Visual signs and symptoms in patients with the visual variant of Alzheimer disease.

BACKGROUND: Prominent visual symptoms can present in the visual variant of Alzheimer's disease (VVAD). Ophthalmologists have a significant role to play in the early diagnosis of VVAD. METHODS: We retrospectively reviewed the files of ten consecutive patients diagnosed with VVAD. All patients had a full neuro-ophthalmologic examination, a formal neurological and neuro-psychological testing, and cerebral MRI to confirm diagnosis. In addition, functional neuroimaging was obtained in seven patients. RESULTS: The common primary symptom at presentation with all patients was difficulty with near vision (reading difficulty n = 8, "visual blur" in near vision n = 2), and difficulty writing (n = 3). Following assessment, impaired reading and writing skills were evident in 9/10 and 8/10 patients respectively. Median distance visual acuity was 20/25 and at near the median visual acuity was J6. Partial homonymous visual field defect was detected in 80 % (8/10) of the patients. Color vision was impaired in all patients when tested with Ishihara pseudoisochromatic plates, but simple color naming was normal in 8/9 tested patients. Simultanagnosia was present in 8/10 patients. Vision dysfunction corresponded with cerebral MRI findings where parieto-occipital cortical atrophy was observed in all patients. PET scan (5 patients) or SPECT (2 patients) revealed parieto-occipital dysfunction (hypometabolism or hypoperfusion) in all 7 tested patients CONCLUSIONS: Visual difficulties are prominent in VVAD. Dyslexia, incomplete homonymous hemianopia, preserved color identification with abnormal color vision on Ishihara, and simultanagnosia were all symptoms observed frequently in this patient series. Ophthalmologists should be aware of the possibility of neurodegenerative disorders such as VVAD in patients with unexplained visual complaints, in particular reading difficulties

Macular dystrophy associated with the mitochondrial DNA A3243G mutation: pericentral pigment deposits or atrophy? Report of two cases and review of the literature.

BACKGROUND: The A3243G point mutation in mitochondrial DNA (mtDNA) is associated with MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes) and MIDD syndromes (maternally inherited diabetes and deafness). Both MELAS and MIDD patients can present with visual symptoms due to a retinopathy, sometimes before the genetic diagnosis is made. CASE PRESENTATION: Patient 1: 46 year-old woman with diabetes mellitus and hearing loss was referred for an unspecified maculopathy detected during screening evaluation for diabetic retinopathy. Visual acuity was 20/20 in both eyes. Fundus examination showed bilateral macular and peripapillary hyperpigmented/depigmented areas.Patient 2: 45 year-old woman was referred for recent vision loss in her left eye. History was remarkable for chronic fatigue, migraine and diffuse muscular pain. Visual acuity was 20/20 in her right eye and 20/30 in her left eye. Fundus exhibited several nummular perifoveal islands of retinal pigment epithelium atrophy and adjacent pale deposits in both eyes.Retinal anatomy was investigated with autofluorescence, retinal angiography and optical coherence tomography. Retinal function was assessed with automated static perimetry, full-field and multifocal electroretinography and electro-oculography. Genetic testing of mtDNA identified a point mutation at the locus 3243. CONCLUSION: Observation of RPE abnormalities in the context of suggestive systemic findings should prompt mtDNA testing

Cross-sectional study assessing the addition of contrast sensitivity to visual acuity when testing for fitness to drive.

