Diagnostic criteria for Susac syndrome.

BACKGROUND: Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome. METHOD: The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012. RESULTS: Integrating the clinical presentation and paraclinical findings, we propose formal criteria and recommend a diagnostic workup to facilitate the diagnosis of Susac syndrome. More than 90% of the cases in the literature fulfilled the proposed criteria for probable or definite Susac syndrome. We surmise that more patients could have been diagnosed with the recommended diagnostic workup. CONCLUSIONS: 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.

Efficiencies of Internet-based digital and paper-based scientific surveys and the estimated costs and time for different-sized cohorts.

AIMS: To evaluate the relative efficiencies of five Internet-based digital and three paper-based scientific surveys and to estimate the costs for different-sized cohorts. METHODS: Invitations 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. RESULTS: The 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. CONCLUSIONS: The 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.

Bacterial spectrum and antimicrobial susceptibility patterns in acquired and connatal lacrimal duct stenosis.

PURPOSE: (1) To determine the current bacteriological spectrum in connatal and acquired lacrimal duct obstruction (cLDO and aLDO, respectively) and (2) to analyze the antimicrobial susceptibility patterns of the recovered isolates. MATERIALS AND METHODS: In a prospective study, 463 samples (30% bilateral LDO) were obtained from the lacrimal ducts of 132 infants and 192 adult patients with symptomatic LDO between 2007 and 2012 at a tertiary eye-care center. The samples were cultured for aerobic and anaerobic bacteria, which were subsequently identified using standard microbiological techniques. Antimicrobial susceptibility testing was performed for each isolate using the disk diffusion method. Data were analyzed using SPSS and chi-square test for significance testing. RESULTS: (1) Among 463 samples investigated, 333 samples were positive, i.e. at least one bacterial isolate was recovered. A total of 72% were recovered (97% of samples from children and 56% of samples from adults), yielding a total of 654 bacterial isolates. Co-colonization with up to five different bacterial species was observed in a large proportion of the samples from children (87%), but in only 20% of those from adults and with a maximum of three different bacteria. Gram-positive bacteria were identified in 72% of the positive samples in both aLDO and cLDO. The most common Gram-positive species in cLDO was Streptococcus pneumoniae (29%), while that in cLDO was Staphylococcus aureus (60%). The most prevalent Gram-negative species were Moraxella catarrhalis (8%) and Haemophilus influenzae (9%) in cLDO and Pseudomonas aeruginosa in aLDO (12%). (2) Susceptibility testing revealed chloramphenicol to be the most active antibiotic with resistance rates of 3% in cLDO and 6% in aLDO, followed by ciprofloxacin (1% and 6%). Erythromycin and gentamicin were the least active of all, with resistances of 41% and 22%, respectively, in cLDO, and 23% and 11% in aLDO. CONCLUSIONS: Bacterial colonization occurs regularly in LDO, with Gram-positive bacteria being found in 97% of cLDO samples and 56% of aLDO samples. A remarkable number of different species were found to co-colonize in cLDO. The most common bacteria in LDO are highly susceptible in vitro to chloramphenicol and ciprofloxacin.

Pterygium recurrence, astigmatism and visual acuity following bare-sclera excision and conjunctival autograft with or without additional phototherapeutic keratectomy.

BACKGROUND: Treatment outcome in patients with pterygium following bare-sclera excision and conjunctival autograft (CAG) with and without phototherapeutic keratectomy (PTK). METHODS: Retrospective comparative analysis of 81 eyes, with primary and recurrent pterygia, that were analyzed for recurrence, best-corrected visual acuity (BCVA) and astigmatism in primary (P1 without PTK, P2 with PTK) and recurrent pterygia (R1 without PTK, R2 with PTK). BCVA and astigmatism were compared in patients with simple CAG alone (group I) or in combination with PTK (group II). RESULTS: Recurrence rates were 4.7, 11.6, 16.2, 23.2 and 32.5% at 3, 6, 12, 24 and >24 months (P1), 7.1% at >24 months (P2). Recurrence rates were 5.3, 10.5, 21.1, 21.1 and 26.3% at 3, 6, 12, 24 and >24 months (R1) and 1 recurrence (7.7%) till month 24, and 3 (23.1%) thereafter (R2). BCVA increased from logarithm of the minimal angle of resolution 0.095 ± 0.141 (mean ± SD) at baseline to 0.066 ± 0.09 (group I), and from 0.090 ± 0.164 to 0.054 ± 0.124 (group II). Astigmatism decreased from -1.01 ± 0.90 dpt at baseline to -0.97 ± 1.24 dpt (group I), and from -1.19 ± 1.55 to -0.75 ± 0.87 dpt (group II). CONCLUSION: In comparison to CAG alone, additional excimer smoothing with PTK tends to increase BCVA and reduces recurrence rates in patients with primary pterygia.

Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS.

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.