Long-Term Outcome in Juvenile Myoclonic Epilepsy

ObjectiveTo describe long-term seizure outcomes in patients with IQ < 70 undergoing resective epilepsy surgery and to analyse whether baseline IQ predicts seizure outcome. MethodsPatients undergoing focal resective epilepsy surgery 1995–2017 at age ≥ 4 years were identified in the population-based Swedish National Epilepsy Surgery Register. Two-year, five-year and long-term (10–20-year follow-up) outcomes were analysed. Seizure outcomes of patients with IQ ≥ 70 and IQ < 70 at baseline were compared in the full cohort and between propensity score matched groups. ResultsFollow-up data were available for 884 patients, 79 of whom had IQ < 70. Matched controls were found for 74 of the IQ < 70 patients. Preoperative MRI pathology was unifocal in 54 % and 79 % of IQ < 70 and IQ ≥ 70 patients before matching compared to 58 % and 62 % after matching, respectively. Patients with IQ < 70 achieved significantly worse seizure outcomes at all time points both when analysing the full cohort and the matched groups. After matching, the proportions of seizure-free patients in the IQ < 70 group were 28 %, 32 % and 32 % at the 2-year, 5-year and long-term follow-ups, respectively. Corresponding figures in the IQ ≥ 70 group were 54 %, 62 % and 60 % (p for difference between IQ groups 0.004, 0.002 and 0.049). In the IQ < 70 group, 36 %, 29 % and 45 % had a ≥ 75 % reduction in seizure frequency at the respective three follow-ups. ConclusionLow preoperative IQ predicts lower chances of seizure freedom after resective epilepsy surgery and few patients with IQ < 70 remain completely seizure-free in the long term. Nevertheless, a significant proportion had a reduction in seizure frequency of at least 75 % at long-term follow-up, indicating an important palliative potential of resective surgery for epilepsy patients with intellectual disability.

This study was undertaken to update and evaluate long-term seizure outcomes in patients with autoimmune encephalitis (AE) based on a large cohort study with long follow-up. In this prospective observational registry study, we analyzed data from patients with AE mediated by common types of neuronal surface antibodies (anti-N-methyl-d-aspartate receptor [NMDAR], anti-leucine-rich glioma-inactivated 1 [LGI1]/contactin-associated protein-like 2 [Caspr2], anti-γ-aminobutyric acid type B receptor [GABAB R]). All patients were recruited from the Department of Neurology at West China Hospital between October 2011 and June 2019, and data were collected prospectively on their demographic and clinical characteristics, treatment strategy, and seizure outcomes, with a median follow-up of 42months (range = 6-93months). Potential risk factors associated with seizure recurrence were also assessed. Of 320 AE patients, 75.9% had acute seizures, among whom >90% of patients had their last seizure within 12months of disease onset. During our follow-up, 21 (9.3%) patients experienced seizure recurrence. Patients with anti-GABAB R encephalitis had a higher cumulative incidence of seizure recurrence than those with anti-NMDAR (log-rank p=.03) or anti-LGI1/Caspr2 encephalitis (log-rank p=.04). Among patients with anti-NMDAR encephalitis, women had a significantly higher cumulative incidence of seizure recurrence than men (log-rank p=.01). Interictal epileptiform discharges (IEDs) or seizures captured on continuous electroencephalogram (EEG) in the acute phase were identified as potential risk factors for seizure recurrence (p=.04, p=.007). Among 163 patients with ≥24months of follow-up, five (3.1%) showed persistent seizures and required ongoing antiseizure medications despite aggressive immunotherapy. Seizure recurrence occurred in a small number of patients and chronic epilepsy occurred in 3.1% of patients during prolonged follow-up. Across all types of AE, risk factors for seizure recurrence were IEDs or seizures captured on EEG in the acute phase; for anti-NMDAR encephalitis, female sex was also a risk factor.

