Current available therapies control Myasthenia gravis (MG) reasonably well, but Health Related Quality of life (HRQOL) remains lower than expected. The aim was provide insights in how HRQOL in MG stands across borders and time, compare the scores to general population controls and other chronic disorders and assess the impact of potential predictors for quality of life such as a) clinical characteristics b) antibodies c) thymoma and d) treatment in a population-based cohort.MethodsWe designed a population-based cross-sectional study including 858 patients, 373 from Norway and 485 from the Netherlands. The Short Form Health Survey 36 (SF-36) and a cross-cultural validated questionnaire were used. Data were in addition compared to the general population, other chronic diseases and previous studies.ResultsMean physical composite score was 59.4 and mental composite score 69.0 with no differences between the countries. The mean HRQOL score was lower in patients with bulbar and generalized symptoms (p < 0.001) compared to sex and age adjusted healthy controls, but not in patients with ocular symptoms or patients in remission. Multivariate analysis revealed that female gender, generalized symptoms and use of secondary immunosuppressive drugs at the time of testing were risk factors for reduced HRQOL.ConclusionsRemission and absence of generalized symptoms were favorable factors for HRQOL in MG patients. Historically, the HRQOL levels have not changed since 2001 and no new clinical predictors could be detected in this exhaustive population-based study. Further studies should explore the impact of non clinical factors like ethnic variations, socio-economic and hormonal factors on HRQOL.Electronic supplementary materialThe online version of this article (doi:10.1186/s12955-015-0298-1) contains supplementary material, which is available to authorized users.

Objectives: To compare the prevalence of myasthenia gravis (MG) subgroups based on immunological markers and clinical presentation in two geographically complete MG populations in northern Europe. Methods: This cross-sectional study included all living MG patients in Norway and a regional cohort from the Netherlands. Patients were identified using their hospital registration codes. Medical charts of subjects >16 years were reviewed. Inclusion criteria were clinical MG, a positive antibody test for acetylcholine receptor (AChR MG) or muscle-specific kinase (MuSK MG), or if seronegative MG, confirmed by an electrophysiological test. Results: 1,205 MG patients (534 Norwegians and 671 Dutch) fulfilled the criteria, giving a higher point prevalence in the Netherlands (167/million, 95% CI 155-180) than in Norway (138/million, 95% CI 126-150). In particular, rates of AChR MG (143 vs. 111/million), MuSK MG (6.5 vs. 0.5/million), and ocular phenotype (62 vs. 24/million) were higher in the Netherlands. Conclusion: Novel findings are an AChR MG geographical north-south gradient and a 2.6-fold more ocular MG patients in the Netherlands than in Norway. The MuSK MG latitudinal gradient supports the notion of a north-south gradient in Europe, with a higher prevalence in the south. The variation is probably explained by genetic differences between the populations, in addition to environmental interactions.

BackgroundCaesarean section (CS) is often a life-saving procedure, but can also lead to serious complications, even more so in low-resource settings. Therefore unnecessary CS should be avoided and optimal circumstances for vaginal delivery should be created. In this study, we aim to audit indications for Caesarean sections and improve decision-making and obstetric management.MethodsAudit of all cases of CS performed from January to August 2013 was performed in a rural referral hospital in Tanzania. The study period was divided in three audit blocks; retrospective (before auditing), prospective 1 and prospective 2. A local audit panel (LP) and an external auditor (EA) judged if obstetric management was adequate and indications were appropriate or if CS could have been prevented and yet retain good pregnancy outcome. Furthermore, changes in modes of deliveries, overall pregnancy outcome and decision-to-delivery interval were monitored.ResultsDuring the study period there were 1868 deliveries. Of these, 403 (21.6%) were Caesarean sections. The proportions of unjustified CS prior to introduction of audit were as high as 34 and 75%, according to the respective judgments of LP and EA. Following introduction of audit, the proportions of unjustified CS decreased to 23% (p = 0.29) and 52% (p = 0.01) according to LP and EA respectively. However, CS rate did not change (20.2 to 21.7%), assisted vacuum delivery rate did not increase (3.9 to 1.8%) and median decision-to-delivery interval was 83 min (range 10 - 390 min).ConclusionsAlthough this is a single center study, these findings suggest that unnecessary Caesarean sections exist at an alarming rate even in referral hospitals and suggest that a vast number can be averted by introducing a focused CS audit system. Our findings indicate that CS audit is a useful tool and, if well implemented, can enhance rational use of resources, improve decision-making and harmonise practice among care providers.

