140 research outputs found

    Efficient organisation of the contralateral hemisphere connectome is associated with improvement in intelligence quotient after paediatric epilepsy surgery

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    ObjectiveAims of epilepsy surgery in childhood include optimising seizure control and facilitating cognitive development. Predicting which children will improve cognitively is challenging. We investigated the association of the pre-operative structural connectome of the contralateral non-operated hemisphere with improvement in intelligence quotient (IQ) post-operatively.MethodsConsecutive children who had undergone unilateral resective procedures for epilepsy at a single centre were retrospectively identified. We included those with pre-operative volume T1-weighted non-contrast brain magnetic resonance imaging (MRI), no visible contralateral MRI abnormalities, and both pre-operative and two years post-operative IQ assessment. The MRI of the hemisphere contralateral to the side of resection was anatomically parcellated into 34 cortical regions and the covariance of cortical thickness between regions was used to create binary and weighted group connectomes.ResultsEleven patients with a post-operative IQ increase of at least 10 points at two years were compared with twenty-four patients with no change in IQ score. Children who gained at least 10 IQ points post-operatively had a more efficiently structured contralateral hemisphere connectome with higher global efficiency (0.74) compared to those whose IQ did not change at two years (0.58, p=0.014). This was consistent across thresholds and both binary and weighted networks. There were no statistically significant group differences in age, sex, age at onset of epilepsy, pre-operative IQ, mean cortical thickness, side or site of procedure, two year post-operative Engel scores or use of anti-seizure medications between the two groups. ConclusionsSurgical procedures to reduce or stop seizures may allow children with an efficiently structured contralateral hemisphere to achieve their cognitive potential. <br/

    Расчет потерь давления воздуха в горных выработках с учетом равномерных и неравномерных утечек

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    Охарактеризовано метод розрахунку втрат тиску повітря у гірничих виробках за наявності витоків (притоків), який може бути використано під час проектування системи провітрювання шахти з метою вибору засобів місцевого керування повітряних потоків, розрахунку конфігурації ліній витоку у виробленому просторі та при оцінці зміни витоків у аварійній ситуації і розробці методів керування ними.The method of calculation losses of pressure of air in the mining workings at presence of leakages (inflows), which can be used for planning of the ventilation system of mine with the purpose of choice facilities of the local control of air blast, calculation of configuration lines of leakages in the worked out goaf and at estimation change of leakages in an emergency situation and development methods of control by them

    Accurate differentiation between physiological and pathological ripples recorded with scalp-EEG

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    OBJECTIVE: To compare scalp-EEG recorded physiological ripples co-occurring with vertex waves to pathological ripples co-occurring with interictal epileptiform discharges (IEDs). METHODS: We marked ripples in sleep EEGs of children. We compared the start of ripples to vertex wave- or IED-start, and duration, frequency, and root mean square (RMS) amplitude of physiological and pathological ripples using multilevel modeling. Ripples were classified as physiological or pathological using linear discriminant analysis. RESULTS: We included 40 children with and without epilepsy. Ripples started (χ2(1) = 38.59, p < 0.001) later if they co-occurred with vertex waves (108.2 ms after vertex wave-start) than if they co-occurred with IEDs (4.3 ms after IED-start). Physiological ripples had longer durations (75.7 ms vs 53.0 ms), lower frequencies (98.3 Hz vs 130.6 Hz), and lower RMS amplitudes (0.9 μV vs 1.8 μV, all p < 0.001) than pathological ripples. Ripples could be classified as physiological or pathological with 98 % accuracy. Ripples recorded in children with idiopathic or symptomatic epilepsy seemed to form two subgroups of pathological ripples. CONCLUSIONS: Ripples co-occurring with vertex waves or IEDs have different characteristics and can be differentiated as physiological or pathological with high accuracy. SIGNIFICANCE: This is the first study that compares physiological and pathological ripples recorded with scalp EEG

    Incidence of and predictors for antiseizure medication gaps in Medicare beneficiaries with epilepsy: a retrospective cohort study.

