bluuebunny/crossref_metadata_2025_split huggingface dataset:
295 Clinical outcome in mog-antibody disease: a large single site cohort
Authors: Silvia Messina , Maciej Jurynczyk , R Woodhall Mark , George Tackley , Saleel Chandratre , Cheryl Hemingway , Patrick Waters , Isabel Leite Maria , Jacqueline Palace 
Published: October 2018

BackgroundMOG-antibody disease has been recently recognized as a demyelinating condition distinct from Multiple Sclerosis. Methods: A single-site study of 106 MOG-antibody positive patients (including a 63-patient incident cohort) describing the clinical phenotype and outcome.ResultsON was the most common onset presentation (51%) and was bilateral in about half. From the survival curve analysis in the incident cohort we estimated that 38% of patients relapsed within 18 months. The risk was lower in patients immunosuppressed for >3 months (p=0.013). Permanent motor disability (EDSS ≥6; limited walking distance) and visual acuity ≤6/36 in at least one eye occurred in 3.7% and 13.2% respectively after a median disease duration of 68 months (range 2–484). Permanent bladder dysfunction was present in 23.6% patients and around 2/3 of these had bowel dysfunction. 20% of males had permanent erectile dysfunction (44% of males with TM at onset).ConclusionThis is the largest single-centre cohort and the only incident cohort published, and shows that MOG-antibody disease is often relapsing but may be modified with 6–12 months of prednisolone. The prognosis is typically good, better than AQP4-antibody positive disease, but many patients are left with significant sphincter and erectile dysfunction and some with visual impairment.
THUR 071 Towards an integrated tool for pnes assessment
Authors: Eleanor d’Ersu , Talal Al-Mayhani , Meneka Sidhu 
Published: October 2018

IntroductionPsychogenic non-epileptic seizures (PNES) can present a real diagnostic challenge, with subsequent impact on the course of the disorder and its management. A number of studies have looked into predictive factors for PNES. However, no guidance is available for applying the findings of these studies in clinical practice.AimThe aim is to combine the most recognised discriminative features into an integrated clinical tool, ultimately, to improve the predictive value of the medical assessment of patients with possible PNES in a clinic setting. This can be invaluable in centres with limited access to video telemetry (VT).MethodsThe following criteria were employed and compared with the results of VT as gold standard:Patient comorbiditiesConversation analysisReview of system questionnaireIctal semiology and autonomic activationPostictal prolactin and lactateResults and conclusionThis QIP will assess the reliability and validity of various criteria in recognising PNES clinically. Based on our findings, an integrated tool will then be designed as an adjunct to the standard medical assessment in settings where VT is not readily available.
WED 148 Imaging findings in autoimmune encephalitis: a retrospective review
Authors: Jawad Fazal , Nouman Sadiq , Michael Bowen , Wali Muhammad Wasim , Vijay Sawlani , Saiju Jacob 
Published: October 2018

BackgroundNeuroimaging findings in autoimmune encephalitis may include normal MRI as well as limbic and extra-limbic involvement. Prompt recognition allows early immunosuppression and improved outcomes.MethodsRetrospective review of patients with Autoimmune encephalitis over the last 5 years.ResultsWe identified N-methyl-d-aspartate receptor (NMDAR) n=8, voltage gated potassium channel (VGKC) n=11, and leucine-rich glioma inactivated 1 (LGI1) n=11 encephalitis patients. 1/8 NMDAR encephalitis patients had abnormal MRI findings of T2 hyper intense signal in right anterior temporal and bilateral frontal and left insular lobe. DWI sequence showed restricted diffusion. 4/11 with VGKC encephalitis had positive MRI findings. Two patients had limbic involvement while one also had involvement of basal ganglia. Two patients had modest cerebral atrophy. Four patients had normal MRI findings. MRI scan was not available for three patients. 5/11 LGI1 encephalitis patients had bilateral mesial temporal lobe changes. In one patient, MRI could not be done due to permanent pacemaker. Five patients had normal MRI findings.ConclusionsIn our series, 33% patients had abnormal MRI findings consistent with a diagnosis of autoimmune encephalitis, which is lower than reported in literature. High clinical suspicion should lead to prompt diagnosis even in the absence of typical encephalitic changes on MRI.
WED 163 Monitoring for outcome assessment and trial recruitment of pwms
Authors: Kimberley Allen-Philbey , Ben Turner , Ozlem Yildiz , Joela Mathews , Gavin Giovannoni , Klaus Schmierer 
Published: October 2018

