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https://www.readbyqxmd.com/read/28701502/wrist-sensor-reveals-sympathetic-hyperactivity-and-hypoventilation-before-probable-sudep
#1
Rosalind W Picard, Matteo Migliorini, Chiara Caborni, Francesco Onorati, Giulia Regalia, Daniel Friedman, Orrin Devinsky
No abstract text is available yet for this article.
July 12, 2017: Neurology
https://www.readbyqxmd.com/read/28691204/mortality-with-brainstem-seizures-from-focal-4-aminopyridine-induced-recurrent-hippocampal-seizures
#2
Muhammad Tariqus Salam, Gaspard Montandon, Roman Genov, Orrin Devinsky, Martin Del Campo, Peter L Carlen
OBJECTIVE: Sudden unexplained death in epilepsy is the leading cause of death in young adult epilepsy patients, typically occurring during the early postictal period, presumably resulting from brainstem and cardiorespiratory dysfunction. We hypothesized that ictal discharges in the brainstem disrupt the cardiorespiratory network, causing mortality. To study this hypothesis, we chose an animal model comprising focal unilateral hippocampal injection of 4-aminopyridine (4-AP), which produced focal recurrent hippocampal seizures with secondary generalization in awake, behaving rats...
July 10, 2017: Epilepsia
https://www.readbyqxmd.com/read/28675558/clinical-studies-and-anti-inflammatory-mechanisms-of-treatments
#3
Jacqueline A French, Matthias Koepp, Yvonne Naegelin, Federico Vigevano, Stéphane Auvin, Jong M Rho, Evan Rosenberg, Orrin Devinsky, Peder S Olofsson, Marc A Dichter
In this exciting era, we are coming closer and closer to bringing an anti-inflammatory therapy to the clinic for the purpose of seizure prevention, modification, and/or suppression. At present, it is unclear what this approach might entail, and what form it will take. Irrespective of the therapy that ultimately reaches the clinic, there will be some commonalities with regard to clinical trials. A number of animal models have now been used to identify inflammation as a major underlying mechanism of both chronic seizures and the epileptogenic process...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28643795/the-genomic-landscape-of-tuberous-sclerosis-complex
#4
Katie R Martin, Wanding Zhou, Megan J Bowman, Juliann Shih, Kit Sing Au, Kristin E Dittenhafer-Reed, Kellie A Sisson, Julie Koeman, Daniel J Weisenberger, Sandra L Cottingham, Steven T DeRoos, Orrin Devinsky, Mary E Winn, Andrew D Cherniack, Hui Shen, Hope Northrup, Darcy A Krueger, Jeffrey P MacKeigan
Tuberous sclerosis complex (TSC) is a rare genetic disease causing multisystem growth of benign tumours and other hamartomatous lesions, which leads to diverse and debilitating clinical symptoms. Patients are born with TSC1 or TSC2 mutations, and somatic inactivation of wild-type alleles drives MTOR activation; however, second hits to TSC1/TSC2 are not always observed. Here, we present the genomic landscape of TSC hamartomas. We determine that TSC lesions contain a low somatic mutational burden relative to carcinomas, a subset feature large-scale chromosomal aberrations, and highly conserved molecular signatures for each type exist...
June 15, 2017: Nature Communications
https://www.readbyqxmd.com/read/28633090/pulmonary-and-cardiac-pathology-in-sudden-unexpected-death-in-epilepsy-sudep
#5
REVIEW
Fábio A Nascimento, Zian H Tseng, Cristian Palmiere, Joseph J Maleszewski, Takayuki Shiomi, Aileen McCrillis, Orrin Devinsky
OBJECTIVE: To review studies on structural pulmonary and cardiac changes in SUDEP cases as well as studies showing pulmonary or cardiac structural changes in living epilepsy patients. METHODS: We conducted electronic literature searches using the PubMed database for articles published in English, regardless of publication year, that included data on cardiac and/or pulmonary structural abnormalities in SUDEP cases or in living epilepsy patients during the postictal period...
