Podcast appearances and mentions of lennox gastaut

Please visit answersincme.com/JTB860 to participate, download slides and supporting materials, complete the post test, and obtain credit. In this activity, an expert in pediatric epilepsy discusses strategies for optimizing the care of children and adults with Lennox-Gastaut syndrome (LGS). Upon completion of this activity, participants should be better able to: Apply diagnostic criteria to accurately recognize LGS in children and adults; Evaluate the latest pharmacologic treatment strategies available for patients with LGS; and Outline approaches to optimize the long-term care of patients with LGS.

Welcome to the NeurologyLive® Mind Moments® podcast. Tune in to hear leaders in neurology sound off on topics that impact your clinical practice. In this episode, epilepsy expert Depobam Samanta, MD, MS, FAAP, FAES, sat down to discuss a recently published guidance paper from members of the Pediatric Epilepsy Research Consortium that provides recommendations for neuromodulation approaches to treat Lennox-Gastaut syndrome (LGS). Samanta, medical director of the Arkansas Children's Comprehensive Epilepsy Program, gave a clinical overview of the paper, highlighting the various neuromodulatory devices and their use, and the ways to improve treatment selection and personalization. Additionally, he talked about some of the potential complications with these devices as well as initiation and titration strategies for easy initiation. Furthermore, he spoke about ways to take neuromodulation to the next level, giving insight on creative trial designs, overcoming sham-controlled studies, and the necessary research to expand these approaches.  Looking for more epilepsy discussion? Check out the NeurologyLive® Epilepsy clinical focus page. Episode Breakdown: 1:05 – Overview of the published guidance and reasons behind the paper  5:15 – Patient selection for neuromodulation and tailoring treatments by patient and preference 8:25 – Overcoming complications with neuromodulation devices  11:25 – Multidisciplinary team necessary for successful implementation of neuromodulation 13:15 – Neurology News Minute 15:15 – Initiation and titration strategies for vagus nerve stimulation, deep brain stimulation, and resective surgery 18:30 – Next steps in research and expanding neuromodulation for patients with LGS 21:10 – Finding creative trial designs to test neuromodulation devices, combination approaches The stories featured in this week's Neurology News Minute, which will give you quick updates on the following developments in neurology, are further detailed here: FDA Accepts BLA for Subcutaneous Autoinjector Formulation of Lecanemab Cell Therapy Bemdaneprocel Advances to Phases 3 Registrational Trial in Parkinson Disease Real-World Study Highlights Positive Treatment Benefits of Tofersen on ALS Disease Progression, Function Thanks for listening to the NeurologyLive® Mind Moments® podcast. To support the show, be sure to rate, review, and subscribe wherever you listen to podcasts. For more neurology news and expert-driven content, visit neurologylive.com.

Anti-seizure medications are the mainstay of epilepsy treatment, but these medications are not effective for all forms of epilepsy, such as Lennox-Gastaut syndrome or LGS. It's a rare form of epilepsy that has no cure. Dr. Sandi Lam, Division Head, Neurosurgery at Lurie Children's,  is working to help pediatric patients with LGS and their families manage this condition by investigating the outcomes that matter most to patients and their families. In this episode, Dr. Lam talks about this important work and how it may help build a roadmap that will empower the future of research for LGS and other pediatric epilepsies and rare diseases.

This week, Joe is joined by Army Veteran and founder of Konala, Trace Miller. Diagnosed with Lennox-Gastaut epilepsy as a child, doctors predicted Trace wouldn't live past 13. Determined to defy the odds, his mother discovered a life-changing dietary treatment, and Trace has been seizure-free since the age of 7. In 2012, Trace enlisted in the Army and served as a Patriot Missile operator. During a deployment, he developed a passion for learning, business, and entrepreneurship. After separating from the military, Trace and his wife opened a food truck, which eventually grew into a bar and, ultimately, Konala. Driven by a passion for healthy eating, Konala offers nutritious meals with the convenience of fast food. Trace shares his insights with Joe on franchising his restaurant and provides valuable advice to aspiring entrepreneurs Episode Resources:  Konala Franchise   About Our Guest    Trace Miller is the founder of Konala, America's first healthy drive-thru fast food franchise, and a U.S. Army veteran. Despite being diagnosed with Lennox-Gastaut epilepsy and told he would not live beyond 13, Trace defied all odds through a life-changing dietary treatment. His journey through overcoming epilepsy ignited his passion for health and nutrition. Trace's diverse background includes service in the Army, firefighting, welding, and scuba diving instruction. Today, hes revolutionizing fast food with Konala, offering nutrient-rich, fresh ingredients while supporting veterans through franchise opportunities.   About Our Sponsors Navy Federal Credit Union   Buying a home can be overwhelming these days , but from start to finish, Navy Federal Credit Union's new Home Buying Center has everything you need to get to closing with confidence, like:  -Verified Preapproval—A preapproval shows sellers you're a serious buyer, potentially giving you a competitive advantage when making an offer. -RealtyPlus—Connects you with an agent for continued support every step of the way. -Lock and Shop—Lets you lock in your interest rate for up to 60 days while you shop. -No-Refi Rate Drop—Gives you peace of mind to buy now, knowing that if interest rates fall, you could decrease your rate without having to refinance for a small $250 fee. If you're overwhelmed by shopping for a new home, Navy Federal's new Home Buying Center has solutions for every step of the process. Learn more here. At Navy Federal, our members are the mission.      Join the conversation on Facebook! Check out Veteran on the Move on Facebook to connect with our guests and other listeners. A place where you can network with other like-minded veterans who are transitioning to entrepreneurship and get updates on people, programs and resources to help you in YOUR transition to entrepreneurship. Want to be our next guest? Send us an email at interview@veteranonthemove.com.  Did you love this episode? Leave us a 5-star rating and review!  Download Joe Crane's Top 7 Paths to Freedom or get it on your mobile device. Text VETERAN to 38470. Veteran On the Move podcast has published 500 episodes. Our listeners have the opportunity to hear in-depth interviews conducted by host Joe Crane. The podcast features people, programs, and resources to assist veterans in their transition to entrepreneurship.  As a result, Veteran On the Move has over 7,000,000 verified downloads through Stitcher Radio, SoundCloud, iTunes and RSS Feed Syndication making it one of the most popular Military Entrepreneur Shows on the Internet Today. Disclosure: Some of the links above are affiliate links. This means that, at zero cost to you, I will earn an affiliate commission if you purchase via the link provided.

