We propose diagnostic criteria for Susac syndrome that may help both experts and physicians not familiar with Susac syndrome to make a correct diagnosis and to prevent delayed treatment initiation.

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Susac syndrome, a rare but probably underdiagnosed combination of encephalopathy, hearing loss, and visual deficits due to branch retinal artery occlusion of unknown aetiology has to be considered as differential diagnosis in various conditions. Particularly, differentiation from multiple sclerosis is often challenging since both clinical presentation and diagnostic findings may overlap. Optical coherence tomography is a powerful and easy to perform diagnostic tool to analyse the morphological integrity of retinal structures and is increasingly established to depict characteristic patterns of retinal pathology in multiple sclerosis. Against this background we hypothesised that differential patterns of retinal pathology facilitate a reliable differentiation between Susac syndrome and multiple sclerosis. In this multicenter cross-sectional observational study optical coherence tomography was performed in nine patients with a definite diagnosis of Susac syndrome. Data were compared with age-, sex-, and disease duration-matched relapsing remitting multiple sclerosis patients with and without a history of optic neuritis, and with healthy controls. Using generalised estimating equation models, Susac patients showed a significant reduction in either or both retinal nerve fibre layer thickness and total macular volume in comparison to both healthy controls and relapsing remitting multiple sclerosis patients. However, in contrast to the multiple sclerosis patients this reduction was not distributed over the entire scanning area but showed a distinct sectorial loss especially in the macular measurements. We therefore conclude that patients with Susac syndrome show distinct abnormalities in optical coherence tomography in comparison to multiple sclerosis patients. These findings recommend optical coherence tomography as a promising tool for differentiating Susac syndrome from MS.

AimsTo evaluate the relative efficiencies of five Internet-based digital and three paper-based scientific surveys and to estimate the costs for different-sized cohorts.MethodsInvitations to participate in a survey were distributed via e-mail to employees of two university hospitals (E1 and E2) and to members of a medical association (E3), as a link placed in a special text on the municipal homepage regularly read by the administrative employees of two cities (H1 and H2), and paper-based to workers at an automobile enterprise (P1) and college (P2) and senior (P3) students. The main parameters analyzed included the numbers of invited and actual participants, and the time and cost to complete the survey. Statistical analysis was descriptive, except for the Kruskal-Wallis-H-test, which was used to compare the three recruitment methods. Cost efficiencies were compared and extrapolated to different-sized cohorts.ResultsThe ratios of completely answered questionnaires to distributed questionnaires were between 81.5% (E1) and 97.4% (P2). Between 6.4% (P1) and 57.0% (P2) of the invited participants completely answered the questionnaires. The costs per completely answered questionnaire were $0.57–$1.41 (E1–3), $1.70 and $0.80 for H1 and H2, respectively, and $3.36–$4.21 (P1–3). Based on our results, electronic surveys with 10, 20, 30, or 42 questions would be estimated to be most cost (and time) efficient if more than 101.6–225.9 (128.2–391.7), 139.8–229.2 (93.8–193.6), 165.8–230.6 (68.7–115.7), or 188.2–231.5 (44.4–72.7) participants were required, respectively.ConclusionsThe study efficiency depended on the technical modalities of the survey methods and engagement of the participants. Depending on our study design, our results suggest that in similar projects that will certainly have more than two to three hundred required participants, the most efficient way of conducting a questionnaire-based survey is likely via the Internet with a digital questionnaire, specifically via a centralized e-mail.

Emotional items were revealed to be the most relevant factors influencing the willingness to donate cornea postmortem, which may be counteracted by means of public education. The relatively low willingness among the medical staff contrasts with previous observations in a professional ophthalmologic society.