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  • Coyne Gardner posted an update 1 day, 7 hours ago

    Tuberous sclerosis complex (TSC) is a multisystem genetic disorder associated with refractory early-onset epilepsy. Current evidence supports surgery as the intervention most likely to achieve long-term seizure freedom, but no specific guidelines are available on TSC pre-surgical workup. This critical review assesses which TSC patients are suitable for surgical treatment, when pre-surgical evaluation should start, and what degree of surgical resection is optimal for postsurgical outcome. We searched for publications from 2000 to 2020 in Pubmed and Embase using the terms “tuberous sclerosis,” “epilepsy,” and “epilepsy surgery”. To evaluate postsurgical seizure outcome, we selected only studies with at least one year of follow-up. Tacrine purchase Overall, we collected data on 1,026 patients from 34 studies. Age at surgery ranged from one month to 54 years. Mean age at surgery was 8.41 years. Of the diagnostic non-invasive pre-surgical tools, MRI and video-EEG were considered most appropriate. Promising data for epileptogenic tnary epilepsy surgery team.We report two cases highlighting the diversity of vagal nerve stimulation (VNS)-related effects on voice and breathing in patients with refractory epilepsy. The patients had both implantation and stimulation-related side effects, which lasted for several months, impacting on their quality of life. The adverse effects appear to be due to recurrent laryngeal nerve paralysis-related vocal cord hypofunction and stimulation-related vocal fold spasms, however, their inter-relationship is complex. In one of the patients, we were able to utilize the novel programming capabilities of the VNS device to reduce the laryngeal side effects without compromising therapeutic efficacy. [Published with video sequences].The identification of the aetiology of a patient’s epilepsy is instrumental in the diagnosis, prognostic counselling and management of the epilepsies. Indeed, the aetiology can be important for determining the recurrence risk of single seizures and so for making a diagnosis of epilepsy. Here, we divide the aetiologies into six categories structural, genetic, infectious, metabolic, immune (all of which are part of the International League Against Epilepsy [ILAE] classification system) and neurodegenerative (which we have considered separately because of its growing importance in epilepsy). These are not mutually exclusive categories and many aetiologies fall into more than one category. Indeed, genetic factors probably play a role, to varying degrees, in the risk of seizures in all people with epilepsy. In each of the categories, we discuss what we regard as the most important aetiologies; importance being determined not only by prevalence but also by clinical significance. The introduction contains information suitable for level 1 competency (entry level), whilst the subsequent sections contain information aimed at level 2 competency (proficiency level) as part of the new ILAE competency-based curriculum. As we move towards precision medicine and targeted therapies, so aetiologies will play an even greater role in the management of epilepsy.

    The influence of age-dependent changes on fractional flow reserve (FFR) or instantaneous free-wave ratio (iFR) and the response to pharmacological hyperaemia has not been investigated.

    We investigated the impact of age on these indices.

    This is as post-hoc analysis of the ADVISE II trial, including a total of 690 pressure recordings (in 591 patients). Age-dependent correlations with FFR and iFR were calculated and adjusted for stenosis severity. Patients were stratified into three age terciles. The hyperaemic response to adenosine, calculated as the difference between resting and hyperaemic pressure ratios, and the prevalence of FFR-iFR discordance were assessed.

    Age correlated positively with FFR (r=0.08, 95% CI 0.01 to 0.15, p=0.015), but not with iFR (r=-0.03, 95% CI -0.11 to 0.04, p=0.411). The hyperaemic response to adenosine decreased with patient age (0.12 ± 0.07, 0.11 ± 0.06, 0.09 ± 0.05, for the 1st[33-58 years], 2nd[59-69 years] and 3rd[70-94 years] age tertiles, respectively, p<0.001) and showed significant correlation with age (r=-0.14, 95% CI -0.21 to -0.06, p<0.001). The proportion of patients with FFR≤0.80 + iFR>0.89 discordance doubled in the first age-tercile (14.1% vs 7.1% vs 7.0%, p=0.005).

    The hyperaemic response of the microcirculation to adenosine administration is age-dependent. FFR values increase with patient age, while iFR values remain constant across the age spectrum. These findings contribute to explain differences observed in functional stenosis classification with hyperaemic and non-hyperaemic coronary indices.

    The hyperaemic response of the microcirculation to adenosine administration is age-dependent. FFR values increase with patient age, while iFR values remain constant across the age spectrum. These findings contribute to explain differences observed in functional stenosis classification with hyperaemic and non-hyperaemic coronary indices.

    In most centers, clinically significant percutaneous paravalvular leak (PVL) closure following valve replacement surgery is reserved for those considered high-risk for surgery. There is paucity of data regarding long-term outcomes of these patients.

    Our goals were to assess long-term outcomes of patients undergoing percutaneous PVL closure.

    100 consecutive transcatheter PVL closure procedures (74 mitral, 26 aortic) were performed in 95 patients between February 2005 and august 2019 at our hospital. Data collected included procedural success rates, indication-specific outcomes and mortality.

    Mean follow-up was 5.6±6.1 years, mean age 62.6±15.2, 45.4 % were female. The device was successfully implanted in 88 procedures (88.0%). Patients who presented with heart failure (n-57) had a significant improvement in the NYHA classification (29.2% class 3/4 versus 100.0%, P<0.001). For patients who presented with hemolytic anemia (n-38), hemoglobin increased (11.94±1.634 vs. 9.72±1.49, P<0.001) and LDH levels were reduced (1,354.90±1,225.55 vs. 2,039.40±1,347.20, P<0.001) following the procedure. Rates of mortality were 3.8% at 90 days, 15.6% after one year, and 27.2% after 5 years.

    For patients who are deemed intermediate to high-risk for repeat surgery, transcatheter PVL closure shows reasonable clinical success rates, with a significant improvement in symptoms, and a relatively low rate of periprocedural complications.

    For patients who are deemed intermediate to high-risk for repeat surgery, transcatheter PVL closure shows reasonable clinical success rates, with a significant improvement in symptoms, and a relatively low rate of periprocedural complications.

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