BackgroundRandomised controlled trials with perfusion selection have shown benefit of endovascular treatment (EVT) for ischaemic stroke between 6 and 24 hours after symptom onset or time last seen well. However, outcomes after EVT in these late window patients without perfusion imaging are largely unknown. We assessed their characteristics and outcomes in routine clinical practice.MethodsThe Multicenter Randomized Clinical Trial of Endovascular Treatment for Acute Ischemic Stroke in the Netherlands Registry, a prospective, multicentre study in the Netherlands, included patients with an anterior circulation occlusion who underwent EVT between 2014 and 2017. CT perfusion was no standard imaging modality. We used adjusted ordinal logistic regression analysis to compare patients treated within versus beyond 6.5 hours after propensity score matching on age, prestroke modified Rankin Scale (mRS), National Institutes of Health Stroke Scale, Alberta Stroke Programme Early CT Score (ASPECTS), collateral status, location of occlusion and treatment with intravenous thrombolysis. Outcomes included 3-month mRS score, functional independence (defined as mRS 0–2), and death.ResultsOf 3264 patients who underwent EVT, 106 (3.2%) were treated beyond 6.5 hours (median 8.5, IQR 6.9–10.6), of whom 93 (87.7%) had unknown time of stroke onset. CT perfusion was not performed in 87/106 (80.2%) late window patients. Late window patients were younger (mean 67 vs 70 years, p<0.04) and had slightly lower ASPECTS (median 8 vs 9, p<0.01), but better collateral status (collateral score 2–3: 68.3% vs 57.7%, p=0.03). No differences were observed in proportions of functional independence (43.3% vs 40.5%, p=0.57) or death (24.0% vs 28.9%, p=0.28). After matching, outcomes remained similar (adjusted common OR for 1 point improvement in mRS 1.04, 95% CI 0.56 to 1.93).ConclusionsWithout the use of CT perfusion selection criteria, EVT in the 6.5–24-hour time window was not associated with poorer outcome in selected patients with favourable clinical and CT/CT angiography characteristics. randomised controlled trials with lenient inclusion criteria are needed to identify more patients who can benefit from EVT in the late window.

Background: Reperfusion status after intra-arterial thrombectomy (IAT) is a critical predictor of functional outcome after acute ischemic stroke. However, most prognostic models have not included a detailed assessment of reperfusion status after IAT. Objective: The aim of this work was to assess the association between successful reperfusion and clinical outcome. Methods: Clinical, radiological, and procedural variables of patients treated with IAT were extracted from our prospective stroke registry. The association with functional outcome using the modified Rankin Scale (mRS) after 3 months was assessed using multivariable logistic regression. An extension of the modified TICI score, eTICI, was used to classify reperfusion status. The prognostic value of reperfusion status after IAT in addition to age, stroke severity, imaging characteristics, treatment with intravenous thrombolysis, and time from symptom onset to the end of IAT was assessed with logistic regression and summarized with receiver operating characteristic curves. Results: In total, 119 patients were included (mean age 66 years). In multivariable analysis, age >80 years (OR 6.8, 95% CI 1.2–39.8), NIHSS at presentation >15 (OR 7.3, 95% CI 2.3–23.5), and incomplete reperfusion status (eTICI score <2C; OR 10.3, 95% CI 3.5–30.6) were the strongest predictors of a poor outcome (mRS 3–6). Adding reperfusion status to the model improved the prognostic accuracy (AUC 0.88, 95% CI 0.91–0.94). Our results indicate a large difference between using an eTICI cutoff of ≥2C versus ≥2B: a cutoff ≥2C improved the predictive value for a good clinical outcome (2C: positive predictive value, PPV, 0.78; 2B: PPV 0.32). Conclusion: Our results promote using reperfusion status for assessing prognosis in ischemic stroke patients treated with IAT. A model using eTICI ≥2C had greater PPV than eTICI ≥2B and could improve prognostic accuracy.

