Coeliac disease,epilepsy, and cerebral calcifications: association with TG6 autoantibodies

A 4‐year‐old boy presented with occipital seizures but normal initial neuroimaging and proved refractory to antiepileptic medications. On repeat neuroimaging after 1 year, he had developed bi‐occipital calcification and was then found to have positive coeliac serology. He was diagnosed with coeliac disease, epilepsy, and cerebral calcifications (CEC) and became seizure free after starting the gluten‐free diet. Positive antibody binding to neurons and glia was demonstrated on indirect immunofluorescence. High levels of immunoglobulin‐A directed against transglutaminase isoenzyme 6 (TG6) were found in the patient’s serum. The positive response to the diet, TG6 antibodies, and neuronal antibody binding suggest that CEC might be autoimmune in nature, as in other extra‐intestinal manifestations of gluten‐related diseases, such as gluten ataxia.     

THE GRIEF EVALUATION MEASURE (GEM): AN INITIAL VALIDATION STUDY

ABSTRACT

This article describes the development of the Grief Evaluation Measure (GEM), a new instrument designed to screen for the development of a complicated mourning response in a bereaved adult. The GEM provides a quantitative and qualitative assessment of risk factors, including the mourner's loss and medical history, coping resources before and after the death, and circumstances surrounding the death. It is designed to provide an in-depth evaluation of the bereaved adult's subjective grief experience and associated symptoms. Reliability and validity studies were conducted with two samples of bereaved adults (n = 23 and n = 92, respectively) from various clinical and support settings. Data on the two central sections of the GEM that assess the mourner's grief response and the level of symptomatology are described. Results indicate that the GEM's internal consistency and test–retest reliability are high. The GEM demonstrates good concurrent validity for established measures of bereavement, trauma, and physical and psychiatric symptoms, and good predictive validity for mourner adjustment one year after initial assessment. Plans for future development and an invitation for other researchers to collaborate with research on the GEM are also discussed.     

Just How Stable Are Escape Rhythms after Atrioventricular Junction Ablation?

Background: Atrioventricular (AV) node ablation with implantation of a permanent pacemaker is an established mode of therapy in the treatment of atrial fibrillation. However, concern exists regarding subsequent dependency on an entirely paced rhythm and the possible sequela of unheralded pacemaker failure. Data regarding escape rhythm lability, an important feature of pacemaker dependency, are limited. Aims and Methods: The purpose of this study was twofold: (1) to determine the characteristics of escape rhythms at predefined serial time intervals following AV node ablation and pacemaker implantation, and (2) to identify risk factors predictive of unstable escape rhythms. Patients undergoing AV node ablation and pacemaker implantation were assessed for the presence or absence of an escape rhythm during pacemaker interrogation at five predetermined serial time points. Baseline demographics and comorbid conditions were evaluated as potential predictors of those with labile escape rhythms. Results : Seventy‐nine percent of the 96 patients studied had an underlying escape rhythm (≥30 beats per minute) immediately postablation. Although the percentage of patients with an escape rhythm increased at each follow‐up interval, the number of patients who consistently demonstrated an escape rhythm declined with each follow‐up, with 28% of patients lacking an escape rhythm at some time point, i.e., labile escape rhythm. There were no significant predictors of a labile escape rhythm. Conclusion: Among patients who have undergone AV node ablation and pacemaker implantation, 72% have a stable escape rhythm over time, but others are at risk for pacemaker dependency, as predicted by an underlying absent or labile escape rhythm. (PACE 2010; 939–944)     

Free testosterone levels in umbilical‐cord blood predict infant head circumference in females

Aim Fetal androgens influence fetal growth as well as postnatal neurocognitive ability. However, to our knowledge, no published study has prospectively examined the impact of early‐life androgens on infant brain growth. We report the association between circulating fetal androgen levels, measured from umbilical‐cord blood at birth, and a proxy measure of brain growth: head circumference. Method Participants were 82 unselected female infants from a large representative birth cohort (mean gestational age 39.4wks, SD 1.7). Umbilical‐cord blood was obtained at birth and analysed for androgen concentrations (total testosterone, androstenedione, dehyrdroepiandrosterone, and its sulphated metabolite). Head circumference and two other measures of growth – weight (mean 3311.4g, SD 461.3) and length – were measured within 3 days of birth and again at approximately 1 year of age (mean age 13.1mo, SD 1.1). Results Multivariate linear regressions found an inverse association between levels of free testosterone and growth in head circumference (correlation=?.24), even when adjusting for sociodemographic/obstetric covariates and head size at birth. Growth in weight and length could not be predicted by free testosterone concentration. Interpretation This is the first report of an association between prenatal androgen levels and postnatal growth in head circumference. These findings suggest that early‐life androgens may impact brain development during infancy.     

Image Integration Using Intracardiac Ultrasound and 3D Reconstruction for Scar Mapping and Ablation of Ventricular Tachycardia

Background: Ablation of ventricular tachycardia (VT) reduces implantable cardioverter defibrillator shocks. Intracardiac ultrasound (ICE) can visualize and quantify the function of all left ventricular wall segments. We thus hypothesized that ICE could identify scar tissue and provide a guide to facilitate substrate‐guided VT ablation. Methods: Eighteen patients underwent VT ablation with real time ICE mapping from the right atrium and ventricle with online 3D‐image reconstruction of scar segments. The left ventricle was also scar mapped by traditional electroanatomic mapping (CARTO) for comparison. Images from these 2 scar mapping techniques were compared to each other as well as to a preprocedure transthoracic echocardiogram. Results: The average age was 65 ± 12 years and 12 (67%) were male (15 [83%] had ischemic cardiomyopathy). Two patients (12%) had recurrence of their clinical VT (1 remained on an antiarrhythmic medication, the other had a repeat ablation) over a follow‐up of 127 ± 33 days. No periprocedural or long‐term adverse events occurred. A total of 248 wall segments were analyzed. All 3 modalities were concordant in scar identification in 193 (78%) segments. The ICE segments correlated with the electroanatomic map in 213 (86%) segments versus 198 (80%), which correlated with transthoracic echocardiography and electroanatomic mapping (P = 0.046). Specifically, the ICE wall motion scores were closer to the electroanatomic mapping in the basal segments and showed a higher accuracy in ischemic heart disease. Conclusion: These data demonstrate that real time ICE images provide accurate chamber geometries and scar boundaries of the left ventricle. These scar borders were more accurate than transthoracic echocardiography and illustrate the feasibility of ICE for substrate‐based ablation for VT. (J Cardiovasc Electrophysiol, Vol. 21, pp. 678‐684, June 2010)