**NOTICIAS ECONÓMICAS Y FINANCIERAS** ☕️ SpaceX logró un hito histórico en su quinto vuelo de prueba al lanzar el gigantesco cohete Starship y recuperar el propulsor de primera etapa Super Heavy usando brazos mecánicos llamados "chopsticks". Este avance acerca a la empresa de Elon Musk a su objetivo de desarrollar cohetes reutilizables que podrían reducir los costos de transporte espacial. Además, fortalece los planes de la NASA para reanudar sus misiones lunares y explorar Marte. Aunque los primeros tres vuelos de prueba terminaron en explosiones, el cuarto en junio de 2024 y el más reciente han sido exitosos, allanando el camino para futuras pruebas de aterrizajes controlados. NASA ya ha seleccionado a Starship para su misión tripulada a la Luna, Artemis 3, programada para septiembre de 2026. Berkshire Hathaway ($BRK.A, $BRK.B), la empresa de Warren Buffett, realizó una compra significativa de acciones de la compañía de radio y radiodifusión SiriusXM ($SIRI) esta semana, adquiriendo un total de $86.73 millones en acciones, lo que elevó su participación total a 192 millones de acciones. La empresa compró 870,000 acciones el miércoles, 1,435,000 acciones el jueves y 1,259,000 acciones el viernes. Berkshire había revelado su participación en $SIRI en noviembre de 2023, luego de que la empresa se convirtiera en pública e independiente tras su transacción con Liberty Media en septiembre. La gigante de software empresarial, ServiceNow ($NOW), anunció una inversión de $1.5 billones en el Reino Unido durante los próximos cinco años, como muestra de confianza hacia el primer ministro Keir Starmer, quien busca atraer inversión extranjera. El dinero se destinará a expandir su negocio en el Reino Unido, incluyendo nuevas oficinas y un crecimiento en la plantilla actual de 1,000 empleados. Además, la inversión se enfocará en la localización de procesamiento de datos para sus modelos de lenguaje grande (LLMs) y la incorporación de GPUs de Nvidia en centros de datos en Londres y Newport. Este esfuerzo busca desarrollar LLMs específicos para clientes y gobiernos del Reino Unido, promoviendo la "soberanía" de la IA en Europa. Lundbeck ($HLBBF) adquirirá Longboard Pharmaceuticals ($LBPH) por un valor de $2.6 billones. Las acciones de $LBPH subieron un 47.81% en el premercado tras el anuncio. Lundbeck lanzará una oferta pública para adquirir todas las acciones en circulación de Longboard. El acuerdo, aprobado por unanimidad por ambas juntas directivas, se cerrará en el cuarto trimestre de este año. A través de esta adquisición, Lundbeck accederá a bexicaserina, un tratamiento en desarrollo para las convulsiones asociadas con epilepsias raras como el síndrome de Dravet y Lennox-Gastaut. La empresa financiará el acuerdo con recursos de caja y su línea de financiamiento bancario, y estima costos de integración de $80 millones que impactarán principalmente en 2024.El ministro de Finanzas de China, Lan Fo'an, ofreció un discurso esperado sobre el plan del país para apoyar la economía, pero no dio detalles sobre cuánto gastará el gobierno para revertir las tendencias deflacionarias. Afirmó que el gobierno tiene una gran capacidad para aumentar el gasto y aliviar las deudas de los gobiernos locales, estabilizar el valor inmobiliario y recapitalizar los principales bancos. Aunque prometió medidas audaces, algunos economistas expresaron decepción por la falta de anuncios sobre el impulso al consumo personal, que consideran clave para el crecimiento a largo plazo. Más detalles se esperan cuando el parlamento chino se reúna este mes. Las acciones que tenemos hoy con predicción bullish en Pre-Market Runners:**

Today we're talking with Army veteran, entrepreneur Trace Miller, about his revolutionary fast food franchise "Konala Protein Bowls and Salads". We'll hear how it combines incredibly healthy food with the traditional drive thru, which is a staple of the American diet … yet slowly poisons us.Trace's inspiration for using whole foods as a cure for disease was not born in the gym. Food was an essential part of how he defeated Lennox-Gastaut epilepsy as a child. His toddler days were filled with seizures, even having to wear a helmet for protection against his bodies violent reaction to a parasite. American doctors offered no hope. Desperate for a cure, his family traveled outside the US for a rare treatment. However it came with a lifelong commitment to a pure and healthy diet. Trace describes the journey through his teenage years, which eventually led him to operating missile systems in the Army. Ironically, his time in the Army would inspire him to pursue personal passions like; scuba diving, stock investing and eventually business ownership.Miller describes how he (and his wife Jammie) initially founded a food truck and bar. But they had even bigger dreams.So building on their life experiences, they designed an alternative to unhealthy fast food franchises …, and Konala Protein Bowls and Salads was born. Hear how soon Konala will be coming to a town near you.Check out the fresh menu and franchise opportunities with Konala Protein Bowls and Salads here:https://konala.com/ To reach CBS Eye on Veterans, Host, Phil Briggsphil@connectingvets.comFollow@philbriggsVet@connectingvetsSee Privacy Policy at https://art19.com/privacy and California Privacy Notice at https://art19.com/privacy#do-not-sell-my-info.