The independent association of plateletcrit with long-term outcomes in patients undergoing primary percutaneous coronary intervention

The 2001 classification subcommittee of the International League Against Epilepsy (ILAE) proposed to 'group JME, juvenile absence epilepsy, and epilepsy with tonic clonic seizures only under the sole heading of idiopathic generalized epilepsies (IGE) with variable phenotype'. The implication is that juvenile myoclonic epilepsy (JME) does not exist as the sole phenotype of family members and that it should no longer be classified by itself or considered a distinct disease entity. Although recognized as a common form of epilepsy and presumed to be a lifelong trait, a long-term follow-up of JME has not been performed. To address these two issues, we studied 257 prospectively ascertained JME patients and encountered four groups: (i) classic JME (72%), (ii) CAE (childhood absence epilepsy) evolving to JME (18%), (iii) JME with adolescent absence (7%), and (iv) JME with astatic seizures (3%). We examined clinical and EEG phenotypes of family members and assessed clinical course over a mean of 11 +/- 6 years and as long as 52 years. Forty per cent of JME families had JME as their sole clinical phenotype. Amongst relatives of classic JME families, JME was most common (40%) followed by grand mal (GM) only (35%). In contrast, 66% of families with CAE evolving to JME expressed the various phenotypes of IGE in family members. Absence seizures were more common in family members of CAE evolving to JME than in those of classic JME families (P < 0.001). Female preponderance, maternal transmission and poor response to treatment further characterized CAE evolving to JME. Only 7% of those with CAE evolving to JME were seizure-free compared with 58% of those with classic JME (P < 0.001), 56% with JME plus adolescent pyknoleptic absence and 62% with JME plus astatic seizures. Long-term follow-up (1-40 years for classic JME; 5-52 years for CAE evolving to JME, 5-26 years for JME with adolescent absence and 3-18 years for JME with astatic seizures) indicates that all subsyndromes are chronic and perhaps lifelong. Seven chromosome loci, three epilepsy-causing mutations and two genes with single nucleotide polymorphisms (SNPs) associating with JME reported in literature provide further evidence for JME as a distinct group of diseases.

Background Cirrhotic severity scoring (CSS) is a noninvasive method that can predict histological severity of cirrhosis. This study is aimed at assessing the predictive value of CSS on long-term outcomes after curative hepatectomy for patients with hepatitis B virus- (HBV-) related hepatocellular carcinoma (HCC) and Child-Pugh grade A liver function and further developing novel nomograms to preoperatively predict posthepatectomy recurrence and survival. Methods Consecutive patients who underwent curative hepatectomy for HCC between 2008 and 2014 were retrospectively studied. According to the CSS, patients were subclassified into 3 groups: no/mild, moderate, and severe cirrhosis. The impact of CSS on recurrence-free survival (RFS) and overall survival (OS) was assessed. Furthermore, RFS and OS nomograms were developed. Results The 5-year RFS and OS rates were 36.1% and 62.8% in the no/mild cirrhosis group, compared with 28.4% and 56.2% in the moderate cirrhosis group, and 16.2% and 33.0% in the severe cirrhosis group. Long-term survival outcomes were significantly worse with the increment of cirrhotic severity. CSS, alpha-fetoprotein level, tumor size, tumor number, and macrovascular invasion were identified as independent predictors of both RFS and OS. Besides, albumin-bilirubin grade was an independent risk factor of OS not RFS. RFS- and OS-predictive nomograms based on these preoperative variables were built. For these 2 nomograms, the C-indexes were 0.696 and 0.732, respectively. Calibration curves exhibited good agreement between actual observation and nomogram prediction. Conclusions CSS was a predictor for long-term outcomes in HCC patients after curative hepatectomy. The novel nomograms exhibited accurate preoperative prediction of posthepatectomy recurrence and OS.

BackgroundPrevious studies suggest the higher the red blood cell distribution width (RDW) the greater the risk of mortality in patients with coronary artery disease (CAD). However, the relationship between RDW and long-term outcome in CAD patients undergoing percutaneous coronary intervention (PCI) with a drug-eluting stent (DES) remains unclear. This study was designed to evaluate the long-term effect of RDW in patients treated with drug-eluting stent for CAD.MethodsIn total of 2169 non-anemic patients (1468 men, mean age 60.2±10.9 years) with CAD who had undergone successful PCI and had at least one drug-eluting stent were included in this study. Patients were grouped according to their baseline RDW: Quartile 1 (RDW<12.27%), Quartile 2 (12.27%≤RDW<13%), Quartile 3 (13%≤RDW<13.5%), and Quartile 4 (RDW≥13.5).ResultsThe incidence of in-hospital mortality and death or myocardial infarction was significantly higher in Quartiles 3 and 4 compared with Quartile 1 (P<0.05). After a follow-up of 29 months, the incidence of all-cause death and stent thrombosis in Quartile 4 was higher than in Quartiles 1, 2, and 3 (P<0.05). The incidence of death/myocardial infarction/stroke and cardiac death in Quartile 4 was higher than in Quartiles 1 and 2 (P<0.05). Multivariate Cox regression analysis showed that RDW was an independent predictor of all-cause death (hazard ratio (HR) = 1.37, 95% confidence interval (CI) = 1.15–1.62, P<0.001) and outcomes of death/myocardial infarction/stroke (HR = 1.21, 95% CI = 1.04–1.39, P = 0.013). The cumulative survival rate of Quartile 4 was lower than that of Quartiles 1, 2, and 3 (P<0.05).ConclusionHigh RDW is an independent predictor of long-term adverse clinical outcomes in non-anemic patients with CAD treated with DES.

