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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
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
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
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
Carl E Stafstrom, Eric M Kossoff
No abstract text is available yet for this article.
July 2016: Epilepsy Currents
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
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
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
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
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
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
Eric H Kossoff
No abstract text is available yet for this article.
September 2015: Epilepsia
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
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
Eric Kossoff
No abstract text is available yet for this article.
November 2014: Epilepsy Currents
Melanie A McNally, Eric H Kossoff
We describe a group of 26 children with no prior history of seizures consistent with benign rolandic epilepsy who had rolandic spikes found coincidentally on EEG. A retrospective chart review as well as phone and email follow-ups with families were completed to assess long-term outcomes. A subset of this group (n=7) with reported comorbid language or learning difficulties was then given an empiric trial of levetiracetam. Seven (27%) children eventually developed seizures, with a median of 14months after the abnormal EEG...
February 2015: Epilepsy & Behavior: E&B
Potjana Jitawatanarat, Tracey L O'Connor, Ellen B Kossoff, Ellis G Levine, Kaweesak Chittawatanarat, Nuttapong Ngamphaiboon
PURPOSE: We evaluated the tolerability and cardiac safety of docetaxel, cyclophosphamide, and trastuzumab (TCyH) for the treatment of early-stage human epidermal growth factor receptor 2 (HER2)-positive breast cancer and compared to the standard trastuzumab-based chemotherapy regimens doxorubicin with cyclophosphamide followed by paclitaxel and trastuzumab (AC-TH) and docetaxel, carboplatin, and trastuzumab (TCaH). METHODS: We retrospectively reviewed early-stage, resectable, HER2-positive breast cancer patients treated with trastuzumab-based chemotherapy at a single comprehensive cancer center between 2004 and 2011...
December 2014: Journal of Breast Cancer
Adrianna Amari, Zahava Turner, James E Rubenstein, Jonathan R Miller, Eric H Kossoff
PURPOSE: Previous research has indicated that children with seizures may prefer high fat foods - a preference compatible with ketogenic and modified Atkins dietary therapies. The purpose of this prospective study was to examine the relationship between fat preference and efficacy of therapeutic diets in treating intractable seizures among a pediatric population. METHODS: Preference for high fat foods was directly assessed in a sample of 30 children prior to commencing either the ketogenic or modified Atkins diet...
February 2015: Seizure: the Journal of the British Epilepsy Association
Mackenzie C Cervenka, Katlyn Patton, Ani Eloyan, Bobbie Henry, Eric H Kossoff
OBJECTIVES: The modified Atkins diet (MAD) is a high fat, low carbohydrate ketogenic diet used to treat intractable seizures in children and adults. The long-term impact on fasting lipid profiles (FLPs) remains unknown. This study was designed to detect significant lipid changes in adults on MAD. METHODS: Patients were observed prospectively. A FLP was obtained in all patients at the first visit then serially. Patients were started on a 20 g per day net carbohydrate limit MAD...
2016: Nutritional Neuroscience
Eric H W Kossoff, W Donald Shields
Individuals with Lennox-Gastaut syndrome (LGS) often do not respond to or become resistant to pharmacologic treatments. Ketogenic diets (KDs) and vagus nerve stimulation (VNS) are nonpharmacologic treatment options for these intractable patients. The classic KD, a high-fat, low-carbohydrate diet with 90% of calories derived from fat, has been used in the treatment of seizures for >90 years. About half of patients with LGS respond to the KD with a >50% reduction in seizures and some patients may achieve a >90% reduction...
September 2014: Epilepsia
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