Inner Retinal Dysfunction in the Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.

The autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is associated with structural retinal abnormalities either directly visible on funduscopy or revealed by optical coherence tomography (OCT). Most patients with ARSACS have a whitish peripapillary appearance corresponding to a thickening of the peripapillary retinal nerve fiber layer. OCT has also shown an absence of the physiological foveal depression. Abnormal electroretinography (ERG) has previously been reported in only two cases, without further details. This report describes a patient with ARSACS in whom careful full-field ERG revealed dysfunction of the retinal On- bipolar cells with sparing of photoreceptor function. This is the first report of inner retinal dysfunction in ARSACS

Vers une typologie des dispositifs hybrides de formation en enseignement supérieur

International audienceAssessing the effects of hybrid system requires a descriptive framework to understand and differentiate their effects. In this perspective, this article proposes a typology of hybrid system from their educational, organizational and material characteristics (modalities of articulation of presential and distant phases, teaching approaches, human support, forms of media and mediation related to the use of a techno-environment, degree of openness).L'évaluation des effets des dispositifs hybrides de formation en enseignement supérieur nécessite un cadre descriptif pour en comprendre les effets différenciés. Dans cette perspective, le présent article propose d'établir une typologie des dispositifs hybrides de formation à partir de leurs caractéristiques pédagogiques, organisationnelles et matérielles (modalités d'articulation des phases présentielles et distantes, accompagnement humain, formes de médiatisation et médiation liées à l'utilisation d'un environnement technopédagogique, degré d'ouverture). Il rend également compte, d'un point de vue méthodologique, de la manière dont les chercheurs investis dans le projet européen HySup (2009-12) ont progressivement élaboré ce cadre et en ont fait une première expérimentation

Neuro-ophthalmologic manifestations of rheumatologic and associated disorders

Inflammatory diseases affecting the eye, the orbital content, or the endocranium are various. The clinical or radiologic presentation are multiple and their diagnosis can be a real challenge. Most relevant studies or case reports on systemic lupus erythematosus, Wegener's granulomatosis, Churg-Strauss syndrome, giant cell arteritis, Behcet's disease, Takayasu's arteritis, relapsing polychondritis, or inflammatory pseudotumor of the orbit are summarized in the present review

Hallucinations et illusions visuelles, des symptomes souvent meconnus du praticien. [Visual hallucinations and illusions, symptoms frequently misdiagnosed by the practitioner]

INTRODUCTION: Visual hallucinations or illusions are not a rare symptom. However, they are often unrecognized. Unawareness of the meaning of these symptoms often mislead both the patient and his physician. PURPOSE: To define and describe the types of visual illusions and hallucinations which can be commonly encountered in neuro-ophthalmological practice. METHODS: Overview article. RESULTS: Hallucinations are a perception not based on sensory input, whereas illusions are a misinterpretation of a correct sensory input. Both phenomenon can be due to medication or drug, or to an altered mental status. Visual hallucinations can be formed (objects, people) or unformed (light, geometric figures). They can be generated either by a lesion on the antechiasmatic pathway, by a seizure phenomenon, by a migrainous phenomenon, or by a release phenomenon secondary to visual differentiation. Investigations will be directed towards a retinopathy, an optic neuropathy, a chiasmal or retrochiasmal lesion, or a bilateral antechiasmal lesion (Charles Bonnet syndrome). Visual illusions include meta-morphopsias, micro- macropsias, polyopia, palinopsia (visual perseveration), achromatopsia, Pulfrich phenomenon, or subjective vertical deviation. Illusions can be due to lesions of the retina, the optic nerve, the visual cortex (primary or associative), or the graviceptive pathways. CONCLUSIONS: As most patients do not spontaneously mention their symptoms, history taking is essential. The first step is to rule out medication or an altered mental status as the possible cause of these symptoms. Then, careful visual function examination should provide a good insight in the location of the lesion