BackgroundThe time constraint of NHS care is difficult to reconcile with the comprehensive neurological assessment required to complete a mandatory EDSS for NHS England funded disease modifying treatment of people with multiple sclerosis (pwMS). We report our experience with parametric data collection in order to (i) inform DMT decisions and (ii) improve characterisation for recruitment of pwMS in clinical trials.MethodsWe implemented a battery consisting of the symbol digit modality test, 9-hole-peg-test, 25ft walking test and ABILHAND. pwMS were encouraged to sign up to the MS Register to record patient reported outcomes and ‘webEDSS’. Data from the MS Register and BartsMS Database were electronically linked.Results334 pwMS at different disease stages (EDSS 0–8.5) have been assessed at least annually. Of these, 64% had a relapsing and 36% a progressive disease course. Clinically meaningful data was obtained and displayed in an engaging portal for interpretation by the clinical team and pwMS.ConclusionIt’s feasible to implement multidimensional monitoring of pwMS to benefit (i) management of pwMS and (ii) trial recruitment, particularly for pwMS with EDSS >6.5. Longitudinal follow-up and data analysis is underway. Standardisation of a simple, regularly collected, dataset may improve performance assessment between centres.
WED 164 EQ-5D in the assessment of relapse recovery in ms
Authors: Wynford-Thomas Ray , Harding Katharine , Hawton Annie , Hirst Claire , Tallantyre Emma , Green Colin , Robertson Neil 
Published: October 2018

Rationale50% of multiple sclerosis (MS) relapses result in permanent disability. Modelling recovery from relapse enables cost-effectiveness of interventions to be evaluated. The expanded disability status scale (EDSS) is cumbersome in clinical practice and may not reflect patient experience. The EQ-5D is a standardised measure of health status and a common currency used by agencies such as NICE.AimTo compare measurable recovery from relapse using EDSS and EQ-5D.MethodEDSS and EQ-5D-3L were collected during clinician defined relapse and at 2 months follow-up, in 89 patients identified in a regional rapid access clinic.ResultsMedian EDSS in relapse was 5.0 and 3.5 at follow-up; 67 (75%) patients experienced improvement in EDSS. The proportion of patients with EQ-5D scores of level 1 (fewest symptoms) increased in all 5 domains at 2 months, although anxiety/depression was the most resistant to improvement. Of those with EDSS improvement, only 49 (73%) reported improvement in their health state (EQ-5D).ConclusionEDSS improvement following relapse is not always accompanied by patient-reported improvement in health state (EQ-5D), which may be more effective in identifying ongoing health issues. Low-level discrepancies may also inform on the relative merit of EQ-5D versus EDSS in assessing recovery.
WED 165 The management of cognitive and behavioural symptoms in ms
Authors: Gloria Onwuneme , TA Hayat Tayyib , A Hosseini Akram 
Published: October 2018

BackgroundApproximately half of MS patients (pwMS) experience cognitive impairments including domains such as memory, concentrations, executive functions, which can be exacerbated by mood disturbances and fatigue. NICE guidelines (CG168) recommend proactive identification of cognitive symptoms, assessment and referral to neuropsychology services or occupational therapy.AimTo assess the local departmental MS service with regards to diagnosis and management of cognitive and behavioural symptoms.MethodWe conducted a retrospective audit of pwMS attending the specialist MS clinic since March 2017. Records were reviewed for documented evidence of: a discussion about cognitive and behavioural changes; use of assessment tools; management; referral. Data was also collected on demographics, presence of comorbidities, use of disease modifying therapy, and cognition-modulating medication.Results69% of pwMS demonstrated cognitive or behavioural symptoms including: fatigue (71%), poor memory (28%), depression, sleep disturbance, and concentration difficulties. Almost half expressed multiple symptoms. 47% of patients were referred for further management with OT, psychology services, or counselling.ConclusionThis study highlights the ongoing need for greater focus on identification of cognitive and behavioural symptoms in the MS population. Furthermore, simple assessment tools such as questionnaires should be considered within the clinic for monitoring cognitive symptoms and response to intervention.
WED 168 Blood-brain barrier imaging with dynamic contrast-enhanced mri
Authors: Aravinthan Varatharaj , Maria Liljeroth , Angela Darekar , Henrik BW Larsson , Ian Galea , Stig P Cramer 
Published: October 2018

BackgroundDynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) can detect subtle blood-brain barrier (BBB) permeability. We developed a protocol and conducted experiments to validate the technique.Methods12 subjects with relapsing-remitting multiple sclerosis (RRMS) and 13 controls were recruited. Whole-brain 3D DCE-MRI at 3 Tesla was used to calculate the influx constant Ki (Patlak method). Values were derived for manual regions of interest (ROI) as well as segmented tissue masks. In controls, cerebral blood volume (CBV) was measured in grey and white matter.ResultsIn RRMS, Ki in visibly-enhancing lesions was significantly higher than in normal-appearing white matter (NAWM) (p=0.002). Ki in NAWM was significantly higher in RRMS than controls, by both ROI (p=0.014) and segmentation (p=0.019) methods. In controls, Ki was significantly higher in grey than white matter (p=0.001). CBV (and therefore vascular surface area) was also significantly higher in grey matter (p=0.005), with a mean ratio of 1.9.ConclusionsOur method produces results in line with the expected behaviour of a BBB permeability marker, and the grey/white matter CBV ratio is in agreement with the histologically-established value.
THUR 179 Ocrelizumab effect on neda in patient subgroups of opera I and opera II
Authors: Ben Turner , Caroline Papeix , Bruce Cree , Ludwig Kappos , Xavier Montalban , Jerry Wolinsky , Regine Buffels , Jian Han , Stephen Hauser 
Published: October 2018

BackgroundIn a pooled analysis of patients with relapsing multiple sclerosis in the Phase III OPERA I and OPERA II (NCT01247324/NCT014112333) studies, ocrelizumab increased the proportion of patients with no evidence of disease activity (NEDA) vs interferon β−1a (IFNβ1a); post hoc subgroup analyses are reported here.MethodsNEDA (no 12 week confirmed disability progression, relapse, new/enlarging T2 lesions or T1 gadolinium enhancing lesions) rates were compared by Cochran–Mantel–Haenszel test on the pooled modified intent-to-treat (mITT) population (ocrelizumab, n=761 [600 mg intravenously every 24 weeks]; IFNβ1a, n=759 [44 μg subcutaneously three times weekly]; excludes patients with NEDA discontinuing for reasons unrelated to efficacy).ResultsTreatment benefit for NEDA in the mITT population with ocrelizumab vs IFNβ1a (47.7% vs 27.1%; p<0.001) was maintained across subgroups (ocrelizumab/IFNβ1a; p<0.001 unless stated): age (<40 years: 44.3%/22.6%; ≥40 years: 52.8%/33.7%), gender (male: 44.0%/22.2%; female: 49.7%/29.6%), prior [last 2 years] disease-modifying therapy (yes: 42.8%/23.9%; no: 49.5%/28.3%), prior relapses [last 12 months] (≤1: 49.2%/29.2%; ≥2: 44.2%/22.6%), baseline T1 Gd-enhancing lesions (none: 59.6%/38.8%; ≥1: 30.1%/10.2%) and baseline EDSS score (EDSS <2.5/<4.0: ocrelizumab 50.5%/50.3%, IFNβ1a 27.5%/26.4%; EDSS ≥2.5/≥4.0: ocrelizumab 46.0%/39.6%, IFNβ1a 26.9%/29.4% [NB: EDSS score ≥4: ocrelizumab/IFNβ1a p=0.043]).ConclusionsSubgroup analyses were consistent with those of the overall pooled population on maintaining NEDA status.
WED 184 Cladribine tablets in clarity patients with high disease activity ms
Authors: Giovannoni Gavin , Rammohan Kottil , Cook Stuart , Comi Giancarlo , Rieckmann Peter , Soelberg-Sorensen Per , Vermersch Patrick , Dangond Fernando , Hicking Christine 
Published: October 2018

