0164: Evolution of preexcitation syndrome in children Brembilla-Perrot, Béatrice; Moulin-Zinsch, Anne; Louis Cloez, Jean ...
Archives of Cardiovascular Diseases Supplements,
January 2015, 2015-01-00, Letnik:
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Journal Article
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Previous studies reported a spontaneous disappearance of preexcitation syndrome (PS) in patients with a long accessory pathway (AP) effective refractory period (ERP) and in children <12 years (y), ...but stability in children >12 y with inducible SVT and short AP-ERP. The purpose of the study was to collect the data of untreated children with a PS, studied 2 times at least one year of interval.
2 baseline electrophysiological studies (EPS) were performed within 1 to 25 years of one another (mean 6±years) in 39 children and teenagers, 17 boys, 22 girls, aged initially from 1 to 19 years (12.5±4), with overt PS. First EPS (EPS1) was indicated for syncope (n=4), atrioventricular reentrant tachycardias (AVRT)(n=17) or for asymptomatic PS (n=18). The protocol was similar, performed in control state (CS) and after isoproterenol.
At EPS2, among patients studied for syncope at EPS1, 1 has still syncope, 2 have AVRT, 1 is asymptomatic. Among patients with AVRT at EPS1, 14 (82%) have still AVRT, 3 are asymptomatic. Among asymptomatic patients, 13 (72%) remain asymptomatic, 2 have AVRT, 3 have syncope. AVRT in children presenting initially with syncope or initially asymptomatic children occurred in 2/4 with inducible AVRT at EPS1. The higher rate conducted by AP was similar in CS and after isoproterenol at EPS2 (178±72bpm, 203±81) and at EPS1 (188±62, 237±83)(p<0.01). AP-ERP’s were similar in CS at EPS2 (283±68ms) and 1 (281±91.5) and tended to increase from 211±72 at ESP1 to 234±58ms at EPS2 (p<0.07) after isoproterenol. AP has lost anterograde conduction in 6 children with initially long APERP but all 5 children with initially inducible AVRT had still inducible AVRT. Two children with initially a long AP-ERP had shorter AP-ERP at EPS2. AVRT was induced at EPS2 in asymptomatic PS with initially negative EPS in 3 children.
Contrary to previous studies, we did not find significant changes of clinical and electrophysiological data in children after a mean follow-up of 6±5 years. Most of children with spontaneous or inducible AVRT’s at the first evaluation have still inducible AVRT’s at the second evaluation. AP-ERP did not increase significantly.
Condensed tannins, responsible for berry and wine astringency, may have been selected during grapevine domestication. This work examines the phylogenetic distribution of condensed tannins throughout ...the Vitaceae phylogenetic tree.
Green berries and mature leaves of representative true-to-type members of the Vitaceae were collected before 'véraison', freeze-dried and pulverized, and condensed tannins were measured following depolymerization by nucleophilic addition of 2-mercaptoethanol to the C4 of the flavan-3-ol units in an organic acidic medium. Reaction products were separated and quantified by ultrahigh pressure liquid chromatography/diode array detection/mass spectrometry.
The original ability to incorporate epigallocatechin (EGC) into grapevine condensed tannins was lost independently in both the American and Eurasian/Asian branches of the Vitaceae, with exceptional cases of reversion to the ancestral EGC phenotype. This is particularly true in the genus Vitis, where we now find two radically distinct groups differing with respect to EGC content. While Vitis species from Asia are void of EGC, 50 % of the New World Vitis harbour EGC. Interestingly, the presence of EGC is tightly coupled with the degree of leaf margin serration. Noticeably, the rare Asian EGC-forming species are phylogenetically close to Vitis vinifera, the only remnant representative of Vitis in Eurasia. Both the wild ancestral V. vinifera subsp. sylvestris as well as the domesticated V. vinifera subsp. sativa can accumulate EGC and activate galloylation biosynthesis that compete for photoassimilates and reductive power.
Scouting techniques combining rapid counting methods must be developed to help growers with immediate decision making in integrated pest management programs. We evaluated a method for estimating ...densities of western flower thrips, Frankliniella occidentalis (Pergande) (Thysanoptera: Thripidae), one of the most damaging insect pests of greenhouse cucumber, Cucumis sativus L., and rose, Rosa × hybrida crops in southeastern France. This method is based on abundance classes of thrips observed on sampling units of flowers and foliage during a period of <1 min. Classes were calibrated using actual counts, and precision was improved by introducing additional predictive variables into multivariate nonparametric regression models. Regression models using infestation variables with and without climatic variables significantly increased calibration precision and made possible the accurate description of population dynamics. Rapid visual scouting methods could be combined for surveys of different pests and diseases. When calibrated, they provide growers or technicians with accurate tools guiding crop protection decisions.