The aim of this study is to quantify the importance of loss of contrast sensitivity (CS) and its relationship to loss of visual acuity (VA), driving restrictions and daytime, on-road driving evaluations in drivers aged 70+. A predictive cross-sectional study. Volunteer participants to a drivers' refresher course for adults aged 70+ delivered by the Swiss Automobile Club in western Switzerland from 2011 to 2013. 162 drivers, male and female, aged 70 years or older. We used a vision screener to estimate VA and the The Mars Letter Contrast Sensitivity Test to test CS. We asked drivers to report whether they found five driving restrictions useful for their condition; restrict driving to known roads, avoid driving on highways, avoid driving in the dark, avoid driving in dense traffic and avoid driving in fog. All participants also underwent a standardised on-road evaluation carried out by a driving instructor. Moderate to severe loss of CS for at least one eye was frequent (21.0% (95% CI 15.0% to 28.1%)) and often isolated from a loss of VA (11/162 cases had a VA ≥0.8 decimal and a CS of ≤1.5 log(CS); 6.8% (95% CI 3.4% to 11.8%)). Drivers were more likely (R <sup>2</sup> =0.116, P=0.004) to report a belief that self-imposed driving restrictions would be useful if they had reduced CS in at least one eye. Daytime evaluation of driving performance seems limited in its ability to correctly identify difficulties related to CS loss (VA: R <sup>2</sup> =0.004, P=0.454; CS: R <sup>2</sup> =0.006, P=0.332). CS loss is common for older drivers. Screening CS and referring for cataract surgery even in the absence of VA loss could help maintain mobility. Reduced CS and moderate reduction of VA were both poor predictors of daytime on-road driving performances in this research study

Posterior Cortical Atrophy: Review of the Recent Literature

Posterior cortical atrophy (PCA) is a group of neurodegenerative dementing disorders characterized by initial predominant visual complaints followed by progressive decline in cognitive functions. The visuospatial and visuoperceptual defects arise from the dysfunction of, respectively, the dorsal (occipito-parietal) and the ventral (occipito-temporal) streams. Clinical symptoms, results of neuropsychological examination, and findings of posterior cerebral atrophy and/or posterior hypoperfusion/hypometabolism contribute to the diagnosis. However, owing to the insidious onset of PCA and the non-specificity of initial symptoms, the diagnosis is often delayed. Specific etiologies include Alzheimer's disease, dementia with Lewy bodies, subcortical gliosis, corticobasal degeneration, and prion-associated diseases. Alzheimer's disease accounts for at least 80% of PCA cases. Recent research has concentrated on better defining the clinical presentation of PCA, improving neuroimaging analysis, testing new neuroimaging techniques, and developing biological measurements. Selected recent papers on PCA are reviewed in this articl

Quand les chercheurs font une recherche sur la recherche. Zoom sur les défis scientifiques et professionnels d’une expérience interculturelle

Au printemps 2005, un groupe de jeunes chercheuses et chercheur s’est créé afin d’établir un panorama des recherches en éducation interculturelle réalisées en Suisse ces dernières années. Les buts poursuivis étaient de : 1) de recenser les travaux accomplis dans ce champ d’investigation entre 1993 et 2006 ; 2) d’analyser les changements éventuels de paradigmes, de démarches, de populations, etc., au cours du temps et selon les diverses régions linguistiques suisses ; 3) de dynamiser, favoriser et développer, grâce à la diffusion des résultats, des relations intercantonales et internationales entre les chercheurs du domaine. La réalisation de ce panorama a donné lieu à un rapport1 présenté lors du colloque « L’éducation en contextes pluriculturels : la recherche entre bilan et prospectives » qui s’est tenu à Genève en juin 2007

Spectrum of digoxin-induced ocular toxicity: a case report and literature review

BACKGROUND: Digoxin intoxication results in predominantly digestive, cardiac and neurological symptoms. This case is outstanding in that the intoxication occurred in a nonagenarian and induced severe, extensively documented visual symptoms as well as dysphagia and proprioceptive illusions. Moreover, it went undiagnosed for a whole month despite close medical follow-up, illustrating the difficulty in recognizing drug-induced effects in a polymorbid patient. CASE PRESENTATION: Digoxin 0.25 mg qd for atrial fibrillation was prescribed to a 91-year-old woman with an estimated creatinine clearance of 18 ml/min. Over the following 2-3 weeks she developed nausea, vomiting and dysphagia, snowy and blurry vision, photopsia, dyschromatopsia, aggravated pre-existing formed visual hallucinations and proprioceptive illusions. She saw her family doctor twice and visited the eye clinic once until, 1 month after starting digoxin, she was admitted to the emergency room. Intoxication was confirmed by a serum digoxin level of 5.7 ng/ml (reference range 0.8-2 ng/ml). After stopping digoxin, general symptoms resolved in a few days, but visual complaints persisted. Examination by the ophthalmologist revealed decreased visual acuity in both eyes, 4/10 in the right eye (OD) and 5/10 in the left eye (OS), decreased color vision as demonstrated by a score of 1/13 in both eyes (OU) on Ishihara pseudoisochromatic plates, OS cataract, and dry age-related macular degeneration (ARMD). Computerized static perimetry showed non-specific diffuse alterations suggestive of either bilateral retinopathy or optic neuropathy. Full-field electroretinography (ERG) disclosed moderate diffuse rod and cone dysfunction and multifocal ERG revealed central loss of function OU. Visual symptoms progressively improved over the next 2 months, but multifocal ERG did not. The patient was finally discharged home after a 5 week hospital stay. CONCLUSION: This case is a reminder of a complication of digoxin treatment to be considered by any treating physician. If digoxin is prescribed in a vulnerable patient, close monitoring is mandatory. In general, when facing a new health problem in a polymorbid patient, it is crucial to elicit a complete history, with all recent drug changes and detailed complaints, and to include a drug adverse reaction in the differential diagnosis

