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https://www.readbyqxmd.com/read/28482736/impact-of-child-life-services-on-children-and-families-admitted-to-start-the-ketogenic-diet
#1
Eric H Kossoff, Lindsay Sutter, Sarah C Doerrer, Courtney A Haney, Zahava Turner
Traditionally the ketogenic diet is started as an inpatient admission to the hospital. Starting in January 2015, child life services were made formally available during ketogenic diet admissions to help families cope. One-page surveys were then provided to 15 parents on the day of discharge and again after 3 months. Every family believed that the child life services were helpful. Children who were developmentally appropriate/mildly delayed had higher parent-reported anxiety scores than those who were moderate to severely delayed (4...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28188074/complications-during-ketogenic-diet-initiation-prevalence-treatment-and-influence-on-seizure-outcomes
#2
Abigail Lin, Zahava Turner, Sarah C Doerrer, Anthony Stanfield, Eric H Kossoff
BACKGROUND: Many centers still admit children for several days to start the ketogenic diet. The exact incidence of adverse effects during the admission and their potential later impact on seizure reduction has not been widely studied. METHODS: We performed a retrospective study of children with intractable epilepsy electively admitted for ketogenic diet initiation at our institution from 2011 to 2016. Charts were reviewed for adverse effects during the admission period and then examined for seizure reduction and compliance at three months...
March 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28188045/treatment-issues-for-children-with-epilepsy-transitioning-to-adult-care
#3
REVIEW
Rima Nabbout, Carol S Camfield, Danielle M Andrade, Alexis Arzimanoglou, Catherine Chiron, Joyce A Cramer, Jacqueline A French, Eric Kossoff, Marco Mula, Peter R Camfield
This is the third of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper focuses on treatment issues that arise during the course of childhood epilepsy and make the process of transition to adult care more complicated. Some AEDs used during childhood, such as stiripentol, vigabatrin, and cannabidiol, are unfamiliar to adult epilepsy specialists. In addition, new drugs are being developed for treatment of specific childhood onset epilepsy syndromes and have no indication yet for adults...
April 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28179470/phase-i-ii-multicenter-ketogenic-diet-study-for-adult-superrefractory-status-epilepticus
#4
MULTICENTER STUDY
Mackenzie C Cervenka, Sara Hocker, Matthew Koenig, Barak Bar, Bobbie Henry-Barron, Eric H Kossoff, Adam L Hartman, John C Probasco, David R Benavides, Arun Venkatesan, Eliza C Hagen, Denise Dittrich, Tracy Stern, Batya Radzik, Marie Depew, Filissa M Caserta, Paul Nyquist, Peter W Kaplan, Romergryko G Geocadin
OBJECTIVE: To investigate the feasibility, safety, and efficacy of a ketogenic diet (KD) for superrefractory status epilepticus (SRSE) in adults. METHODS: We performed a prospective multicenter study of patients 18 to 80 years of age with SRSE treated with a KD treatment algorithm. The primary outcome measure was significant urine and serum ketone body production as a biomarker of feasibility. Secondary measures included resolution of SRSE, disposition at discharge, KD-related side effects, and long-term outcomes...
March 7, 2017: Neurology
https://www.readbyqxmd.com/read/28110175/ketogenic-diet-therapy-for-epilepsy-during-pregnancy-a-case-series
#5
Elles J T M van der Louw, Tanya J Williams, Bobbie J Henry-Barron, Joanne F Olieman, Johannes J Duvekot, Marijn J Vermeulen, Natalja Bannink, Monique Williams, Rinze F Neuteboom, Eric H Kossoff, Coriene E Catsman-Berrevoets, Mackenzie C Cervenka
PURPOSE: Evaluation of ketogenic diet (KD) therapies for seizure control during pregnancy when safety and appropriate management become considerations. Until now, no information has been available on seizure reduction and human pregnancy related outcomes in women treated with KD therapies. METHOD: We describe two cases of pregnant women with epilepsy treated with KD therapy either as monotherapy (Case 1) or as adjunctive therapy (Case 2). RESULTS: Case 1: A 27 year old woman, gravida1, started the classic KD with medium chain triglyceride (MCT) emulsion and 75g carbohydrate-restriction, later reduced to 47g...
February 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28053859/is-there-a-role-for-diet-monotherapy-in-adult-epilepsy
#6
Mackenzie C Cervenka, Bobbie J Henry-Barron, Eric H Kossoff
Ten adults were treated with ketogenic diet monotherapy for epilepsy. Four patients were naïve to antiseizure drugs (ASDs), and six previously tried and stopped ASDs. Of four treatment-naïve participants, two (50%) were free from disabling seizures on Modified Atkins Diet (MAD) monotherapy for > 1 year. Two (50%) stopped. Four of six patients (67%) who had previously tried ASDs became seizure-free on diet monotherapy, and two experienced > 50% seizure reduction. Side effects included amenorrhea, weight loss, osteoporosis, and hyperlipidemia...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/27710878/how-effective-is-the-ketogenic-diet-for-electrical-status-epilepticus-of-sleep
#7
REVIEW
Sarah Aminoff Kelley, Eric Heath Kossoff
Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation...