BackgroundPatients with high disease activity (HDA) relapsing-remitting MS are less likely to attain no evidence of disease activity (NEDA; no relapses, MRI activity or progression).ObjectivePost-hoc analysis to compare the proportion of patients with NEDA with cladribine tablets 3.5 mg/kg (CT3.5) vs placebo.MethodsPatients from CLARITY were retrospectively stratified using 2 definitions of HDA based on relapse history, prior treatment, and MRI characteristics: HRA (n=261) and HRA plus disease activity on treatment (HRA+DAT) [n=289]). Data for patients treated with CT3.5 or placebo who fulfilled these criteria and achieved NEDA status were compared over the 2 years using odds ratios (OR) and 95% CI.ResultsHRA subgroup: 76% of CT3.5-treated patients were relapse-free and 84% were T1 Gd+ lesion free vs 49% and 31%, respectively, for placebo. HRA+DAT subgroup: 77% of CT3.5-treated were relapse-free and 85% were T1 Gd+ lesion free vs 50% and 32%, respectively, for placebo. In the HRA and HRA+DAT subgroups, 43.2% and 43.7%, respectively, of CT3.5-treated patients were disease activity free compared with 8.7%, (OR: 8.02; 95% CI: 3.93 to 16.35; p<0.0001) and 9.0% (OR: 7.82; 95% CI: 4.03 to 15.19; p<0.0001) respectively, for placebo. In the overall population, composite NEDA score favored CT over placebo (OR: 4.46; 95% CI: 3.18 to 6.26; p<0.0001).ConclusionsTreatment with CT3.5 significantly increased the proportion of HDA patients with NEDA vs placebo.Disclaimerhttp://medpub-poster.merckgroup.com/ABN2018DISC_NEDA.pdf
Analysis of urinary macrophage migration inhibitory factor in systemic lupus erythematosus
Authors: Vincent Fabien B , Slavin Laura , Hoi Alberta Y , Kitching Arthur Richard , Mackay Fabienne , Harris James , Kandane-Rathnayake Rangi , Morand Eric F 
Published: October 2018

ObjectiveTo characterise the clinical relevance of urinary macrophage migration inhibitory factor (uMIF) concentrations in patients with systemic lupus erythematosus (SLE).MethodsMIF, adjusted for urine creatinine, was quantified by ELISA in urine samples from 64 prospectively recruited patients with SLE. Serum MIF and urinary monocyte chemoattractant protein 1 (uMCP-1) were quantified by ELISA in a subset of patients (n = 39). Disease activity was assessed using the SLE Disease Activity Index-2000 (SLEDAI-2K) score.ResultsuMIF was detectable in all patients with SLE. uMIF was positively correlated with overall SLEDAI-2K, was significantly higher in patients with SLE with high disease activity (SLEDAI-2K≥10) compared with those with inactive disease (SLEDAI-2K<4), and this association remained significant after adjusting for ethnicity, flare and use of immunosuppressants. uMIF was also significantly higher in SLE patients with flare of disease, although not confirmed in multivariable analysis. No significant differences in uMIF levels were observed according to the presence of renal disease activity, as assessed by renal SLEDAI-2K or biopsy-confirmed lupus nephritis. In contrast, uMCP-1 was significantly higher in SLE patients with active renal disease. uMIF expression was not associated with irreversible organ damage accrual or glucocorticoid use.ConclusionsThese data suggest uMIF as a potential overall but not renal-specific SLE biomarker, whereas uMCP-1 is a renal-specific SLE biomarker.
Arterial occlusions increase the risk of in-stent restenosis after vertebral artery ostium stenting
Authors: Li Jingzhi , Hua Yang , Needleman Laurence , Forsberg Flemming , Eisenbray John R , Li Zhaojun , Liu Ran , Tian Xiaojie , Jiao Liqun , Liu Ji-Bin 
Published: October 2018

ObjectiveThe study was designed to investigate if vascular occlusion in the internal carotid artery (ICA) or the contralateral vertebral artery (VA) contribute to developing in-stent restenosis (ISR) in patients with vertebral artery ostium stenosis (VAOS).Methods420 consecutive patients treated with VAOS stents (from a population of 8145 patients with VAOS) from January 2013 to December 2014 were analyzed in this retrospective study; 216 with drug eluted stents and 204 with bare metal stents. Based on pre-stent DSA findings, patients were divided into four groups: both carotid and vertebral arteries patent (PAT), ICA occlusion (ICA-OCC), contralateral VA occlusion (CVA-OCC), and combined occlusions (C-OCC). The incidence of ISR (stenosis >50%) was compared between groups using Cox regression analysis.ResultsOf the 420 patients, the mean incidence of ISR was 36.4%, with a median 12 months of follow-up (IQR 3–12). Logistic regression analysis showed that drug eluting stent had less ISR than bare metal stent (OR=0.38, 95% CI 0.19 to 0.75, P=0.01). Cox regression analysis showed that CVA-OCC (HR=1.63, P=0.02) and C-OCC (HR=3.30, P=0.001) were risk factors for ISR but not ICA-OCC (P=0.31). In the CVA-OCC and C-OCC groups, in-stent peak systolic velocity (PSV) ≥140 cm/s, 1 day after successful stenting, was associated with subsequent development of ISR (OR=2.81, 95% CI 1.06 to 7.43, P=0.04).ConclusionContralateral VA occlusion at the time of stenting increased the risk of ISR, especially if stent PSV on day 1 was >140 cm/s. Bare metal stents had more ISR than drug eluting stents.
Risk factors for post-9/11 chronic rhinosinusitis in Fire Department of the City of New York workers
Authors: Putman Barbara , Zeig-Owens Rachel , Singh Ankura , Hall Charles B , Schwartz Theresa , Webber Mayris P , Cohen Hillel W , Prezant David J , Bachert Claus , Weiden Michael D 
Published: October 2018