Epilepsy Update https://cmetracker.net/UTHSCSA/Publisher?page=pubOpen#/getCertificate/10097704     Welcome to the latest episode of Pediatrics Now, brought to you by the Department of Pediatrics. This week, we're honored to have Dr. Daniel Lapid, a distinguished new member of the neurology team at the University of Texas Health Science Center.  Dr. Lapid shares how his role as a pediatric neurologist and father has shaped his approach to patient care. In this episode, we dive deep into the advancements in pediatric epilepsy treatment, focusing on Responsive Neurostimulation (RNS) and its recent application in children. Dr. Lapid explains how this innovative device works, the conditions it treats, and its potential benefits for young patients with epilepsy. We also explore the crucial topic of infantile spasms, discussing their symptoms, diagnosis, and the importance of early intervention. Dr. Lapid provides valuable insights on the treatment options available, including the ketogenic diet, and shares a compelling case study to illustrate these points. Finally, we touch on the significance of strong patient-doctor communication and the impact of personal experiences on medical practice. Dr. Lapid's passion for neurology and dedication to improving patient outcomes shine through in this informative and engaging episode. Here's more about Lennox-Gastaut syndrome. Don't miss this enlightening discussion on the latest developments in pediatric epilepsy care.    

Lennox-Gastaut syndrome and Dravet syndrome are two rare, developmental and epileptic encephalopathies. Drug developers have sought to address epilepsies by altering the electrical activity in the brain. Ovid therapeutics, though, has taken a novel approach with its experimental therapy soticlestat by seeking to restore homeostasis to the brain. We spoke to Meg Alexander, chief strategy officer of Ovid, about rare epilepsies, how the company's experimental therapy soticlestat works, and the potential to apply the approach to other CNS conditions. Since recording this episode, there have been new results on soticlestat released. Ovid's partner Takeda this week reported that soticlestat narrowly missed its primary endpoint in its phase 3 Dravet syndrome study while showing clinically meaningful and nominally significant effects in multiple key secondary efficacy endpoints. It also missed its primary endpoint of reduction in major motor drop seizures as compared to placebo in a separate phase 3 study in Lennox-Gastaut syndrome. Takeda said it will be engaging with regulators to determine the best path forward.

In this episode, Ayesha spoke with Jeremy Levin, D. Phil, MB BChir, CEO and Chairman of Ovid Therapeutics Inc., and Meg Alexander, Chief Strategy Officer at Ovid.Ovid Therapeutics is a biopharmaceutical company focused on the development of medicines for epilepsies and seizure-related neurological disorders.Prior to founding Ovid, Dr. Levin was president and CEO of Teva Pharmaceutical Industries and a member of the executive committee at Bristol-Myers Squibb (BMS) Company where he was the architect, lead and implementer of the String of Pearls Strategy, which transformed BMS and facilitated the initiation and massive growth of the immuno-oncology revolution in the biopharmaceutical industry. Dr. Levin also serves on the board and executive committee of the Biotechnology Innovation Organization (BIO) as the immediate past chairman. Dr. Levin was voted as one of the 25 most influential biotechnology leaders by Fierce Biotech, one of the top three biotechnology CEOs by The Healthcare Technology Report and one of the PharmaVoice100 CEOs in 2020 and 2021. He was selected by Endpoints in 2021 as one of the 60 living pioneers of the industry, and has received several awards throughout his career. Dr. Levin has practiced medicine at university hospitals in England, South Africa and Switzerland. Meg Alexander serves as Ovid's chief strategy officer, a role in which she oversees and supports the company's corporate strategy and planning, performance measurement and risk mitigation. She also oversees the company's corporate affairs function with a focus on engaging Ovid's stakeholders across patient and caregiver communities, policymakers, press and investors.Ms. Alexander has worked in the biopharmaceutical industry for two decades and has played a key role in launching more than 25 new medicines for many conditions including rare epilepsies, ALS, HIV, multiple sclerosis, Batten's disease, cardiovascular disease and cancer. She has advised and led campaigns for large healthcare organizations, including Pfizer, Novartis, Amgen, Eli Lilly, Nestle and Coca-Cola among many others. Prior to Ovid, she founded and led the Reputation & Risk Management Group, a consultancy within Syneos Health, one of the world's largest clinical research organizations. Ms. Alexander was named a “Disruptor” and a “Rising Star” in her industry by PM360 and the Healthcare Business Women's Association, respectively.This year, OVID is expecting several CNS data readouts, including one for its lead product candidate soticlestat, which is currently in Phase III trials for the treatment of refractory seizures in Lennox-Gastaut syndrome and Dravet syndrome, a rare epilepsy with high unmet need.Tune in to the episode to learn more about Ovid Therapeutics' developmental pipeline featuring treatments for epilepsies and seizures associated with rare brain conditions.  For more life science and medical device content, visit the Xtalks Vitals homepage. https://xtalks.com/vitals/  Follow Us on Social MediaTwitter: https://twitter.com/Xtalks Instagram: https://www.instagram.com/xtalks/ Facebook: https://www.facebook.com/Xtalks.Webinars/ LinkedIn: https://www.linkedin.com/company/xtalks-webconferences YouTube: https://www.youtube.com/c/XtalksWebinars/featured

Please visit answersincme.com/FPV860 to participate, download slides and supporting materials, complete the post test, and obtain credit. In this activity, an expert in neurology discusses the diagnosis and pharmacological management of Lennox-Gastaut syndrome (LGS), and is joined by a patient advocate to discuss the impact of LGS on patients and their families. Upon completion of this activity, participants should be better able to: Recognize the clinical impact of LGS on the lives of patients with the disease and their families; Outline strategies to accurately diagnose LGS in children and adults; and Describe the pharmacological treatment options available for patients with LGS.

The information contained in this podcast is intended for a European healthcare professional audience for continuing education purposes only. This podcast was supported by Jazz Pharmaceuticals, which  had input into the concept and topic, but not the content of the podcast. The views expressed in this podcast are those of the individual speakers and do not necessarily reflect the views of Jazz Pharmaceuticals. Lennox–Gastaut syndrome (LGS) is a rare, severe, and progressive childhood-onset epilepsy characterised by recurrent seizures. LGS can be challenging to diagnose due to multiple aetiologies, varying clinical presentation at onset, and a lack of specific biological markers. These challenges can be compounded in adulthood due to the evolving presentation of LGS over time, and incomplete medical histories. In this insightful podcast, experts Steve Chung, University of Arizona, Phoenix, USA; and Rhys Thomas, Royal Victoria Infirmary, Newcastle, UK, explore the identification, diagnosis, and management of LGS in both children and adults based on their experiences in real-life clinical settings.