Relapses occur in 15%-25% of patients with leucine-rich glioma-inactivated 1 antibody (LGI1-Ab) autoimmune encephalitis and may cause additional disability. In this study, we clinically characterized ...the relapses and identified factors predicting their occurrence.
This is a retrospective chart review of patients with LGI1-Ab encephalitis diagnosed at our center between 2005 and 2022. Relapse was defined as worsening of previous or appearance of new symptoms after at least 3 months of clinical stabilization.
Among 210 patients, 30 (14%) experienced a total of 33 relapses. The median time to first relapse was 23.9 months (range: 4.9-110.1, interquartile range IQR: 17.8). The CSF was inflammatory in 11/25 (44%) relapses, while LGI1-Abs were found in the serum in 16/24 (67%) and in the CSF in 12/26 (46%); brain MRI was abnormal in 16/26 (62%) relapses. Compared with the initial episode, relapses manifested less frequently with 3 or more symptoms (4/30 patients, 13% vs 28/30, 93%;
< 0.001) and had lower maximal modified Rankin scale (mRS) score (median 3, range: 2-5, IQR: 1 vs 3, range: 2-5, IQR: 0;
= 0.001). The median mRS at last follow-up after relapse (2, range: 0-4, IQR: 2) was significantly higher than after the initial episode (1, range: 0-4, IQR: 1;
= 0.005). Relapsing patients did not differ in their initial clinical and diagnostic features from 85 patients without relapse. Nevertheless, residual cognitive dysfunction after the initial episode (hazard ratio:13.8, 95% confidence interval 1.5; 129.5;
= 0.022) and no administration of corticosteroids at the initial episode (hazard ratio: 4.8, 95% confidence interval 1.1; 21.1;
= 0.036) were significantly associated with an increased risk of relapse.
Relapses may occur years after the initial encephalitis episode and are usually milder but cause additional disability. Corticosteroid treatment reduces the risk of future relapses, while patients with residual cognitive dysfunction after the initial episode have an increased relapse risk.
The choice of an antiepileptic drug (AED) in patients with epilepsy is mainly based on efficacy and safety of each drug. However, these criteria of drug selection should be further evaluated ...according to the epileptic syndromes, and adjusted to the sex and age of the patient. Unfortunately, very few studies have been conducted based on these latter criteria. We conducted a survey on the management of epilepsy treatment in adults. This survey was undertaken in France, and led to the establishment of a French consensus on antiepileptic drug treatment in adult patients with newly diagnosed epilepsy. Patients were grouped into 18 categories according to the epileptic syndrome (absence epilepsy, juvenile myoclonic epilepsy, undetermined idiopathic generalized epilepsy, symptomatic or cryptogenic partial epilepsy and unclassified epilepsy), and to the patient's gender and age. Our survey suggests that there is a consensus among French epileptologists for the choice of AEDs, mainly based on the epilepsy syndrome. Gender also plays a crucial role. Sodium valproate and lamotrigine are the two drugs of choice for generalized epilepsies, as well as for undetermined epilepsies. Lamotrigine is often prefered for women of childbearing age. First line AEDs in partial epilepsy are carbamazepine (particularly for men), lamotrigine (particularly for women), and gabapentin (in the elderly). In cases of failure and/or intolerance to one of these AED, the principal alternatives are oxcarbazepine, sodium valproate and topiramate.
Syncope in Wolff-Parkinson-White syndrome (WPW) may reveal an arrhythmic event, but also can be without relationships with WPW. Vagal-related syncope represents the most frequent cause of syncope in ...population. Electrophysiologic study (EPS) is recommended to evaluate the cause of syncope. The purpose of the study was to evaluate the influence of the age of the patient on the causes of syncope.
97 patients, 51 males, 46 females, mean age 35±17, with WPW were admitted for unexplained dizziness and/or syncope; they had no other heart disease and no documented arrhythmias. EPS consisted of atrial pacing and programmed atrial stimulation using 1 and 2 extrastimuli in control state and after isoproterenol.
29 patients were aged from 9 to 19 years (mean 15±1.4)(children and teenagers/group I). 44 were aged from 20 to 49 years (mean 34±8) (adults/group II). 24 were aged from 50 to 70 years (mean 60±8) (elderly/group III). A potentially malignant form (rapid conduction in accessory pathway (AP) >240bpm in control state or >300bpm after isoproterenol and atrial fibrillation or antidromic tachycardia induction) was noted in 10 group I patients (34%) more frequently than in group II patients (5/44) (11%) (p <0.017) and III (0/24) (0%)(p<0.001). Differences between group II and III were not significant (0.08). Orthodromic reentrant tachycardia was induced in 10 group I patients (34%), 20 group II patients (45%) (NS) and 15 group III patients (62.5%)(p<0.04) compared to group I. No tachycardia was induced in 9 group I patients (31%), 19 group II patients (43%)(NS) and 9 group III patients (37.5%)(NS).