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    BACKGROUND For the two-thirds of patients with epilepsy who achieve seizure remission on antiseizure medications (ASMs), patients and clinicians must weigh the pros and cons of long-term ASM treatment. However, little work has evaluated how often ASM discontinuation occurs in practice. We describe the incidence of and predictors for sustained ASM fill gaps to measure discontinuation in individuals potentially eligible for ASM withdrawal. METHODS This was a retrospective cohort of Medicare beneficiaries. We included patients with epilepsy by requiring International Classification of Diseases codes for epilepsy/convulsions plus at least one ASM prescription each year 2014-2016, and no acute visit for epilepsy 2014-2015 (i.e., potentially eligible for ASM discontinuation). The main outcome was the first day of a gap in ASM supply (30, 90, 180, or 360 days with no pills) in 2016-2018. We displayed cumulative incidence functions and identified predictors using Cox regressions. RESULTS Among 21,819 beneficiaries, 5191 (24%) had a 30-day gap, 1753 (8%) had a 90-day gap, 803 (4%) had a 180-day gap, and 381 (2%) had a 360-day gap. Predictors increasing the chance of a 180-day gap included number of unique medications in 2015 (hazard ratio [HR] 1.03 per medication, 95% confidence interval [CI] 1.01-1.05) and epileptologist prescribing physician (≥25% of that physician's visits for epilepsy; HR 2.37, 95% CI 1.39-4.03). Predictors decreasing the chance of a 180-day gap included Medicaid dual eligibility (HR 0.75, 95% CI 0.60-0.95), number of unique ASMs in 2015 (e.g., 2 versus 1: HR 0.37, 95% CI 0.30-0.45), and greater baseline adherence (> 80% versus ≤80% of days in 2015 with ASM pill supply: HR 0.38, 95% CI 0.32-0.44). CONCLUSIONS Sustained ASM gaps were rarer than current guidelines may suggest. Future work should further explore barriers and enablers of ASM discontinuation to understand the optimal discontinuation rate

    Antiseizure medication withdrawal risk estimation and recommendations: A survey of American Academy of Neurology and EpiCARE members

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    Objective Choosing candidates for antiseizure medication (ASM) withdrawal in well‐controlled epilepsy is challenging. We evaluated (a) the correlation between neurologists' seizure risk estimation (“clinician predictions”) vs calculated predictions, (b) how viewing calculated predictions influenced recommendations, and (c) barriers to using risk calculation.MethodsWe asked US and European neurologists to predict 2‐year seizure risk after ASM withdrawal for hypothetical vignettes. We compared ASM withdrawal recommendations before vs after viewing calculated predictions, using generalized linear models. Results Three‐hundred and forty‐six neurologists responded. There was moderate correlation between clinician and calculated predictions (Spearman coefficient 0.42). Clinician predictions varied widely, for example, predictions ranged 5%‐100% for a 2‐year seizure‐free adult without epileptiform abnormalities. Mean clinician predictions exceeded calculated predictions for vignettes with epileptiform abnormalities (eg, childhood absence epilepsy: clinician 65%, 95% confidence interval [CI] 57%‐74%; calculated 46%) and surgical vignettes (eg, focal cortical dysplasia 6‐month seizure‐free mean clinician 56%, 95% CI 52%‐60%; calculated 28%). Clinicians overestimated the influence of epileptiform EEG findings on withdrawal risk (26%, 95% CI 24%‐28%) compared with calculators (14%, 95% 13%‐14%). Viewing calculated predictions slightly reduced willingness to withdraw (−0.8/10 change, 95% CI −1.0 to −0.7), particularly for vignettes without epileptiform abnormalities. The greatest barrier to calculator use was doubting its accuracy (44%). Significance Clinicians overestimated the influence of abnormal EEGs particularly for low‐risk patients and overestimated risk and the influence of seizure‐free duration for surgical patients, compared with calculators. These data may question widespread ordering of EEGs or time‐based seizure‐free thresholds for surgical patients. Viewing calculated predictions reduced willingness to withdraw particularly without epileptiform abnormalities

    Magnesium treatment for patients with refractory status epilepticus due to POLG1-mutations

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    Mutations in the gene encoding of the catalytic subunit of mtDNA polymerase gamma (POLG1) can cause typical Alpers' syndrome. Recently, a new POLG1 mutation phenotype was described, the so-called juvenile-onset Alpers' syndrome. This POLG1 mutation phenotype is characterized by refractory epilepsy with recurrent status epilepticus and episodes of epilepsia partialis continua, which often necessitate admission to the intensive care unit (ICU) and pose an important mortality risk. We describe two previously healthy unrelated teenage girls, who both were admitted with generalized tonic-clonic seizures and visual symptoms leading to a DNA-supported diagnosis of juvenile-onset Alpers' syndrome. Despite combined treatment with anti-epileptic drugs, both patients developed status epilepticus requiring admission to the ICU. Intravenous magnesium as anti-convulsant therapy was initiated, resulting in clinical and neurophysiological improvement and rapid extubation of both patients. Treating status epilepticus in juvenile-onset Alpers' syndrome with magnesium has not been described previously. Given the difficulties encountered while treating epilepsy in patients with this syndrome, magnesium therapy might be considered