Up to this point, I have resisted doing any episodes around cannabis because 1. I'm a little old-school 2. I probably don't know enough and 3. I want to avoid, in the words of my guest, “being painted with green paint” and tainting the brand.Separating emotions from logic, however, if there are cannabis components that could be effective therapies, it would be foolish to ignore them. There is already one cannabis-derived compound approved by the FDA. Epidiolex is pharmaceutical-grade cannabidiol (CBD) prescribed for the treatment of seizures associated with Lennox-Gastaut syndrome and Dravet syndrome. Pebble Life Science is taking a rigorous approach to investigate whether other compounds derived from hemp might be effective as therapies, either for other neurological disorders or even cancer, initially focused on ovarian.I spoke with Patrick Moran, the founder and CEO of Pebble Life Science about their approach, the challenges they have faced, and the success they have had in forming collaborations with the MD Anderson Cancer Center as well as participating in the NCI Applicant Assistance Program.Everything we discussed is in the pre-clinical phase, preparing for and gearing up toward clinical trials. A key event in this journey was the 2018 farm bill that allows the cultivation of hemp as long as the THC content is below 0.3%. That opened the door to growing and extracting components from hemp, all non-psychotropic, for investigation.Thanks for reading cc: Life Science! Subscribe for free to receive new posts and support my work.Either way, I appreciate you spending time here.One obstacle was bridging the gap between (clinically unproven) claims of cannabis killing cancer and putting in place procedures to rigorously investigate those possibilities. Another challenge, as mentioned above, was breaking through the cloud of suspicion that exists around cannabis. After being invited to apply for a drug development grant by the Cancer Prevention and Research Institute of Texas, one reviewer labeled them as drug dealers.While the research staff took that hard, their legal team suggested taking their data and the positive feedback they had received from the other three CPRIT reviewers and contacting the NCI. That led to an NCI sponsorship for the NIH Applicant Assistant Program which Patrick said was invaluable in helping his executive team prepare to submit an SBIR grant to help fund clinical trials.Based on pre-clinical studies in mice, Pebble is currently focused on the potential of a hemp-derived compound as both an adjuvant therapy and a frontline monotherapy for ovarian cancer. At the end of our interview, I asked Patrick what he had learned that would be helpful to any biotech startup whether or not they are in the cannabis space.Interestingly, I've recently had another conversation with someone I hope will be on this podcast soon. We concluded that science is difficult. People can be more so. It's yet one more challenge to get people to change their thinking or to deal with the obstacles they place in front of you, intentionally or not. I hope this episode help you figure that out on your own journey.There were a few terms I had to look up in our conversation. You may find these useful:RPPA analysisEntourage effectYour deepest insights are your best branding. I'd love to help you share them. Chat with me about custom content for your life science brand. Or visit my website.0 Comments This is a public episode. If you would like to discuss this with other subscribers or get access to bonus episodes, visit cclifescience.substack.com

ONCE UPON A GENE - EPISODE 207 Breaking Barriers in Brain Health with Tracy Dixon-Salazar, PhD For Epilepsy Awareness Month, I am joined by Tracy Dixon-Salazar, a rad scientist-mom and patient advocate. Her adult daughter Savannah was diagnosed with LGS as a child and in this episode, Tracy shares her best advice for advocating for your child and also the wisdom she's learned along the way if you're a parent with a new diagnosis.  EPISODE HIGHLIGHTS Can you share about yourself and your rare disease journey? My daughter Savannah is 30 years old and she started having seizures when she was 2 years old, with no family history, no head injury and no explanation of what caused them. She had seizures everyday, up to hundreds on a bad day. She was diagnosed with epilepsy, which evolved into Lennox-Gastaut syndrome (LGS), a severe form of epilepsy. There wasn't a lot of information available when we received a diagnosis and it was really scary as we learned more about the disease. From the time Savannah was diagnosed at 5 years old to the time she turned 18 years old, she had more than 40,000 seizures and it seemed all I could do was count them. She tried and failed 26 different treatments and nothing worked. I wanted to end her suffering, so I enrolled in college and started taking science classes to better understand the medical papers I was reading. Then after about 12 years, I got my Ph.D. in developmental neuroscience. I went on to do post-op in neurogenetics which is when the genomic revolution happened. My job in the lab was to sequence exomes and my boss suggested that we sequence Savannah. It took a year of analyzing and we found that she has a lot of genetic mutations in her calcium signaling pathway and I knew there was something to it, which led us to a drug that's not used for epilepsy, but gave Savannah a 95% reduction in her seizures within two weeks.  What is the importance of genetic testing for diseases under a larger umbrella disease? It's critical to get genetic and genomic testing. Genetic testing is important especially if you aren't responding to the current treatments. Whole exome testing is becoming more affordable and a great place to start, but if you can get panel testing, do that because even if there's no available treatment, the panel may reveal other data that's helpful and you can help pioneer new treatment options. If you have unexplained rare disease, do not pass go, do not collect $200, go now and push to get genetic testing.  What promising areas of research is the LGS Foundation funding that you believe can change the landscape of treatment? We funded the first mouse model of Lennox-Gastaut syndrome, which replicates the brain waves. There are 7 FDA-approved drugs for LGS, none that target the electroencephalogram (EEG), they all target seizures. We are now targeting focused ultrasound in an animal model that replicates the brain waves of LGS. This is a non-invasive therapy we're using to see if we can prevent the evolution to abnormal brain waves. Additionally, we're funding research to quantify the LGS diagnosis through EEG. We also just launched the Learn form Every Patient Database, a natural history study. LINKS AND RESOURCES MENTIONED ONCE UPON A GENE - EPISODE 172 - From Cancer Biologist to Rare Disease Mom - Digging Into the Data to Better Understand SCN8A with Madeleine Oudin PhD - Professor of Biomedical Engineering at Tufts https://effieparks.com/podcast/episode-172-digging-into-the-data-to-better-understand-scn8a-with-madeleine-oudin-phd LGS Foundation Website https://www.lgsfoundation.org/ LGS Foundation Facebook https://www.facebook.com/lgsfoundation1 LGS Support Groups https://www.lgsfoundation.org/get-connected-and-supported/online-communities/ LGS Learn From Every Patient Database https://www.lgsfoundation.org/lgs-learn-from-every-patient-database/ Dante Labs https://us.dantelabs.com/