November 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27665735/the-molecular-and-phenotypic-spectrum-of-iqsec2-related-epilepsy
#8
Ayelet Zerem, Kazuhiro Haginoya, Dorit Lev, Lubov Blumkin, Sara Kivity, Ilan Linder, Cheryl Shoubridge, Elizabeth Emma Palmer, Michael Field, Jackie Boyle, David Chitayat, William D Gaillard, Eric H Kossoff, Marjolaine Willems, David Geneviève, Frederic Tran-Mau-Them, Orna Epstein, Eli Heyman, Sarah Dugan, Alice Masurel-Paulet, Ame'lie Piton, Tjitske Kleefstra, Rolph Pfundt, Ryo Sato, Andreas Tzschach, Naomichi Matsumoto, Hirotomo Saitsu, Esther Leshinsky-Silver, Tally Lerman-Sagie
OBJECTIVE: IQSEC2 is an X-linked gene associated with intellectual disability (ID) and epilepsy. Herein we characterize the epilepsy/epileptic encephalopathy of patients with IQSEC2 pathogenic variants. METHODS: Forty-eight patients with IQSEC2 variants were identified worldwide through Medline search. Two patients were recruited from our early onset epileptic encephalopathy cohort and one patient from personal communication. The 18 patients who have epilepsy in addition to ID are the subject of this study...
November 2016: Epilepsia
https://www.readbyqxmd.com/read/27615012/response-to-second-treatment-after-initial-failed-treatment-in-a-multicenter-prospective-infantile-spasms-cohort
#9
MULTICENTER STUDY
Kelly G Knupp, Erin Leister, Jason Coryell, Katherine C Nickels, Nicole Ryan, Elizabeth Juarez-Colunga, William D Gaillard, John R Mytinger, Anne T Berg, John Millichap, Douglas R Nordli, Sucheta Joshi, Renée A Shellhaas, Tobias Loddenkemper, Dennis Dlugos, Elaine Wirrell, Joseph Sullivan, Adam L Hartman, Eric H Kossoff, Zachary M Grinspan, Lorie Hamikawa
OBJECTIVE: Infantile spasms (IS) represent a severe epileptic encephalopathy presenting in the first 2 years of life. Recommended first-line therapies (hormonal therapy or vigabatrin) often fail. We evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome. METHODS: The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of IS...
November 2016: Epilepsia
https://www.readbyqxmd.com/read/27582673/epileptic-encephalopathy-in-infants-and-children
#10
Carl E Stafstrom, Eric M Kossoff
No abstract text is available yet for this article.
July 2016: Epilepsy Currents
https://www.readbyqxmd.com/read/27060389/establishing-an-adult-epilepsy-diet-center-experience-efficacy-and-challenges
#11
Mackenzie C Cervenka, Bobbie J Henry, Elizabeth A Felton, Katlyn Patton, Eric H Kossoff
OBJECTIVES: Over 250 medical centers worldwide offer ketogenic diets to children with epilepsy; however, access to these therapies has been extremely limited for adults until recent years. We examine our 5-year experience creating and implementing a dedicated Adult Epilepsy Diet Center designed to provide adults with epilepsy access to ketogenic diets. MATERIAL AND METHODS: Outpatients seen at the Johns Hopkins Adult Epilepsy Diet Center from August 2010 thru September 2015 age 18years and older were enrolled in a prospective open-label observational study...
May 2016: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/26997037/anticonvulsant-efficacy-in-sturge-weber-syndrome
#12
Emma H Kaplan, Eric H Kossoff, Catherine D Bachur, Milton Gholston, Jihoon Hahn, Matthew Widlus, Anne M Comi
OBJECTIVE: We analyzed individuals with epilepsy due to Sturge-Weber syndrome to determine which anticonvulsants provided optimal seizure control and which resulted in the fewest side effects. METHODS: One-hundred-eight records from a single center were retrospectively analyzed for Sturge-Weber syndrome brain involvement, epilepsy, Sturge-Weber syndrome neuroscores, and currently used anticonvulsants. RESULTS: Of the fourteen anticonvulsants that had been employed, the most often used agents were oxcarbazepine or carbamazepine, and levetiracetam...