Patients with unknown time of stroke onset (UOS) represent around one-third of ischemic stroke patients. These are patients with wake-up stroke (WUS) or daytime-unwitnessed stroke (DUS), often presenting outside the time-window for reperfusion therapy. UOS patients presenting between 4.5 and 12 h after time of last-seen-well were included. Clinical and imaging characteristics were compared between WUS and DUS patients. Good functional outcome was defined as a modified Rankin scale of ≤2 at follow-up. Sixty-one UOS patients were included: 42 WUS and 19 DUS patients. Stroke severity at presentation was mild to moderate with a median National Institutes of Health Stroke Scale of 5 in WUS and 6 in DUS patients. Time between last-seen-well and presentation at the hospital was shorter in patients with DUS compared to WUS (506 vs 362 min, p < 0.01). CT imaging results were similar, with a median Alberta Stroke Program Early CT Score of 10 for both WUS and DUS patients. After correction for age and NIHSS at presentation, no difference in good functional outcome was found between WUS (52%) and DUS (22%). In patients with unknown onset ischemic strokes presenting between 4.5 and 12 h after time of last-seen-well, clinical and radiological features were in large part similar between WUS and DUS. The outcome in the overall cohort was rather poor despite a favorable neuroimaging profile at presentation. These findings underscore the need for clinical trials in patients in whom stroke onset time is unknown.

Our findings indicate that Asian immigrant MG patients carry genetic factors or environmental/lifestyle factors which contribute to their specific phenotype, even after migration. Muscle Nerve 55: 819-827, 2017.

Background: It is unknown if ambulance paramedics adequately assess neurological deficits used for prehospital stroke scales to detect anterior large-vessel occlusions. We aimed to compare prehospital assessment of these stroke-related deficits by paramedics with in-hospital assessment by physicians. Methods: We used data from 2 prospective cohort studies: the LPSS (Leiden Prehospital Stroke Study) and PRESTO study (Prehospital Triage of Patients With Suspected Stroke). In both studies, paramedics scored 9 neurological deficits in stroke code patients in the field. Trained physicians scored the National Institutes of Health Stroke Scale (NIHSS) at hospital presentation. Patients with transient ischemic attack were excluded because of the transient nature of symptoms. Spearman rank correlation coefficient (r s ) was used to assess correlation between the total prehospital assessment score, defined as the sum of all prehospital items, and the total NIHSS score. Correlation, sensitivity and specificity were calculated for each prehospital item with the corresponding NIHSS item as reference. Results: We included 2850 stroke code patients. Of these, 1528 had ischemic stroke, 243 intracranial hemorrhage, and 1079 stroke mimics. Correlation between the total prehospital assessment score and NIHSS score was strong (r s =0.70 [95% CI, 0.68–0.72]). Concerning individual items, prehospital assessment of arm (r s =0.68) and leg (r s =0.64) motor function correlated strongest with corresponding NIHSS items, and had highest sensitivity (arm 95%, leg 93%) and moderate specificity (arm 71%, leg 70%). Neglect (r s =0.31), abnormal speech (r s =0.50), and gaze deviation (r s =0.51) had weakest correlations. Neglect and gaze deviation had lowest sensitivity (52% and 66%) but high specificity (84% and 89%), while abnormal speech had high sensitivity (85%) but lowest specificity (65%). Conclusions: The overall prehospital assessment of stroke code patients correlates strongly with in-hospital assessment. Prehospital assessment of neglect, abnormal speech, and gaze deviation differed most from in-hospital assessment. Focused training on these deficits may improve prehospital triage.