June 17, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28617940/quality-of-life-in-childhood-epilepsy-in-pediatric-patients-enrolled-in-a-prospective-open-label-clinical-study-with-cannabidiol
#6
Evan C Rosenberg, Jay Louik, Erin Conway, Orrin Devinsky, Daniel Friedman
Recent clinical trials indicate that cannabidiol (CBD) may reduce seizure frequency in pediatric patients with certain forms of treatment-resistant epilepsy. Many of these patients experience significant impairments in quality of life (QOL) in physical, mental, and social dimensions of health. In this study, we measured the caregiver-reported Quality of Life in Childhood Epilepsy (QOLCE) in a subset of patients enrolled in a prospective, open-label clinical study of CBD. Results from caregivers of 48 patients indicated an 8...
June 15, 2017: Epilepsia
https://www.readbyqxmd.com/read/28591836/focal-cortical-anomalies-and-language-impairment-in-16p11-2-deletion-and-duplication-syndrome
#7
Karen Blackmon, Thomas Thesen, Sophie Green, Emma Ben-Avi, Xiuyuan Wang, Benjamin Fuchs, Ruben Kuzniecky, Orrin Devinsky
Individuals with copy number variants (CNV) in the 16p11.2 chromosomal region are at high risk for language disorders. We investigate whether the extent and location of focal cortical anomalies are associated with language impairment in individuals with 16p11.2 CNVs. High-resolution T1-weighted MRI scans from 30 16p11.2 deletion (16p-del), 25 16p11.2 duplication (16p-dup), and 90 noncarrier controls (NCC) were analyzed to derive personalized cortical anomaly maps through single-case cortical thickness (CT) comparison to age-matched normative samples...
June 7, 2017: Cerebral Cortex
https://www.readbyqxmd.com/read/28538134/trial-of-cannabidiol-for-drug-resistant-seizures-in-the-dravet-syndrome
#8
RANDOMIZED CONTROLLED TRIAL
Orrin Devinsky, J Helen Cross, Linda Laux, Eric Marsh, Ian Miller, Rima Nabbout, Ingrid E Scheffer, Elizabeth A Thiele, Stephen Wright
BACKGROUND: The Dravet syndrome is a complex childhood epilepsy disorder that is associated with drug-resistant seizures and a high mortality rate. We studied cannabidiol for the treatment of drug-resistant seizures in the Dravet syndrome. METHODS: In this double-blind, placebo-controlled trial, we randomly assigned 120 children and young adults with the Dravet syndrome and drug-resistant seizures to receive either cannabidiol oral solution at a dose of 20 mg per kilogram of body weight per day or placebo, in addition to standard antiepileptic treatment...
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28513070/influence-of-quercetin-on-the-interaction-of-gliclazide-with-human-serum-albumin-spectroscopic-and-docking-approaches
#9
Miroslava Kameníková, Paul Georg Furtmüller, Mária Klacsová, Arturo Lopez-Guzman, José Luis Toca-Herrera, Anika Vitkovská, Ferdinand Devínsky, Pavel Mučaji, Milan Nagy
Protein-binding interactions are displacement reactions which have been implicated as the causative mechanisms in many drug-drug interactions. Thus, the aim of presented study was to analyse human serum albumin-binding displacement interaction between two ligands, hypoglycaemic drug gliclazide and widely distributed plant flavonoid quercetin. Fluorescence analysis was used in order to investigate the effect of substances on intrinsic fluorescence of human serum albumin (HSA) and to define binding and quenching properties of ligand-albumin complexes in binary and ternary systems, respectively...
