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JOURNAL ARTICLE
Marta Amengual-Gual, Iván Sánchez Fernández, Alejandra Vasquez, Nicholas S Abend, Anne Anderson, Ravindra Arya, Cristina Barcia Aguilar, J Nicholas Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Tracy A Glauser, Joshua L Goldstein, Howard P Goodkin, Yi-Chen Lai, Mohamad A Mikati, Lindsey A Morgan, Edward J Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, Latania Reece, James J Riviello, Kumar Sannagowdara, Theodore Sheehan, Robert C Tasker, Dmitry Tchapyjnikov, Alexis A Topjian, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
PURPOSE: Delayed treatment in status epilepticus (SE) is independently associated with increased treatment resistance, morbidity, and mortality. We describe the prehospital management pathway and Emergency Medical Services (EMS) timeliness in children who developed refractory convulsive status epilepticus (RCSE). METHODS: Retrospective multicenter study in the United States using prospectively collected observational data from June 2011 to March 2020. We selected pediatric patients (one month-21 years) with RCSE initiated outside the hospital and transported to the hospital by EMS...
October 2023: Seizure: the Journal of the British Epilepsy Association
#2
JOURNAL ARTICLE
Katrina Peariso, Ravindra Arya, Tracy Glauser, Nicholas S Abend, Cristina Barcia Aguilar, Marta Amengual-Gual, Anne Anderson, Brian L Appavu, J Nicholas Brenton, Jessica Carpenter, Kevin E Chapman, Justice Clark, William D Gaillard, Marina Gaínza-Lein, Joshua Goldstein, Howard Goodkin, Zachary Grinspan, Rejean M Guerriero, Paul S Horn, Linda Huh, Robert Kahoud, Sarah A Kelley, Eric H Kossoff, Kush Kapur, Yi-Chen Lai, B Oyinkan Marquis, Tiffani McDonough, Mohamad A Mikati, Lindsey Morgan, Edward Novotny, Adam P Ostendorf, Eric T Payne, Juan Piantino, James Riviello, Tristan Sands, Carl E Stafstrom, Robert C Tasker, Dmitry Tchapyjnikov, Alejandra Vasquez, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
BACKGROUND AND OBJECTIVES: The objective of this study was to determine patient-specific factors known proximate to the presentation to emergency care associated with the development of refractory convulsive status epilepticus (RSE) in children. METHODS: An observational case-control study was conducted comparing pediatric patients (1 month-21 years) with convulsive SE whose seizures stopped after benzodiazepine (BZD) and a single second-line antiseizure medication (ASM) (responsive established status epilepticus [rESE]) with patients requiring more than a BZD and a single second-line ASM to stop their seizures (RSE)...
August 1, 2023: Neurology
#3
JOURNAL ARTICLE
Anita Arinda, Simple Ouma, Kenneth Kalani, Pratamesh Ramasubramanian, Tyler Johnson, Antoinette Charles, Colby Newson, Olivia Ratliff, Angelina Kakooza-Mwesige, Paula Njeru, Martin Kaddumukasa, Mark Kaddumukasa, Bradley Kolls, Aatif Husain, Dmitry Tchapyjnikov, Michael Haglund, Anthony Fuller, Neil Prose, Deborah Koltai
OBJECTIVE: This study evaluated an epilepsy training program for healthcare workers that was designed to improve their knowledge of epilepsy, its treatment, and its psychosocial effects. METHODS: This single group, before and after survey was conducted in three regional referral hospitals in Uganda. Healthcare workers participated in a 3-day epilepsy training program and were assessed immediately prior to and following the program using a 39-item epilepsy knowledge questionnaire...
November 26, 2022: Epilepsy & Behavior: E&B
#4
JOURNAL ARTICLE
Kate Stanley, Joseph Hostyk, Linh Tran, Marta Amengual-Gual, Patricia Dugan, Justice Clark, Hyunmi Choi, Dmitry Tchapyjnikov, Piero Perucca, Cecilia Fernandes, Danielle Andrade, Orrin Devinsky, Gianpiero L Cavalleri, Chantal Depondt, Arjune Sen, Terence O'Brien, Erin Heinzen, Tobias Loddenkemper, David B Goldstein, Mohamed A Mikati, Norman Delanty
Large international consortia examining the genomic architecture of the epilepsies focus on large diagnostic subgroupings such as "all focal epilepsy" and "all genetic generalized epilepsy". In addition, phenotypic data are generally entered into these large discovery databases in a unidirectional manner at one point in time only. However, there are many smaller phenotypic subgroupings in epilepsy, many of which may have unique genomic risk factors. Such a subgrouping or "microphenotype" may be defined as an uncommon or rare phenotype that is well recognized by epileptologists and the epilepsy community, and which may or may not be formally recognized within the International League Against Epilepsy classification system...