BackgroundThis meta-analysis aimed to synthesize current evidence on the association between the Geriatric Nutritional Risk Index (GNRI) and long-term outcomes in patients undergoing hemodialysis.MethodsElectronic databases were systematically searched for relevant studies that investigated the association between GNRI and long-term outcomes in hemodialysis patients until November 2023. The primary outcome was the association between the GNRI (i.e., low versus high) and overall mortality risk, while the secondary outcome was the relationship between the GNRI and cardiovascular mortality risk.ResultsThirty cohort studies involving 55,864 patients were included. A low GNRI was found to be significantly associated with increased overall mortality (hazard ratio [HR]: 2.42, 95% confidence interval [CIs]: 2.10–2.79, p < 0.00001, I2 = 65%). Each unit increase in GNRI corresponded to a 5% reduction in mortality risk (HR: 0.95, 95% CI: 0.93–0.96, p < 0.00001, I2 = 79%). The association remained consistent across Asian (HR = 2.45, 95% CI: 2.08–2.88, p < 0.00001, I2 = 70%) and non-Asian subgroups (HR = 2.3, 95% CI: 1.72–3.06, p < 0.00001, I2 = 23%). Meta-regression analysis of patient age (coefficient: −0.002; p = 0.896), male proportion (coefficient: 0.002; p = 0.875), percentage of diabetes mellitus (coefficient: −0.003; p = 0.605), and follow-up duration (coefficient: −0.003; p = 0.431) revealed that these moderator variables did not significantly influence the association between GNRI and overall mortality risk. Cardiovascular mortality risk also increased with low GNRI (HR, 1.93; 95%CI: 1.51–2.45, p < 0.00001; I2 = 2%). Similarly, an inverse association was observed between the GNRI values and cardiovascular mortality risk (HR, 0.94; 95% CI: 0.91–0.97; p < 0.0001; I2 = 65%) (per unit increase).ConclusionThe GNRI is a simple nutritional screening tool that can be used to effectively stratify patients undergoing hemodialysis globally. Further studies are warranted to determine whether nutrition optimization based on the GNRI improves long-term outcomes.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, CRD42023483729.

Prognostic Impact of the Geriatric Nutritional Risk Index on Long-Term Outcomes in Patients Who Underwent Percutaneous Coronary Intervention

The authors report long-term follow-up seizure outcome in patients who underwent corpus callosotomy during the period 1981-2001 at the Montreal Neurological Institute. The records of 95 patients with a minimum follow-up of 5 years (mean 17.2 years) were retrospectively evaluated with respect to seizure, medication outcomes, and prognostic factors on seizure outcome. All patients had more than one type of seizure, most frequently drop attacks and generalized tonic-clonic seizures. The most disabling seizure type was drop attacks, followed by generalized tonic-clonic seizures. Improvement was noted in several seizure types and was most likely for generalized tonic-clonic seizures (77.3%) and drop attacks (77.2%). Simple partial, generalized tonic, and myoclonic seizures also benefited from anterior callosotomy. The extent of the callosal section was correlated with favorable seizure outcome. The complications were mild and transient and no death was seen. This study confirms that anterior callosotomy is an effective treatment in intractable generalized seizures that are not amenable to focal resection. When considering this procedure, the treating physician must thoroughly assess the expected benefits, limitations, likelihood of residual seizures, and the risks, and explain them to the patient, his or her family, and other caregivers.