Syncope in WPW syndrome frequently is directly related to the prexcitation. The cause can be a reentrant tachycardia complicated by a vagal reaction or a rapid atrial fibrillation or tachycardia. The malignant forms as a cause of syncope were frequently noted in children and teenagers, rarely in adults and never in elderly. The management of syncope in children and teenagers with a preexcitation syndrome requires urgently an electrophysiologic study.
BACKGROUND AND OBJECTIVESWhile patients with paraneoplastic autoimmune encephalitis (AE) with gamma-aminobutyric-acid B receptor antibodies (GABABR-AE) have poor functional outcomes and high ...mortality, the prognosis of nonparaneoplastic cases has not been well studied.METHODSPatients with GABABR-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples.RESULTSA total of 111 patients (44/111 40% women) were enrolled, including 84 of 111 (76%) paraneoplastic and 18 of 111 (16%) nonparaneoplastic cases (cancer status was undetermined for 9 patients). Patients presented with seizures (88/111 79%), cognitive impairment (54/111 49%), and/or behavioral disorders (34/111 31%), and 54 of 111 (50%) were admitted in intensive care unit (ICU). Nonparaneoplastic patients were significantly younger (median age 54 years range 19-88 vs 67 years range 50-85 for paraneoplastic cases, p < 0.001) and showed a different demographic distribution. Nonparaneoplastic patients more often had CSF pleocytosis (17/17 100% vs 58/78 74%, p = 0.02), were almost never associated with KTCD16-abs (1/16 6% vs 61/70 87%, p < 0.001), and were more frequently treated with second-line immunotherapy (11/18 61% vs 18/82 22%, p = 0.003). However, no difference of IgG subclass or HLA association was observed, although sample size was small (10 and 26 patients, respectively). After treatment, neurologic outcome was favorable (mRS ≤2) for 13 of 16 (81%) nonparaneoplastic and 37 of 84 (48%) paraneoplastic cases (p = 0.03), while 3 of 18 (17%) and 42 of 83 (51%) patients had died at last follow-up (p = 0.008), respectively. Neurologic outcome no longer differed after adjustment for confounding factors but seemed to be negatively associated with increased age and ICU admission. A better survival was associated with nonparaneoplastic cases, a younger age, and the use of immunosuppressive drugs.DISCUSSIONNonparaneoplastic GABABR-AE involved younger patients without associated KCTD16-abs and carried better neurologic and vital prognoses than paraneoplastic GABABR-AE, which might be due to a more intensive treatment strategy. A better understanding of immunologic mechanisms underlying both forms is needed.
While patients with paraneoplastic autoimmune encephalitis (AE) with gamma-aminobutyric-acid B receptor antibodies (GABA
R-AE) have poor functional outcomes and high mortality, the prognosis of ...nonparaneoplastic cases has not been well studied.
Patients with GABA
R-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples.
A total of 111 patients (44/111 40% women) were enrolled, including 84 of 111 (76%) paraneoplastic and 18 of 111 (16%) nonparaneoplastic cases (cancer status was undetermined for 9 patients). Patients presented with seizures (88/111 79%), cognitive impairment (54/111 49%), and/or behavioral disorders (34/111 31%), and 54 of 111 (50%) were admitted in intensive care unit (ICU). Nonparaneoplastic patients were significantly younger (median age 54 years range 19-88 vs 67 years range 50-85 for paraneoplastic cases,
< 0.001) and showed a different demographic distribution. Nonparaneoplastic patients more often had CSF pleocytosis (17/17 100% vs 58/78 74%,
= 0.02), were almost never associated with KTCD16-abs (1/16 6% vs 61/70 87%,
< 0.001), and were more frequently treated with second-line immunotherapy (11/18 61% vs 18/82 22%,
= 0.003). However, no difference of IgG subclass or HLA association was observed, although sample size was small (10 and 26 patients, respectively). After treatment, neurologic outcome was favorable (mRS ≤2) for 13 of 16 (81%) nonparaneoplastic and 37 of 84 (48%) paraneoplastic cases (
= 0.03), while 3 of 18 (17%) and 42 of 83 (51%) patients had died at last follow-up (
= 0.008), respectively. Neurologic outcome no longer differed after adjustment for confounding factors but seemed to be negatively associated with increased age and ICU admission. A better survival was associated with nonparaneoplastic cases, a younger age, and the use of immunosuppressive drugs.
Nonparaneoplastic GABA
R-AE involved younger patients without associated KCTD16-abs and carried better neurologic and vital prognoses than paraneoplastic GABA
R-AE, which might be due to a more intensive treatment strategy. A better understanding of immunologic mechanisms underlying both forms is needed.
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