September 27, 2021: American Journal of Medical Genetics. Part A
#5
MULTICENTER STUDY
Theodore Sheehan, Marta Amengual-Gual, Alejandra Vasquez, Nicholas S Abend, Anne Anderson, Brian Appavu, Ravindra Arya, Cristina Barcia Aguilar, J Nicholas Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Marina Gaínza-Lein, Tracy A Glauser, Joshua L Goldstein, Howard P Goodkin, Réjean M Guerriero, Linda Huh, Michele Jackson, Kush Kapur, Robert Kahoud, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lindsey A Morgan, Edward J Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, Latania Reece, James J Riviello, Tristan T Sands, Kumar Sannagowdara, Renee Shellhaas, Garnett Smith, Robert C Tasker, Dmitry Tchapyjnikov, Alexis A Topjian, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Bo Zhang, Tobias Loddenkemper
OBJECTIVE: This study was undertaken to evaluate benzodiazepine (BZD) administration patterns before transitioning to non-BZD antiseizure medication (ASM) in pediatric patients with refractory convulsive status epilepticus (rSE). METHODS: This retrospective multicenter study in the United States and Canada used prospectively collected observational data from children admitted with rSE between 2011 and 2020. Outcome variables were the number of BZDs given before the first non-BZD ASM, and the number of BZDs administered after 30 and 45 min from seizure onset and before escalating to non-BZD ASM...
November 2021: Epilepsia
#6
JOURNAL ARTICLE
Marina Gaínza-Lein, Cristina Barcia Aguilar, Juan Piantino, Kevin E Chapman, Iván Sánchez Fernández, Marta Amengual-Gual, Anne Anderson, Brian Appavu, Ravindra Arya, James Nicholas Brenton, Jessica L Carpenter, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Tracy A Glauser, Joshua L Goldstein, Howard P Goodkin, Linda Huh, Robert Kahoud, Kush Kapur, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lindsey A Morgan, Anuranjita Nayak, Edward Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Latania Reece, James Riviello, Kumar Sannagowdara, Tristan T Sands, Theodore Sheehan, Robert C Tasker, Dmitry Tchapyjnikov, Alejandra Vasquez, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Bo Zhang, Tobias Loddenkemper
OBJECTIVE: This study was undertaken to describe long-term clinical and developmental outcomes in pediatric refractory status epilepticus (RSE) and identify factors associated with new neurological deficits after RSE. METHODS: We performed retrospective analyses of prospectively collected observational data from June 2011 to March 2020 on pediatric patients with RSE. We analyzed clinical outcomes from at least 30 days after RSE and, in a subanalysis, we assessed developmental outcomes and evaluated risk factors in previously normally developed patients...
September 2021: Epilepsia
#7
JOURNAL ARTICLE
Alejandra Vasquez, Raquel Farias-Moeller, Iván Sánchez-Fernández, Nicholas S Abend, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, James N Brenton, Jessica L Carpenter, Kevin Chapman, Justice Clark, William D Gaillard, Tracy Glauser, Joshua L Goldstein, Howard P Goodkin, Rejean M Guerriero, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lindsey A Morgan, Edward J Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, James J Riviello, Tristan T Sands, Kumar Sannagowdara, Robert C Tasker, Dmitry Tchapyjnikov, Alexis Topjian, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
OBJECTIVES: To characterize the pediatric super-refractory status epilepticus population by describing treatment variability in super-refractory status epilepticus patients and comparing relevant clinical characteristics, including outcomes, between super-refractory status epilepticus, and nonsuper-refractory status epilepticus patients. DESIGN: Retrospective cohort study with prospectively collected data between June 2011 and January 2019. SETTING: Seventeen academic hospitals in the United States...