Adults with juvenile myoclonic epilepsy (JME) have subtle brain structural abnormalities in the frontothalamocortical network, poorer cognitive function, and worse long-term social outcomes, even when their seizures are controlled and/or remitted. The natural history of JME and development of abnormalities in brain structure and cognition from epilepsy onset has not been studied. The maturational trajectories of cognitive and brain development were prospectively compared between 19 children with new-onset JME in the first 2 years after diagnosis and 57 healthy controls. Cognitive abilities of children with JME were similar to or worse than healthy controls at baseline but failed to reach the competence level of healthy controls at follow-up across most of the tested cognitive abilities. Abnormal patterns of brain development, as assessed by magnetic resonance imaging studies, were evident in children with JME and included attenuation of age-related decline in cortical volume, thickness, and surface area compared to typically developing children. The altered brain developmental trajectory in the JME group was evident in higher-association frontoparietotemporal brain regions (p < 0.05, corrected for multiple comparisons). Children with JME have abnormal structural brain development and impaired cognitive development early in the course of their epilepsy.

Although a number of recent studies on the surgical treatment of insular low-grade glioma (LGG) have demonstrated that aggressive resection leads to increased overall patient survival and decreased malignant progression, less attention has been given to the results with respect to tumor-related epilepsy. The aim of this investigation was to evaluate the impact of volumetric, histological, and intraoperative neurophysiological factors on seizure outcome in patients with insular LGG. The authors evaluated predictors of seizure outcome with special emphasis on both the extent of tumor resection (EOR) and the tumor's infiltrative pattern quantified by computing the difference between the preoperative T2- and T1-weighted MR images (ΔVT2T1) in 52 patients with preoperative drug-resistant epilepsy. The 12-month postoperative seizure outcome (Engel class) was as follows: seizure free (Class I), 67.31%; rare seizures (Class II), 7.69%; meaningful seizure improvement (Class III), 15.38%; and no improvement or worsening (Class IV), 9.62%. Poor seizure control was more common in patients with a longer preoperative seizure history (p < 0.002) and higher frequency of seizures (p = 0.008). Better seizure control was achieved in cases with EOR ≥ 90% (p < 0.001) and ΔVT2T1 < 30 cm(3) (p < 0.001). In the final model, ΔVT2T1 proved to be the strongest independent predictor of seizure outcome in insular LGG patients (p < 0.0001). No or little postoperative seizure improvement occurs mainly in cases with a prevalent infiltrative tumor growth pattern, expressed by high ΔVT2T1 values, which consequently reflects a smaller EOR.

Background Major emergency abdominal surgery (e.g. ileus, perforation) is relatively common and associated with postoperative complications and mortality. Long-term management of patients with atrial fibrillation (AF) associated with a secondary precipitant has recently received increased attention. It is further unknown whether the conflicting results regarding long-term outcomes in patients with new onset AF depends on surgical subtype or the arrhythmia itself. Purpose The aim of this study was to compare long-term clinical outcomes (AF related hospitalization, stroke, and mortality) in patients with perioperative new onset AF in relation to major emergency abdominal surgery and patients with non-perioperative new onset AF. Methods We crosslinked data from Danish nationwide registries and identified all patients who underwent major emergency abdominal surgery (2000-2018) and were diagnosed with new onset AF perioperatively, and patients who developed new onset AF in a non-perioperative setting. Patients with new onset perioperative AF were matched in a 1:5 ratio on age, sex and year of AF diagnosis with patients with non-perioperative new onset AF. From discharge, we examined unadjusted rates of outcomes and adjusted hazard ratios of outcomes were assessed using multivariable Cox regression analysis. Results The study population comprised 794 (out of 42,021) patients with perioperative new onset AF and 3970 patients with non-perioperative new onset AF (median age of 78 years [interquartile range: 70-83] and 57.3% women in both groups). In general, patients with new-onset perioperative AF had lower comorbid burden compared with patients with non-perioperative new onset AF. During the first month after the hospital admission, 15.0% of the patients with perioperative AF and 36.1% of the patients with new onset AF initiated oral anticoagulation therapy (after 2010, 19.7% and 41.2%, respectively). Two years after discharge, 3.9% of patients with perioperative AF and 5.8% of patients with new onset AF (p = 0.031) had had a stroke, while 32.9% and 40.1% (p = 0.002) had had a new hospitalization with AF. Cumulative incidences of stroke and AF related hospitalization are depicted in the Figure. In the multivariable models, perioperative AF was associated with a similar rate of stroke and lower rate of AF related hospitalization compared with non-perioperative new onset AF (HR 1.03, 95% confidence interval, CI: 0.54-1.96 for stroke and HR 0.68, 95% CI: 0.50-0.92 for AF related hospitalization, respectively). Conclusion Perioperative new onset atrial fibrillation in relation to major emergency abdominal surgery was associated with long-term similar rates of stroke and lower rates of AF related hospitalization compared with new onset atrial fibrillation in the non-perioperative setting. This study demonstrates a need for more knowledge about the progress of atrial fibrillation in the setting of non-cardiac surgery.Cumulative Incidence - strokeCumulative Incidence - AF_re