ObjectivesChronic rhinosinusitis (CRS) has high socioeconomic burden but underexplored risk factors. The collapse of the World Trade Center (WTC) towers on 11 September 2001 (9/11) caused dust and smoke exposure, leading to paranasal sinus inflammation and CRS. We aim to determine which job tasks are risk factors for CRS in WTC-exposed Fire Department of the City of New York (FDNY) firefighters and emergency medical services (EMS) workers.MethodsThis cohort study included a 16-year follow-up of 11 926 WTC-exposed FDNY rescue/recovery workers with data on demographics, WTC exposure, job tasks and first post-9/11 complete blood counts. Using multivariable Cox regression, we assessed the associations of WTC exposure, work assignment (firefighter/EMS), digging and rescue tasks at the WTC site and blood eosinophil counts with subsequent CRS, adjusting for potential confounders.ResultsThe rate of CRS was higher in firefighters than EMS (1.80/100 person-years vs 0.70/100 person-years; p<0.001). The combination of digging and rescue work was a risk factor for CRS (HR 1.54, 95% CI 1.23 to 1.94, p<0.001) independent of work assignment and WTC exposure.ConclusionsCompared with EMS, firefighters were more likely to engage in a combination of digging and rescue work, which was a risk factor for CRS. Chronic irritant exposures associated with digging and rescue work may account for higher post-9/11 CRS rates among firefighters.
Early coronary angiography and survival after out-of-hospital cardiac arrest: a systematic review and meta-analysis
Authors: Khera Rohan , CarlLee Sheena , Blevins Amy , Schweizer Marin , Girotra Saket 
Published: October 2018

BackgroundAlthough acute myocardial infarction is a common cause of out-of-hospital cardiac arrest (OHCA), the role of early coronary angiography in OHCA remains uncertain. We conducted a meta-analysis of observational studies to determine the association of early coronary angiography with survival in OHCA.MethodsWe searched multiple electronic databases for published studies on early coronary angiography in OHCA between 1 January 1990 and 18 January 2017. Studies were included if (1) restricted to only OHCA, (2) included an exposure group that underwent early coronary angiography within 1  day of arrest onset and a concurrent control group that did not undergo early coronary angiography, and (3) reported survival outcomes. We used a random-effects model to obtain pooled OR. I2﻿ statistics and Cochran’s Q test were used to determine between-study heterogeneity.ResultsA total of 17 studies with 14 972 patients were included, of whom 6424 (44%) received early coronary angiography. Early coronary angiography was associated with higher odds of survival (pooled OR 2.54 (95% CI 1.94 to 3.33)) and survival with favourable neurological outcome (pooled OR 2.37 (95%  CI 1.71 to 3.28)). However, there was substantial heterogeneity in our pooled estimate (I2=88%  and p value for Cochran’s test <0.0001 for both outcomes). The large heterogeneity in pooled estimates was reduced after including adjusted estimates when available, and was explained by differences in methodological rigour and characteristics of included studies.ConclusionAmong patients resuscitated from OHCA, early coronary angiography is associated with increased survival to discharge and favourable neurological outcome.
Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology
Authors: Erikssen Gunnar , Aboulhosn Jamil , Lin Jeannette , Liestøl Knut , Estensen Mette E , Gjesdal Ola , Skulstad Helge , Døhlen Gaute , Lindberg Harald Lauritz 
Published: October 2018

ObjectivePatients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV).MethodsAll 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX.ResultsAmong the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five  years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6).ConclusionSRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.