    Sharon Macdonald – Memorylands: Heritage and Identity in Europe Today

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    Memorylands: Heritage and Identity in Europe Today é um livro de síntese que assinala o culminar de anos de trabalho da antropóloga britânica Sharon Macdonald sobre os temas relacionados do património, da memória e da identidade em contextos europeus. Em Reimagining Culture: Histories, Identities and the Gaelic Renaissance, de 1997, situava o eixo analítico entre o local e o global, focando o seu olhar na questão dos muitos “renascimentos identitários” de escala local e regional que à época –..

    Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study

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    Introduction: Acquired demyelinating syndromes (ADS) are immune-mediated demyelinating disorders of the central nervous system in children. A nationwide, multicentre and prospective cohort study was initiated in the Netherlands in 2006, with a reported ADS incidence of 0.66/100,000 per year and MS incidence of 0.15/100,000 per year in the period between 2007 and 2010. In this study, we provide an update on the incidence and the long-term follow-up of ADS in the Netherlands. Methods: Children < 18 years with a first attack of demyelination were included consecutively from January 2006 to December 2016. Diagnoses were based on the International Paediatric MS study group consensus criteria. Outcome data were collected by neurological and neuropsychological assessments, and telephone call assessments. Results: Between 2011 and 2016, 55/165 of the ADS patients were diagnosed with MS (33%). This resulted in an increased ADS and MS incidence of 0.80/100,000 per year and 0.26/100,000 per year, respectively. Since 2006 a total of 243 ADS patients have been included. During follow-up (median 55 months, IQR 28–84), 137 patients were diagnosed with monophasic disease (56%), 89 with MS (37%) and 17 with multiphasic disease other than MS (7%). At least one form of residual deficit including cognitive impairment was observed in 69% of all ADS patients, even in monophasic ADS. An Expanded Disability Status Scale score of ≥ 5.5 was reached in 3/89 MS patients (3%). Conclusion: The reported incidence of ADS in Dutch children has increased since 2010. Residual deficits are common in this group, even in monophasic patients. Therefore, long-term follow-up in ADS patients is warranted

    A survey of the European Reference Network EpiCARE on clinical practice for selected rare epilepsies

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    Objective: Clinical care of rare and complex epilepsies is challenging, because evidence-based treatment guidelines are scarce, the experience of many physicians is limited, and interdisciplinary treatment of comorbidities is required. The pathomechanisms of rare epilepsies are, however, increasingly understood, which potentially fosters novel targeted therapies. The objectives of our survey were to obtain an overview of the clinical practice in European tertiary epilepsy centers treating patients with 5 arbitrarily selected rare epilepsies and to get an estimate of potentially available patients for future studies. Methods: Members of the European Reference Network for rare and complex epilepsies (EpiCARE) were invited to participate in a web-based survey on clinical practice of patients with Dravet syndrome, tuberous sclerosis complex (TSC), autoimmune encephalitis, and progressive myoclonic epilepsies including Unverricht Lundborg and Unverricht-like diseases. A consensus-based questionnaire was generated for each disease. Results: Twenty-six of 30 invited epilepsy centers participated. Cohorts were present in most responding centers for TSC (87%), Dravet syndrome (85%), and autoimmune encephalitis (71%). Patients with TSC and Dravet syndrome represented the largest cohorts in these centers. The antiseizure drug treatments were rather consistent across the centers especially with regard to Dravet syndrome, infantile spasms in TSC, and Unverricht Lundborg / Unverricht-like disease. Available, widely used targeted therapies included everolimus in TSC and immunosuppressive therapies in autoimmune encephalitis. Screening for comorbidities was routinely done, but specific treatment protocols were lacking in most centers. Significance: The survey summarizes the current clinical practice for selected rare epilepsies in tertiary European epilepsy centers and demonstrates consistency as well as heterogeneity in the treatment, underscoring the need for controlled trials and recommendations. The survey also provides estimates for potential participants of clinical trials recruited via EpiCARE, emphasizing the great potential of Reference Networks for future studies to evaluate new targeted therapies and to identify novel biomarkers.info:eu-repo/semantics/publishedVersio
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