The CHADS2 and CHA2 DS2 -VASc scores are clinical risk stratification instruments that are used clinically to assess the risk of stroke in patients with atrial fibrillation (AF). The aim of this study was to evaluate whether the prestroke CHADS2 and CHA2 DS2 -VASc scores could be useful for predicting infarction severity and long-term outcomes in patients with acute ischaemic stroke. This prospective study included all 1494 patients who had acute ischaemic stroke without haemorrhagic transformation which was evidenced with magnetic resonance (MR) imaging during hospitalization. Total infarction volume and arterial stenosis score were calculated based on MR imaging. National Institutes of Health Stroke Scale scores (NIHSSs) were obtained at admission and discharge by board-certified neurologists. The clinical outcomes were defined as composite endpoints of restroke and mortality and were recorded with the mean follow-up period of 37.5months. There were 195 (13.1%) patients with AF. The patients with AF had significantly higher median CHADS2 and CHA2 DS2 -VASc scores than the patients without AF (P<.001). Patients with higher CHADS2 and CHA2 DS2 -VASc scores had significantly higher total infarction volume, arterial stenosis score and NIHSS scores at discharge and poorer clinical outcomes. After adjusting for age, gender and AF, only CHA2 DS2 -VASc scores could predict both restroke and composite endpoints. Prestroke CHA2 DS2 -VASc scores appear to have better clinical value for predicting the severity of infarction and long-term clinical outcomes in acute ischaemic stroke patients with and without AF.

Left atrial (LA) function and strain patterns by magnetic resonance imaging (MRI) have been investigated as markers of several cardiovascular pathologies, including cardiac amyloidosis (CA). However, associations with clinical outcomes have not been investigated. To compare LA function and strain by MRI in CA patients to a matched cohort of patients without cardiovascular disease (CVD) and evaluate the association with long-term clinical outcomes in CA patients. Retrospective case control. A total of 51 patients with CA and 51 age-, gender-, and race-matched controls without CVD who underwent MRI in sinus rhythm. ECG-gated balanced steady-state free precession sequence at 1.5T. All measurements were completed by one investigator (M.M.B.). LA function and strain parameters were measured including LA indexed minimum and maximum volumes, LA reservoir (R), contractile (CT), and conduit (CD) strain. We compared groups after adjusting for age, hypertension, New York Heart Association class, modified staging system (troponin-I, BNP, estimated GFR) and left ventricular ejection fraction (LVEF) for an endpoint of all-cause mortality and a composite endpoint of heart failure hospitalization (HFH) or death. Differences between groups were evaluated with t tests for continuous variables or χ2 tests for categorical variables. A multivariable regression model was used to assess the associations of the P values-two-sided tests-<0.05 were considered statistically significant. CA patients with median follow up of 4.9 (8.5) months had significantly lower LA strain and higher LA volumes in comparison to the matched cohort. In the multivariable analysis, only LVEF was significantly associated with death while ƐCT (OR 0.6, CI: 0.41-0.89), indexed minimum LA volume (OR 1.06, CI: 1.02-1.13) and indexed maximum LA volume (OR 1.08, CI: 1.01-1.15) were significantly associated with the composite outcome of death or HFH. In this retrospective study of CA patients, ƐCT and indexed minimum and maximum LA volumes were significantly associated with the composite outcome of death or HFH. 3 TECHNICAL EFFICACY: Stage 3.

Evidence of small vessel disease in patients with juvenile myoclonic epilepsy based on the peak width of skeletonized mean diffusivity