CDKL5 deficiency disorder (CDD) is a serious and rare, genetic condition characterized by early onset and difficult to control seizures, as well as severe neuro developmental impairment. Last year, the U.S. Food and Drug Administration approved Marinus Pharmaceuticals Ztalmy to treat seizures associated with CDD, the first FDA approved therapy for the condition. Marinus is seeking to expand the use of Ztalmy in other seizure disorders including tuberous sclerosis complex and Lennox-Gastaut syndrome. We spoke to Alex Aimetti, chief scientific officer of Marinus, about Ztalmy, how it works, and the efforts to expand its use to other seizure disorders.

CME credits: 0.75 Valid until: 30-06-2024 Claim your CME credit at https://reachmd.com/programs/cme/new-and-emerging-therapies-for-complex-epileptic-syndromes/15345/ This Expert Exchange webcast is designed to enhance clinicians' awareness of emerging pharmacotherapies that demonstrate promise in optimizing seizure burden in patients with Lennox-Gastaut and Dravet syndromes.=

CME credits: 0.50 Valid until: 17-04-2024 Claim your CME credit at https://reachmd.com/programs/cme/special-report-management-considerations-and-unmet-needs-in-lgs-and-dravet-syndrome/15112/ Lennox-Gastaut syndrome and Dravet syndrome are 2 rare epileptic disorders with a pediatric onset. They‘re often refractory to therapy and have significant side effects due to polytherapy. Listen in as Drs. Nilika Singhal, Elizabeth Thiele, and M. Scott Perry discuss treatment options and how you can lessen the impact of polypharmacy on your patients.=

First off, apologies from our team for the late posting, I had a sudden medical emergency myself that put me in the hospital.  But... we are back and will be posting a new episode on that incident in the near future!  Thank you for trusting us to return to show production, even if we are 30 hours late!  We are running with back to back reruns based on listeners requests.  For this week's show, let me re-introduce you to Meg Pecora from our first season in July of 2018. At the time Meg was a recent college graduate from Columbia in Chicago. Now she is a recently engaged artist with chronic pain due to Fibromyalgia.  She was approved for the Illinois medical cannabis program and discusses the process of obtaining the authorization and finally "prescription" for medical cannabis as a pain reliever.  ​ Medical marijuana uses the marijuana plant or chemicals in it to treat diseases or conditions. It's basically the same product as recreational marijuana, but it's taken for medical purposes. The marijuana plant contains more than 100 different chemicals called cannabinoids. Each one has a different effect on the body. Delta-9-tetrahydrocannabinol (THC) and cannabidiol (CBD) are the main chemicals used in medicine. THC also produces the "high" people feel when they smoke marijuana or eat foods containing it. ​"The greatest amount of evidence for the therapeutic effects of cannabis relate to its ability to reduce chronic pain, nausea and vomiting due to chemotherapy, and spasticity [tight or stiff muscles] from MS," Bonn-Miller says. ​Medical marijuana received a lot of attention a few years ago when parents said that a special form of the drug helped control seizures in their children. The FDA recently approved Epidiolex, which is made from CBD, as a therapy for people with very severe or hard-to-treat seizures. In studies, some people had a dramatic drop in seizures after taking this drug.  ​The cannabidiol Epidiolex was approved in 2018 for treating seizures associated with two rare and severe forms of epilepsy, Lennox-Gastaut syndrome and Dravet syndrome. In addition, the FDA has approved two man-made cannabinoid medicines -- dronabinol (Marinol, Syndros) and nabilone (Cesamet) -- to treat nausea and vomiting from chemotherapy.  ​ (credits: WEBMD & Marcel Bonn-Miller Ph.D.)

MedLink Neurology Podcast is delighted to feature selected episodes from BrainWaves, courtesy of James E Siegler MD, its originator and host. BrainWaves is an academic audio podcast whose mission is to educate medical providers through clinical cases and topical reviews in neurology, medicine, and the humanities, and episodes originally aired from 2016 to 2021. Originally released: February 6, 2020 The sole FDA-approved indication for clobazam is as a therapeutic adjunct in Lennox-Gastaut syndrome. But as more and more clinicians publish their experience with the off-label success of certain therapies, we may see new indications for this and other pharmacologic agents in the treatment of neurologic disease. Produced by James E Siegler. Unless otherwise mentioned in the podcast, no competing financial interests exist in the content of this episode. Music courtesy of Chris Zabriskie, Milton Arias, and John Bartmann. Sound effects by Mike Koenig and Daniel Simion. BrainWaves' podcasts and online content are intended for medical education only and should not be used for clinical decision-making. Be sure to follow us on Twitter @brainwavesaudio for the latest updates to the podcast. REFERENCES Bresnahan R, Martin-McGill KJ, Williamson J, Michael BD, Marson AG. Clobazam add-on therapy for drug-resistant epilepsy. Cochrane Database Syst Rev 2019;10(10):CD004154. PMID 31638272 Keene DL, Whiting S, Humphreys P. Clobazam as an add-on drug in the treatment of refractory epilepsy of childhood. Can J Neurol Sci 1990;17(3):317-9. PMID 2207888 Mahmoud SH, Rans C. Systematic review of clobazam use in patients with status epilepticus. Epilepsia Open 2018;3(3):323-330. PMID 30187002 Montenegro MA, Arif H, Nahm EA, Resor SR Jr, Hirsch LJ. Efficacy of clobazam as add-on therapy for refractory epilepsy: experience at a US epilepsy center. Clin Neuropharmacol 2008;31(6):333-8. PMID 19050410 Montenegro MA, Cendes F, Noronha AL, et al. Efficacy of clobazam as add-on therapy in patients with refractory partial epilepsy. Epilepsia 2001;42(4):539-42. PMID 11440350 Ng YT, Collins SD. Clobazam. Neurotherapeutics 2007;4(1):138-44. PMID 17199029 Remy C. Clobazam in the treatment of epilepsy: a review of the literature. Epilepsia 1994;35 Suppl 5:S88-91. PMID 8039479 Schmidt D, Rohde M, Wolf P, Roeder-Wanner U. Clobazam for refractory focal epilepsy. A controlled trial. Arch Neurol 1986;43(8):824-6. PMID: 3089202. PMID 3089202 YOUTUBE EXCERPTS (shared via fair use)  Kathryn Davis MD (Hospital of the University of Pennsylvania): https://www.youtube.com/watch?v=XInZ7...  Patty McGoldrick NP MPA (Beth Israel Deaconness Medical Center/Roosevelt Hospital): https://www.youtube.com/watch?v=sE2Bk...  We believe that the principles expressed or implied in the podcast remain valid, but certain details may be superseded by evolving knowledge since the episode's original release date.