May 16, 2017: Luminescence: the Journal of Biological and Chemical Luminescence
https://www.readbyqxmd.com/read/28496353/early-manifestations-of-renal-disease-in-patients-with-tuberous-sclerosis-complex
#10
Laura Malaga-Dieguez, Robert Spencer, Laura J Pehrson, Suzanne Vento, Kimberly Menzer, Orrin Devinsky, Howard Trachtman
OBJECTIVES: Renal manifestations are the second most significant cause of morbidity and mortality in patients with tuberous sclerosis complex (TSC), and include renal cysts, angiomyolipomas, fat-poor lesions, and malignant tumors. These lesions begin in childhood and often lead to chronic kidney disease (CKD). Little is known on the incidence of early modifiable risk factors of CKD, such as proteinuria and hypertension, or subtle decreases in glomerular filtration rate that correspond to the early stages of CKD in children with TSC...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28464309/psychiatric-and-behavioral-comorbidities-in-epilepsy-a-critical-reappraisal
#11
REVIEW
Anne T Berg, Hamada H Altalib, Orrin Devinsky
Psychiatric and behavioral disorders are important aspects of epilepsy and have received increasing attention in the last several years. The literature upon which most of the field relies contains some biases that must be carefully examined and resolved in future studies. First, in the pediatric epilepsy literature, many reports find that children with epilepsy have high levels of behavioral and psychiatric disorders when compared to appropriate controls. Most of these studies rely on parent-proxy completed instruments to assess these behavioral endpoints...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28456095/a-survey-of-medical-examiner-death-certification-of-vignettes-on-death-in-epilepsy-gaps-in-identifying-sudep
#12
Daniel S Atherton, Gregory G Davis, Cyndi Wright, Orrin Devinsky, Dale Hesdorffer
OBJECTIVE: Lack of standardized terminology on death certificates (DCs) of SUDEP type cases may obscure the presence of epilepsy in these deaths. Most DCs for individuals dying unexpectedly with epilepsy are certified by medical examiners (MEs). The purpose of this study was to gauge death certification practices of MEs when interpreting SUDEP cases and assess implications for valid surveillance of SUDEP. MATERIALS AND METHODS: A survey consisting of clinical vignettes describing deaths in individuals with epilepsy was sent to medical examiners...
April 22, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28438541/synthesis-structural-characterization-and-biological-activity-of-novel-knoevenagel-condensates-on-dld-1-human-colon-carcinoma
#13
Lucia Rišiaňová, Eva Fischer-Fodor, Jindra Valentová, Corina Tatomir, Nicoleta Corina Decea, Piroska Virag, Iveta Pechová, Ferdinand Devínsky, Natalia Miklašová
Biologically active Knoevenagel condensates (1-14) of diarylheptanoids: 1,7-bis(3-methoxy-4-hydroxyphenyl)hepta-1,7-diene-3,5-dione and 1,7-bis(3-ethoxy-4-hydroxyphenyl)hepta-1,7-diene-3,5-dione, were synthesized and structurally characterized. Compounds 1-14 exhibited cytotoxicity against colon carcinoma cells, and their antiproliferative effect was associated with a significant decrease of multidrug resistance proteins. One of the underlying mechanisms of these effects is the reduction of intracellular and extracellular SOD enzymes by compounds 1, 12 and 14, which render the tumor cells more vulnerable to oxidative stress...
April 13, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28427020/experiences-with-premorbid-sudep-discussion-among-participants-in-the-north-american-sudep-registry-nasr
#14
Jay Louik, Kyra Doumlele, Fizza Hussain, Laura Crandall, Jeffrey Buchhalter, Dale Hesdorffer, Elizabeth Donner, Orrin Devinsky, Daniel Friedman
The North American SUDEP Registry (NASR) is a repository of clinical data and biospecimens in cases of sudden unexpected death in epilepsy (SUDEP), a leading cause of epilepsy-related deaths. We assessed whether bereaved families were aware of SUDEP before their family member's death and their preferences for SUDEP disclosure. At enrollment, next-of-kin of SUDEP cases completed an intake interview, including questions assessing premorbid SUDEP discussions. Only 18.1% of the 138 next-of-kin recalled a previous discussion of SUDEP with a healthcare provider or support resource...
May 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28392266/cannabinoids-as-hippocampal-network-administrators
#15
REVIEW
Carl R Lupica, Yuhan Hu, Orrin Devinsky, Alexander F Hoffman
Extensive pioneering studies performed in the hippocampus have greatly contributed to our knowledge of an endogenous cannabinoid system comprised of the molecular machinery necessary to process these endocannabinoid lipid messengers and their associated cannabinoid receptors. Moreover, a foundation of knowledge regarding the function of hippocampal circuits, and its role in supporting synaptic plasticity has facilitated our understanding of the roles cannabinoids play in the diverse behaviors in which the hippocampus participates in both normal and pathological states...