May 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/26961268/diet-redux-outcomes-from-reattempting-dietary-therapy-for-epilepsy
#13
Eric H Kossoff, Sarah C Doerrer, Steven P Winesett, Zahava Turner, Bobbie J Henry, Stacey Bessone, Anthony Stanfield, Mackenzie C Cervenka
The outcome for patients attempting dietary therapy for epilepsy a second time is unknown. Twenty-six subjects treated with the ketogenic diet as children who then began either the ketogenic diet or a Modified Atkins Diet (MAD) at least 6 months later were evaluated. The mean age at the first diet trial was 5.6 years and at the second diet trial was 11.5 years. Most restarted dietary therapy because of persistent seizures (65%) or recurrence after seizure freedom (19%). Overall, 77% had a ≥50% seizure reduction with the first diet, and 50% with the second diet, P = ...
July 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/26803281/use-of-dietary-therapies-amongst-patients-with-glut1-deficiency-syndrome
#14
Hannah R Kass, S Parrish Winesett, Stacey K Bessone, Zahava Turner, Eric H Kossoff
PURPOSE: GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. METHODS: A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously, and information analyzed in a database. RESULTS: Surveys were received from 92 families, of which 90 (98%) had been treated with dietary therapies...
February 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/26649186/glycemic-modulation-in-neuro-oncology-experience-and-future-directions-using-a-modified-atkins-diet-for-high-grade-brain-tumors
#15
Roy E Strowd, Mackenzie C Cervenka, Bobbie J Henry, Eric H Kossoff, Adam L Hartman, Jaishri O Blakeley
Dietary glycemic modulation through high-fat, low-carbohydrate diets, which induce a state of systemic ketosis and alter systemic metabolic signaling, have been incorporated into the clinical management of patients with neurological disease for more than a century. Mounting preclinical evidence supports the antitumor, proapoptotic, and antiangiogenic effects of disrupting glycolytic metabolism through dietary intervention. In recent years, interest in incorporating such novel therapeutic strategies in neuro-oncology has increased...
September 2015: Neuro-oncology Practice
https://www.readbyqxmd.com/read/26423430/delivery-of-epilepsy-care-to-adults-with-intellectual-and-developmental-disabilities
#16
REVIEW
Orrin Devinsky, Miya Asato, Peter Camfield, Eric Geller, Andres M Kanner, Seth Keller, Michael Kerr, Eric H Kossoff, Heather Lau, Sanjeev Kothare, Baldev K Singh, Elaine Wirrell
Epilepsy is common in people with intellectual and developmental disabilities (IDD). In adulthood, patients with IDD and epilepsy (IDD-E) have neurologic, psychiatric, medical, and social challenges compounded by fragmented and limited care. With increasing neurologic disability, there is a higher frequency of epilepsy, especially symptomatic generalized and treatment-resistant epilepsies. The causes of IDD-E are increasingly recognized to be genetic based on chromosomal microarray analysis to identify copy number variants, gene panels (epilepsy, autism spectrum disorder, intellectual disability), and whole-exome sequencing...
October 27, 2015: Neurology
https://www.readbyqxmd.com/read/26198753/commentary-breaking-down-the-barriers-to-using-dietary-therapy-for-refractory-epilepsy
#17
COMMENT
Eric H Kossoff
No abstract text is available yet for this article.
September 2015: Epilepsia
https://www.readbyqxmd.com/read/26033161/what-are-the-minimum-requirements-for-ketogenic-diet-services-in-resource-limited-regions-recommendations-from-the-international-league-against-epilepsy-task-force-for-dietary-therapy
#18
Eric H Kossoff, Nabil Al-Macki, Mackenzie C Cervenka, Heung D Kim, Jianxiang Liao, Katherine Megaw, Janak K Nathan, Ximena Raimann, Rocio Rivera, Adelheid Wiemer-Kruel, Emma Williams, Beth A Zupec-Kania
Despite the increasing use of dietary therapies for children and adults with refractory epilepsy, the availability of these treatments in developing countries with limited resources remains suboptimal. One possible contributory factor may be the costs. There is often reported a significant perceived need for a large ketogenic diet team, supplements, laboratory studies, and follow-up visits to provide this treatment. The 2009 Epilepsia Consensus Statement described ideal requirements for a ketogenic diet center, but in some situations this is not feasible...
September 2015: Epilepsia
https://www.readbyqxmd.com/read/25994046/the-ketogenic-diet-in-pharmacoresistant-childhood-epilepsy
#19
REVIEW
Steven Parrish Winesett, Stacey Kordecki Bessone, Eric H W Kossoff
Available pharmacologic treatments for seizures are limited in their efficacy. For a patient with seizures, pharmacologic treatment with available anticonvulsant medications leads to seizure control in <70% of patients. Surgical resection can lead to control in a select subset of patients but still leaves a significant number of patients with uncontrolled seizures. The ketogenic diet and related diets have proven to be useful in pharmacoresistant childhood epilepsy.
June 2015: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/25678869/danger-in-the-pipeline-for-the-ketogenic-diet
#20
Eric Kossoff
No abstract text is available yet for this article.
November 2014: Epilepsy Currents
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