Outrora defensor da autonomia do médico, agora o Conselho Federal de Medicina (CFM) perde força ao tentar restringir o uso do canabidiol para poucos contextos clínicos? Quem deve tratar deste tema, o CFM, a Agência Nacional de Vigilância Sanitária (Anvisa), ou ambos? No primeiro episódio da sétima temporada do podcast Conversa de Médico, o advisor de psiquiatria do Medscape em português Dr. Sivan Mauer recebe seu colega Dr. Marco Bessa, especialista em psiquiatria da infância e adolescência, e em dependência química, para falar sobre a Resolução CFM Nº 2324 de 11/10/2022, publicada às vésperas das eleições presidenciais de 2022, que “Aprova o uso do canabidiol para o tratamento de epilepsias da criança e do adolescente refratárias às terapias convencionais na Síndrome de Dravet e Lennox-Gastaut e no Complexo de Esclerose Tuberosa”. Aperte o play e ouça o interessante debate sobre essa medida que gerou uma intensa resposta da sociedade civil ― sobretudo na internet.

Raúl Elizalde Garza es abogado y empresario mexicano. Actualmente es CEO de HempMeds, compañía pionera en la comercialización de productos de CBD y otros cannabinoides extraídos del hemp en Estados Unidos y Latinoamérica. Inició como activista a favor del uso del cannabis en 2013, debido al padecimiento de su pequeña hija Graciela Elizalde, quien padece del síndrome Lennox Gastaut y tenía hasta 400 convulsiones diarias que no pudieron ser tratadas con medicamentos convencionales en 2013.El día de hoy hablamos sobre su historia, de cómo fue que logró legalizar la marihuana medicinal en México y cómo terminó siendo CEO de HempMeds, empresa filial de la compañía estadounidense Medical Marijuana Inc, para México y posteriormente para América Latina. Para conocer más sobre HempMeds te compartimos su página oficial:Página Oficial: HempMeds México Conducido por Moishe Sandler, sígueme en:Página web: Health Coach SandlerFacebook: Moishe SandlerInstagram: @hcsandlerProducido por: @genuinamedia  Distribuido por Genuina Media

Mayela Benavides y Raúl Elizalde son los padres de Grace Elizalde Benavides, quien a temprana edad fue diagnosticada con el Síndrome de Lennox-Gastaut, una variante de epilepsia muy difícil de tratar. Por años, Grace probó una infinidad de tratamientos. Desde anticonvulsivos de todo tipo hasta una operación en el cerebro llamada callosotomía. Después de intentarlo todo y no tener resultados, Maye y Raúl iniciaron su labor por la legalización de la cannabis medicinal para mejorar la calidad de vida de su hija. El día de hoy, nos comparten su historia, el proceso que vivieron como padres ante una enfermedad tan difícil como la de Grace. Hablamos de la negación, el duelo y la aceptación. Platicamos también sobre las fortalezas que tuvieron como pareja, para poder sobrepasar la situación y no solo ayudar a su hija, sino a miles de familias al reducir la brecha de acceso al cannabis medicinal para familias de escasos recursos.Para conocer más sobre el caso te compartimos la página web de su fundación:Página web: Pro GraceConducido por Moishe Sandler, sígueme en:Página web: Health Coach SandlerFacebook: Moishe SandlerInstagram: @hcsandlerProducido por: @genuinamedia Distribuido por Genuina Media

Esse é o Maconhômetro Debate, um projeto do Cannabis Monitor com a proposta de contextualizar e aprofundar temas relevantes envolvendo a maconha no Brasil e mundo. Nesse episódio, Monique Prado recebe o advogado e pesquisador Emilio Figueiredo, a educadora canábica e antropóloga Luna Vargas e médico de família e também pesquisador Rodrigo Pastor para repercutir a famigerada resolução nº 2324/2022 do Conselho Federal de Medicina (CFM), publicada em 14 de outubro no Diário Oficial da União, que restringiu a prescrição do canabidiol por médicos, permitindo apenas para epilepsias resistentes ao tratamento na infância e adolescência relacionadas às síndromes de Dravet e Lennox-Gastaut e ao complexo de esclerose tuberosa. A nova resolução do CFM também foi acrescida de uma proibição aos médicos de ministrar palestras e cursos sobre uso do CBD ou produtos derivados de cannabis “fora do ambiente científico” e fazer qualquer divulgação publicitária. Ou seja, a entidade coibiu os médicos de divulgarem os benefícios medicinais da maconha aos seus pacientes e também os produtos à base de cannabis disponíveis legalmente hoje no mercado. A medida evidenciou ainda mais o grau de politização ideológica conservadora e negacionista presente e dominante na sua diretoria, que meses atrás chancelava medicamentos comprovadamente ineficazes contra a COVID-19, como a famigerada Cloroquina. A pressão social de pacientes, ativistas, médicos, entidades, associações, jornalistas e advogados foi grande, inclusive com manifestações realizadas em frente às sedes dos conselhos estaduais do RJ, SP e BA, e também em Brasília, na sede do CFM, onde 3 ativistas foram recebidos pela diretoria para apresentarem as suas demandas. Após tanta repercussão negativa, o CFM suspendeu a resolução e abriu uma consulta pública sobre cannabis medicinal aberta à toda a sociedade para discutir a liberação ou veto a respeito do uso de medicamentos formulados com derivados da planta. Confira o Debate e fique por dentro das impressões de quem acompanhou tudo de muito perto! Apoie o Cannabis Monitor: http://apoia.se/cannabismonitor

