 93 index patients with genetically unsolved adult-onset ataxia despite extensive genetic investigation and genotyped the FGF14 repeat locus. Patients were recruited across 4 different regions of Brazil. RESULTS: Of the 93 index patients, 8 (9%) carried an FGF14 (GAA)≥250 expansion. The expansion was also identified in 1 affected relative. Seven patients were of European descent, 1 was of African descent, and 1was of admixed American ancestry. One patient carrying a (GAA)376 expansion developed ataxia at age 28 years, confirming that GAA-FGF14 ataxia can occur before the age of 30 years. One patient displayed episodic symptoms, while none had downbeat nystagmus. Cerebellar atrophy was observed on brain MRI in 7 of 8 patients (87%). DISCUSSION: Our results suggest that GAA-FGF14 ataxia is a common cause of adult-onset ataxia in the Brazilian population, although larger studies are needed to fully define its epidemiology

Josée Pellerin : Gagner des vertiges tout à son aise / Farouk Kaspaules : Traces

"Two types of representation are discussed here simultaneously: the first, photographic, features the exterior of private residences while the other, in textual superposition, delivers clues to a scene unfolding inside this microcosm. The co-presence of image and text on the same surface generates a narrative relating to the secrets inherent in any domestic space. In order to stimulate the viewer's ability to produce narratives, the texts chosen go far beyond the usual status of legend and function as Polaroids, objective and unaffected snapshots. / Farouk Kaspaules' exhibition, entitled "Traces", focuses on the images and reality of the Arab population of the Marsh region and the Tigris and Euphrates region, extending all the way to Iraq from north in Kurdistan. The work of Kaspaules, a Canadian artist born in Iraq, a country he left in the mid-1970s for political reasons, plays the formal structural achievements of art with independent political content. The techniques used for this work stimulate us and give no graphic or easy answer. Each piece alludes to a moment of communion with the environment, highlighting moments of contemplation threatened by imminent danger. These images, executed and silkscreened on velum and paper, give the impression of having been subjected to the effect of several filters to evoke a feeling of human history that is fading, breaking up and erasing." -- Publisher's website