April 6, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28384699/sudden-unexpected-death-in-epilepsy-among-patients-with-benign-childhood-epilepsy-with-centrotemporal-spikes
#16
Kyra Doumlele, Daniel Friedman, Jeffrey Buchhalter, Elizabeth J Donner, Jay Louik, Orrin Devinsky
Importance: Children with benign epilepsy with centrotemporal spikes (BECTS) have traditionally been considered to have a uniformly good prognosis. However, benign may be a misnomer because BECTS is linked to cognitive deficits, a more severe phenotype with intractable seizures, and the potential for sudden unexpected death in epilepsy (SUDEP). Objective: To determine if cases of BECTS are present in the North American SUDEP Registry (NASR). Design, Setting, and Participants: The NASR is a clinical and biospecimen repository established in 2011 to promote SUDEP research...
June 1, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28364691/incidence-of-seizures-following-initial-ischemic-stroke-in-a-community-based-cohort-the-framingham-heart-study
#17
Maria Stefanidou, Rohit R Das, Alexa S Beiser, Banu Sundar, Margaret Kelly-Hayes, Carlos S Kase, Orrin Devinsky, Sudha Seshadri, Daniel Friedman
PURPOSE: We examined the incidence of seizures following ischemic stroke in a community-based sample. METHODS: All subjects with incident ischemic strokes in the Framingham Original and Offspring cohorts between 1982 and 2003 were identified and followed for up to 20 years to determine incidence of seizures. Seizure-type was based on the 2010 International League Against Epilepsy (ILAE) classification. Disability was stratified into mild/none, moderate and severe, based on post-stroke neurological deficit documentation according to the Framingham Heart Study (FHS) protocol and functional status was determined using the Barthel Index...
April 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28358339/novel-palladium-ii-complexes-that-influence-prominin-1-cd133-expression-and-stem-cell-factor-release-in-tumor-cells
#18
Eva Fischer-Fodor, Roman Mikláš, Lucia Rišiaňová, Mihai Cenariu, Ioana-Georgeta Grosu, Piroska Virag, Maria Perde-Schrepler, Ciprian Tomuleasa, Ioana Berindan-Neagoe, Ferdinand Devínsky, Natalia Miklášová
New Pd(II) complexes of 1,7-bis(2-methoxyphenyl)hepta-1,6-diene-3,5-dione were synthesized and structurally characterized. The complexes were tested in vitro on human colon and hepatic carcinoma cell lines, normal hepatic cells and hematopoietic progenitor cells. Biological tests proved that Pd(II) complexes 1 and 2 (containing a curcumin derivative) exhibit a strong in vitro antitumor effect against the cells derived from human colorectal carcinoma and the hepatic metastasis of a colorectal carcinoma. Complex 1 has an outstanding inhibitory effect against BRAF-mutant colon carcinoma and hepatocarcinoma cell growth; 1 and 2 are both more active than the free ligand and have the capacity to trigger early apoptotic processes...
March 30, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28290070/parieto-frontal-gyrification-and-working-memory-in-healthy-adults
#19
Sophie Green, Karen Blackmon, Thomas Thesen, Jonathan DuBois, Xiuyuan Wang, Eric Halgren, Orrin Devinsky
Gyrification of the cortical mantle is a dynamic process that increases with cortical surface area and decreases with age. Increased gyrification is associated with higher scores on cognitive tasks in adults; however, the degree to which this relationship is independent of cortical surface area remains undefined. This study investigates whether regional variation in gyrification is associated with domain-general and domain-specific cognition. Our hypothesis is that increased local gyrification confers a functional advantage that is independent of surface area...
March 13, 2017: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/28284051/amygdala-enlargement-temporal-lobe-epilepsy-subtype-or-nonspecific-finding
#20
Anny Reyes, Thomas Thesen, Ruben Kuzniecky, Orrin Devinsky, Carrie R McDonald, Graeme D Jackson, David N Vaughan, Karen Blackmon
OBJECTIVE: Amygdala enlargement (AE) is observed in patients with temporal lobe epilepsy (TLE), which has led to the suggestion that it represents a distinct TLE subtype; however, it is unclear whether AE is found at similar rates in other epilepsy syndromes or in healthy controls, which would limit its value as a marker for focal epileptogenicity. METHODS: We compared rates of AE, defined quantitatively from high-resolution T1-weighted MRI, in a large multi-site sample of 136 patients with nonlesional localization related epilepsy (LRE), including TLE and extratemporal (exTLE) focal epilepsy, 34 patients with idiopathic generalized epilepsy (IGE), and 233 healthy controls (HCs)...
May 2017: Epilepsy Research
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