Mayela asegura que Grace es su gran maestra de vida. Después de que nació, ella sabía que algo no estaba bien con su hija. A pesar de que su pediatra le decía que no se preocupara, su instinto materno le decía otra cosa. Mayela no descansó hasta dar con el diagnóstico adecuado: Síndrome de Lennox-Gastaut. Fueron muchos los medicamentos que Grace tomó sin que hubiera mejoría, incluso una operación que estaba en etapa de prueba la cual tampoco funcionó. Sus 400 convulsiones diarias eran ya insostenibles. Un día Mayela y su esposo se enteraron del caso de una niña de Estados Unidos que tomaba Cannabis Medicinal y había mejorado mucho, por lo que se dieron a la tarea de investigar más al respecto hasta lograr que Grace se convirtiera en la primera paciente en México en obtener acceso legal a Cannabis Medicinal, mejorando drásticamente su calidad de vida. Su caso y su lucha lograron modificar la Ley General de Salud y el Código Penal Federal para que más pacientes pudieran tener acceso. Posterior a esto, Mayela funda y preside la Asociación Civil “Por Grace”, primera fundación de Cannabis Medicinal en México. Hoy ella agradece enormemente todo lo que Grace ha traído a su vida y está convencida de que el dolor se convierte en amor. Una historia de entrega y gratitud. Facebook: Efecto Inspiración Instagram: @efectoinspiracion

In this week's podcast, Neurology Today's editor-in-chief discusses the approval of fenfluramine for Lennox-Gastaut syndrome, how social isolation affects dementia risk, and the competitiveness of neurology in this year's residency match.

Welcome to the NeurologyLive® Mind Moments™ podcast. Tune in to hear leaders in neurology sound off on topics that impact your clinical practice. In this episode, we spoke with a number of individuals who presented data and spoke at the American Academy of Neurology Annual Meeting, on topics ranging from COVID-19's clinical presentation, overall trends in the care of patients with multiple sclerosis, the potential of regenerative medicine in traumatic brain injury, the real-world and long-term use of new therapy for Lennox-Gastaut syndrome, the optimal timing for the newest therapy in neuromyelitis optica spectrum disorder, the Axon Registry and incorporating EHR information into its data collection, and the impact of the pandemic on clinician burnout and mental health. Note: As part of AAN policy for the Annual Meeting, all individuals were asked to remain masked throughout the week, so there may be some moments of muffled noises as participants adjust or shift their masks. Those featured include (in order of appearance):  Jennifer Frontera, MD Stephen Krieger, MD Peter McAllister, MD Kelly Knupp, MD, MSCS Bruce Cree, MD, PhD, MAS Shrujal Baxi, MD, MPH Indu Subramanian, MD. Episode Breakdown: 1:50 – Jennifer Frontera, MD, on the long-term data collected on COVID-19 post-infection neurological sequelae. 9:55 – Stephen Krieger, MD, on the trends in thinking about multiple sclerosis pathogenesis and quality of life. 14:05 – Peter McAllister, MD, on the phase 2 STEMTRA trial of stem cell therapy in patients with traumatic brain injury. 16:45 – Kelly Knupp, MD, MSCS, on the long-term use of fenfluramine in Lennox-Gastaut syndrome and clinical pearls on titration. 19:00 – Bruce Cree, MD, PhD, MAS, on the effect of inebilizumab in patients with neuromyelitis optica spectrum disorder regardless of disease duration. 21:05 – Shrujal Baxi, MD, MPH, on the AAN's Axon Registry and how Verana Health is playing a role in data collection. 23:55 – Indu Subramanian, MD, on the effects of COVID-19 on physicians and clinicians and the difficulties that have challenged the field. Click here for more coverage of AAN 2022. Thanks for listening to the NeurologyLive® Mind Moments™ podcast. To support the show, be sure to rate, review, and subscribe wherever you listen to podcasts. For more neurology news and expert-driven content, visit neurologylive.com.