December 1, 2021: Pediatric Critical Care Medicine
#8
JOURNAL ARTICLE
Claudine Sculier, Cristina Barcia Aguilar, Nicolas Gaspard, Marina Gaínza-Lein, Iván Sánchez Fernández, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, Brian T Burrows, James N Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, William D Gaillard, Tracy A Glauser, Joshua L Goldstein, Howard P Goodkin, Mark Gorman, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Anuranjita Nayak, Katrina Peariso, James Riviello, Allison Rusie, Katherine Sperberg, Coral M Stredny, Robert C Tasker, Dmitry Tchapyjnikov, Alejandra Vasquez, Mark S Wainwright, Angus A Wilfong, Korwyn Williams, Tobias Loddenkemper
OBJECTIVE: We aimed to characterize the clinical profile and outcomes of new onset refractory status epilepticus (NORSE) in children, and investigated the relationship between fever onset and status epilepticus (SE). METHODS: Patients with refractory SE (RSE) between June 1, 2011 and October 1, 2016 were prospectively enrolled in the pSERG (Pediatric Status Epilepticus Research Group) cohort. Cases meeting the definition of NORSE were classified as "NORSE of known etiology" or "NORSE of unknown etiology...
July 2021: Epilepsia
#9
JOURNAL ARTICLE
Cristina Barcia Aguilar, Marta Amengual-Gual, Iván Sánchez Fernández, Nicholas S Abend, Anne Anderson, Brian Appavu, Ravindra Arya, J Nicholas Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Marina Gaínza-Lein, Tracy Glauser, Joshua L Goldstein, Howard P Goodkin, Rejean M Guerriero, Linda Huh, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lyndsey A Morgan, Edward J Novotny, Adam Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, James Riviello, Kumar Sannagowdara, Theodore Sheehan, Tristan T Sands, Robert C Tasker, Dmitry Tchapyjnikov, Alexis A Topjian, Alejandra Vasquez, Mark S Wainwright, Angus A Wilfong, Korwyn Williams, Tobias Loddenkemper
BACKGROUND: Time to treatment in pediatric refractory status epilepticus is delayed. We aimed to evaluate the influence of weekends and holidays on time to treatment of this pediatric emergency. METHODS: We performed a retrospective analysis of prospectively collected observational data of pediatric patients with refractory status epilepticus. RESULTS: We included 329 patients (56% males) with a median (p25 to p75) age of 3.8 (1.3 to 9) years...
July 2021: Pediatric Neurology
#10
JOURNAL ARTICLE
Meredith Sooy, Rachel L Randell, Dmitry Tchapyjnikov, Klaus Werner, Kristina Nazareth-Pidgeon
A 4-year-old boy with atypical, complete DiGeorge and CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities and ear abnormalities) syndromes presented with frequent episodes of a painful, markedly erythematous eruption associated with swelling. Evaluation revealed non-specific findings on skin biopsy at the time of eruption and no pathogenic mutation in the SCN9A gene. The patient was diagnosed with secondary erythromelalgia based on clinical presentation. Erythromelalgia is a rare disorder characterised by recurrent episodes of pain and erythema typically affecting the distal extremities...
February 18, 2021: BMJ Case Reports
#11
JOURNAL ARTICLE
Steven P Trau, Emily C Sterrett, Lydia Feinstein, Linh Tran, William B Gallentine, Dmitry Tchapyjnikov
PURPOSE: Convulsive status epilepticus (CSE) is a medical emergency associated with high rates of morbidity and mortality. Although guidelines for CSE management recommend rapid treatment of seizures, prior studies show that compliance with these guidelines is low. In this study, we assessed if implementation of a paper-based clinical pathway for the treatment of CSE improves the timeliness and appropriate dosing of first and second line anti-seizure medications (ASM). METHODS: A non-digital CSE treatment protocol was implemented as part of a quality improvement initiative in 2016...
October 2020: Seizure: the Journal of the British Epilepsy Association
#12
JOURNAL ARTICLE
Alejandra Vasquez, Marina Gaínza-Lein, Nicholas S Abend, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, J Nicholas Brenton, Jessica L Carpenter, Kevin Chapman, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Tracy Glauser, Joshua L Goldstein, Howard P Goodkin, Rejean M Guerriero, Kush Kapur, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lindsey A Morgan, Edward J Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, James J Riviello, Kumar Sannagowdara, Robert C Tasker, Dmitry Tchapyjnikov, Alexis Topjian, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
OBJECTIVE: To identify factors associated with low benzodiazepine (BZD) dosing in patients with refractory status epilepticus (RSE) and to assess the impact of BZD treatment variability on seizure cessation. METHODS: This was a retrospective study with prospectively collected data of children with convulsive RSE admitted between June 2011 and January 2019. We analyzed the initial and total BZD dose within 10 minutes of treatment initiation. We used logistic regression modeling to evaluate predictors of low BZD dosing and multivariate Cox regression analysis to assess the impact of low BZD dosing on time to seizure cessation...