La recherche empirique en education interculturelle en Suisse: comparaison entre la Suisse francophone et la Suisse germanophone

C’est aux chercheurs en éducation interculturelle et à leurs travaux que s’est intéressée l’étude rapportée ici : quelles sont les thématiques traitées, auprès de quelles populations, avec quelles méthodes? Le champ de l’éducation interculturelle s’est maintenant bien établi, institutionnellement dans des cursus de formation de plus en plus nombreux, mais aussi dans la communauté scientifique grâce par exemple à l’IAIE (International Association for Intercultural Education). En Suisse, un groupe de travail dédié à l’éducation interculturelle existe depuis le début des années 1980 au sein de la Société suisse de recherche en éducation. C’est de ce groupe de travail qu’émane la présente étude

La recherche suisse en éducation interculturelle. Panorama des recherches empiriques réalisées entre 1993 et 2006: Rapport final.

Le projet de réaliser un recensement des études suisses faites dans le domaine de l’éducation interculturelle durant ces 15 dernières années environ est né d’une volonté de réanimation du groupe de travail « Approches interculturelles » de la SSRE qui était inactif depuis plusieurs années. Avec ce recensement, le groupe de travail de la SSRE – qui s’est entre-temps nommé « Éducation interculturelle » – apporte une nouvelle pierre à la construction du champ de la recherche en éducation interculturelle en Suisse. Ce recensement fait suite à trois moments-clé de la vie de ce groupe de travail, qui avaient à chaque fois permis aux chercheurs1 suisses de faire le point sur leurs travaux : la parution en 1981 du livre collectif Etre migrant I (Gretler, Perret-Clermont, & Poglia, 1981), suivie en 1995 par le 2e volume, Être migrant II (Poglia, Perret-Clermont, Gretler, & Dasen, 1995), ainsi que la tenue à Berne en 1993 du colloque Inter-93 (Allemann-Ghionda, 1994)

Unsatisfactory outcomes in myasthenia gravis: influence by care providers

Myasthenia gravis (MG) can be difficult to treat despite an available therapeutic armamentarium. Our aim was to analyze the factors leading to unsatisfactory outcome (UO). To this end we used the Myasthenia Gravis Foundation of America classification system. Forty one patients with autoimmune MG were followed prospectively from January 2003 to December 2007. Outcomes were assessed throughout follow-up and at a final visit. ‘Unchanged', ‘worse', ‘exacerbation' and ‘died of MG' post-intervention status were considered UOs. During follow-up, UO rates reached 54% and were related to undertreatment (41%), poor treatment compliance (23%), infections (23%), and adverse drug effects (13%). The UO rate at final study assessment was 20%. UO during follow-up was significantly (P=0.004) predictive of UOs at final assessment. When care was provided by neuromuscular (NM) specialists, patients had significantly better follow-up scores (P=0.01). At final assessment UO rates were 7% and significantly better in patients treated by NM specialists, compared to other physicians where UO rates reached 27%. UO was a frequent finding occurring in more than half our patients during follow-up. Nearly two-thirds of the UOs could have been prevented by appropriate therapeutic adjustments and improved compliance. The differential UO rates at follow-up, their dependency on the degree to which the management was specialized and their correlation with final outcomes suggest that specialized MG care improves outcome