November 10, 2020: Neurology
#13
JOURNAL ARTICLE
Iván Sánchez Fernández, Nicholas S Abend, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, Cristina Barcia Aguilar, James Nicholas Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, Raquel Farias-Moeller, William D Gaillard, Marina Gaínza-Lein, Tracy Glauser, Joshua Goldstein, Howard P Goodkin, Réjean M Guerriero, Yi-Chen Lai, Tiffani McDonough, Mohamad A Mikati, Lindsey A Morgan, Edward Novotny, Eric Payne, Katrina Peariso, Juan Piantino, Adam Ostendorf, Tristan T Sands, Kumar Sannagowdara, Robert C Tasker, Dimtry Tchapyjnikov, Alexis A Topjian, Alejandra Vasquez, Mark S Wainwright, Angus Wilfong, Kowryn Williams, Tobias Loddenkemper
OBJECTIVE: To determine whether publication of evidence on delays in time to treatment shortens time to treatment in pediatric refractory convulsive status epilepticus (rSE), we compared time to treatment before (2011-2014) and after (2015-2019) publication of evidence of delays in treatment of rSE in the Pediatric Status Epilepticus Research Group (pSERG) as assessed by patient interviews and record review. METHODS: We performed a retrospective analysis of a prospectively collected dataset from June 2011 to September 2019 on pediatric patients (1 month-21 years of age) with rSE...
September 1, 2020: Neurology
#14
JOURNAL ARTICLE
Iván Sánchez Fernández, Marina Gaínza-Lein, Nicholas S Abend, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, J Nicholas Brenton, Jessica L Carpenter, Kevin E Chapman, Justice Clark, Raquel Farias-Moeller, William Davis Gaillard, Tracy A Glauser, Joshua Goldstein, Howard P Goodkin, Réjean M Guerriero, Kurt Hecox, Michele Jackson, Kush Kapur, Sarah A Kelley, Eric H W Kossoff, Yi-Chen Lai, Tiffani L McDonough, Mohamad A Mikati, Lindsey A Morgan, Edward J Novotny, Adam P Ostendorf, Eric T Payne, Katrina Peariso, Juan Piantino, James J Riviello, Kumar Sannagowdara, Carl E Stafstrom, Robert C Tasker, Dmitry Tchapyjnikov, Alexis A Topjian, Alejandra Vasquez, Mark S Wainwright, Angus Wilfong, Korwyn Williams, Tobias Loddenkemper
PURPOSE: To evaluate whether the onset of pediatric refractory status epilepticus (rSE) is related to time of day. METHOD: We analyzed the time of day for the onset of rSE in this prospective observational study performed from June 2011 to May 2019 in pediatric patients (1 month to 21 years of age). We evaluated the temporal distribution of pediatric rSE utilizing a cosinor analysis. We calculated the midline estimating statistic of rhythm (MESOR) and amplitude...
August 2019: Seizure: the Journal of the British Epilepsy Association
#15
MULTICENTER STUDY
Arnold J Sansevere, Ravindra Arya, Iván Sánchez Fernández, William D Gaillard, Robert C Tasker, Yi-Chen Lai, Anne E Anderson, Dmitry Tchapyjnikov, Kevin E Chapman, J Nicholas Brenton, Jessica L Carpenter, Marina Gaínza-Lein, Joshua L Goldstein, Howard P Goodkin, Michele C Jackson, Kush Kapur, Mohamad A Mikati, Katrina Peariso, Tracy A Glauser, Alexis A Topjian, Mark Wainwright, Angus A Wilfong, Korwyn L Williams, Tobias Loddenkemper, Nicholas S Abend
PURPOSE: We aimed to determine whether clinical EEG reports obtained from children in the intensive care unit with refractory status epilepticus could provide data for comparative effectiveness research studies. METHODS: We conducted a retrospective descriptive study to assess the documentation of key variables within clinical continuous EEG monitoring reports based on the American Clinical Neurophysiology Society's standardized EEG terminology for children with refractory status epilepticus from 10 academic centers...
September 2019: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
#16
JOURNAL ARTICLE
Dmitry Tchapyjnikov, Matthew W Luedke
Cefepime is a fourth-generation cephalosporin antibiotic known to have neurotoxic side effects. Recent reports have described patients on cefepime presenting with altered mentation and concurrent triphasic wave discharges on electroencephalogram (EEG). Some have described this clinical presentation as cefepime-induced encephalopathy, while others have termed it as cefepime-induced nonconvulsive status epilepticus (NCSE). We report on 4 patients who developed cefepime-associated altered mentation with triphasic discharges on EEG...
April 2019: Neurohospitalist
#17
JOURNAL ARTICLE
Jesse Slone, Yanyan Peng, Adam Chamberlin, Belinda Harris, Julie Kaylor, Marie T McDonald, Monica Lemmon, Mays Antonine El-Dairi, Dmitry Tchapyjnikov, Laura A Gonzalez-Krellwitz, Elizabeth A Sellars, Allyn McConkie-Rosell, Laura G Reinholdt, Taosheng Huang
Mitochondrial dysfunction lies behind many neurodegenerative disorders, owing largely to the intense energy requirements of most neurons. Such mitochondrial dysfunction may work through a variety of mechanisms, from direct disruption of the electron transport chain to abnormal mitochondrial biogenesis. Recently, we have identified biallelic mutations in the mitochondrial flavoprotein "ferredoxin reductase" (FDXR) gene as a novel cause of mitochondriopathy, peripheral neuropathy, and optic atrophy...
December 2018: Journal of Human Genetics
#18
COMPARATIVE STUDY
Alejandra Vasquez, Marina Gaínza-Lein, Iván Sánchez Fernández, Nicholas S Abend, Anne Anderson, J Nicholas Brenton, Jessica L Carpenter, Kevin Chapman, Justice Clark, William D Gaillard, Tracy Glauser, Joshua Goldstein, Howard P Goodkin, Yi-Chen Lai, Tobias Loddenkemper, Tiffani L McDonough, Mohamad A Mikati, Anuranjita Nayak, Eric Payne, James Riviello, Dmitry Tchapyjnikov, Alexis A Topjian, Mark S Wainwright, Robert C Tasker
OBJECTIVE: We aimed to evaluate and compare the status epilepticus treatment pathways used by pediatric status epilepticus research group (pSERG) hospitals in the United States and the American Epilepsy Society (AES) status epilepticus guideline. METHODS: We undertook a descriptive analysis of recommended timing, dosing, and medication choices in 10 pSERG hospitals' status epilepticus treatment pathways. RESULTS: One pathway matched the timeline in the AES guideline; nine pathways described more rapid timings...
September 2018: Pediatric Neurology
#19
MULTICENTER STUDY
Ravindra Arya, Katrina Peariso, Marina Gaínza-Lein, Jessica Harvey, Ann Bergin, J Nicholas Brenton, Brian T Burrows, Tracy Glauser, Howard P Goodkin, Yi-Chen Lai, Mohamad A Mikati, Iván Sánchez Fernández, Dmitry Tchapyjnikov, Angus A Wilfong, Korwyn Williams, Tobias Loddenkemper
PURPOSE: To describe the efficacy and safety of ketogenic diet (KD) for convulsive refractory status epilepticus (RSE). METHODS: RSE patients treated with KD at the 6/11 participating institutions of the pediatric Status Epilepticus Research Group from January-2011 to December-2016 were included. Patients receiving KD prior to the index RSE episode were excluded. RSE was defined as failure of ≥2 anti-seizure medications, including at least one non-benzodiazepine drug...
August 2018: Epilepsy Research
#20
MULTICENTER STUDY
I Sánchez Fernández, M Gaínza-Lein, N S Abend, A E Anderson, R Arya, J N Brenton, J L Carpenter, K E Chapman, J Clark, W D Gaillard, T A Glauser, J L Goldstein, H P Goodkin, A R Helseth, M C Jackson, K Kapur, Y-C Lai, T L McDonough, M A Mikati, A Nayak, K Peariso, J J Riviello, R C Tasker, D Tchapyjnikov, A A Topjian, M S Wainwright, A Wilfong, K Williams, T Loddenkemper
OBJECTIVE: To identify factors associated with treatment delays in pediatric patients with convulsive refractory status epilepticus (rSE). METHODS: This prospective, observational study was performed from June 2011 to March 2017 on pediatric patients (1 month to 21 years of age) with rSE. We evaluated potential factors associated with increased treatment delays in a Cox proportional hazards model. RESULTS: We studied 219 patients (53% males) with a median (25th-75th percentiles [p25 -p75 ]) age of 3...
May 8, 2018: Neurology
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