Welcome to this special episode of the NeurologyLive® Mind Moments™ podcast. Tune in to hear leaders in neurology sound off on topics that impact your clinical practice. In 2022 and onward, whenever the FDA makes a major decision for the field of neurology, we'll be releasing short special episodes to offer a snapshot of the news, including the main takeaways for the clinical community, as well as highlights of the efficacy and safety profile of the agent in question. In this episode, we're covering the recent approvals of ganaxolone (Ztalmy; Marinus Pharmaceuticals) in cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder, also known as CDD, as well as fenfluramine (Fintepla; Zogenix/UCB) in Lennox-Gastaut syndrome (LGS). Offering commentary is a pair of experts. The first is Scott Demarest, MD, MSCS, neurologist and clinical director of precision medicine, Children's Hospital Colorado, and assistant professor of pediatrics-neurology, University of Colorado, and an investigator of the Marigold trial (NCT03572933). The second is Kelly G. Knupp, MD, MSCS, FAES, pediatric neurologist, Children's Hospital Colorado, and associate professor of pediatrics-neurology, University of Colorado, and an investigator of Study 1601 (NCT03355209). For more of NeurologyLive®'s coverage of ganaxolone's approval, head here: Ganaxolone Wins Landmark Approval for CDKL5 Deficiency Disorder. For more of NeurologyLive®'s coverage of fenfluramine's approval, head here: Fenfluramine FDA-Approved for Treatment of Lennox-Gastaut Syndrome. For more coverage of epilepsy and seizure disorders, head to NeurologyLive®'s Epilepsy clinical focus page. Episode Breakdown: 0:30 – Ganaxolone (Ztalmy; Marinus) approved for CDD 2:30 – Scott Demarest, MD, with his reaction to the approval 3:00 – Data from the Marigold study of ganaxolone 4:45 – Safety profile of ganaxolone in development 5:10 – Demarest on the landscape of care for CDD 6:20 – Clinical takeaways ahead of DEA scheduling for ganaxolone 8:40 – Fenfluramine (Fintepla; Zogenix/UCB) approved for LGS 10:55 – Kelly Knupp, MD, with her reaction to the new indication 11:30 – Efficacy data on fenfluramine in LGS 13:50 – Safety profile in LGS and Dravet syndrome 16:05 – Knupp on the potential to improve quality of life and optimal use Thanks for listening to the NeurologyLive® Mind Moments™ podcast. To support the show, be sure to rate, review, and subscribe wherever you listen to podcasts. For more neurology news and expert-driven content, visit neurologylive.com. REFERENCES 1. Marinus Pharmaceuticals Announces FDA Approval of ZTALMY® (ganaxolone) for CDKL5 Deficiency Disorder. News release. March 18, 2022. Accessed March 21, 2022. https://www.businesswire.com/news/home/20220318005282/en/Marinus-Pharmaceuticals-Announces-FDA-Approval-of-ZTALMY%C2%AE-ganaxolone-for-CDKL5-Deficiency-Disorder 2. FINTEPLA® (fenfluramine) Oral Solution Now FDA Approved for Treatment of Seizures Associated with Lennox-Gastaut Syndrome (LGS). News release. UCB. March 28, 2022. Accessed March 28, 2022. https://finance.yahoo.com/news/fintepla-fenfluramine-oral-solution-now-050000058.html

Para tratar pacientes con epilepsia, el CBD interactúa principalmente con los receptores cannabinoides CB1 en el cerebro. Esta interacción puede mejorar el funcionamiento general del sistema endocannabinoide de la persona, causando una reducción de las convulsiones. Se prescribe para tratar formas graves de epilepsia, como el síndrome de Dravet y el síndrome de Lennox-Gastaut. Ambos durante la infancia. - Siguenos en: https://www.facebook.com/NeurologiaHIM/ Instagram: @neuropediahim www.cerebrosendesarrollo.com https://www.youtube.com/channel/UCHhTfioddb3AxttXlXh4WRw Para dudas y comentarios escríbenos a: cerebrosendesarrollo@gmail.com --- Send in a voice message: https://anchor.fm/cerebros-en-desarrollo/message

J and D talk to Codi Peterson, owner of PharmD Approved, about the Endocannabinoid System (ECS) -- the physiological system in our bodies that responds to cannabis.  He shares what products are available to pediatric patients suffering from seizures and cancer. Codi also gets D to make a big confession and crushes the myth about cannabis and our memory. Our guest reviewer, G, tells listeners about the surprising efficacy of the CBD product he consumes in order to alleviate the pain of Crohn's disease. Vocabudulary lesson: endocannabinoid system, aka ECS (n.): the complex cell-signaling system identified in the early 1990s by researchers exploring THC, a well-known cannabinoid.Epidiolex (n.): the first FDA‑approved prescription cannabidiol used to treat seizures associated with Lennox-Gastaut syndrome, Dravet syndrome, or tuberous sclerosis complex in patients 1 year of age and older.Marinol (n.): a man-made form of cannabis used to treat patients suffering from loss of appetite, nausea and vomiting due to cancer and chemotherapy.transmucosal (adj.): supplying a medication through or across a mucous membrane (as of the mouth).

El sindrome de West o de síndrome de espasmos infantiles es una encefalopatía epiléptica de la infancia , una enfermedad que es grave pero poco frecuente. Se caracteriza por tres elementos: espasmos epilépticos, retardo del desarrollo psicomotor e hipsarritmia en el electroencefalograma, aunque uno de estos puede estar ausente. Es ante todo una epilepsia del niño: la mayoría de los casos comienzan entre los 2 y los 6 años. Es posible un inicio más precoz, sobre todo en las formas secundarias, siendo entonces imposible precisar si el síndrome de Lennox-Gastaut es la continuación sin transición de un Síndrome de West. Sin embargo, el síndrome puede aparecer en la segunda mitad de la infancia, en la adolescencia e incluso en el adulto. Recuerda que cuidar de un hijo con cualquier síndrome puede ser todo un reto. Trabaje codo a codo con el equipo médico que atiende a su hijo para programar y asistir a todas sus visitas y tratamientos médicos. - Siguenos en: FB: https://www.facebook.com/AMHIMCDMX Instagram: amhimcdmx Web: www.amhim.com.mx/ Youtube: https://www.youtube.com/channel/UCQpZKT2QYr5R_-oSL68Vfng Para dudas y comentarios escríbenos a: pediatrasmexicopodcast@gmail.com --- Send in a voice message: https://anchor.fm/pediatria-mexico/message

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

Go online to PeerView.com/RGT860 to view the activity, download slides and practice aids, and complete the post-test to earn credit. Seizure management in patients with Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS), or tuberous sclerosis complex (TSC) can be extremely challenging, since current antiepileptic drugs, even in combination, are usually insufficient for preventing recurrent severe seizures associated with these treatment-resistant epilepsies. A prescription pharmaceutical formulation of highly purified cannabidiol (CBD) is approved for the treatment of seizures in patients 1 year of age or older with DS, LGS, and TSC, making it the first plant-derived cannabis-based medicine approved by the FDA. In this activity, based on a recent live webcast, neurology and pharmacy expert panelists will interpret the latest clinical evidence shaping optimal use of FDA-approved CBD for the management of severe treatment-resistant epilepsies and will provide their perspectives on recent regulatory decisions that impact patient and provider access to this therapy. Upon completion of this activity, participants will be able to: Describe the rationale for and potential benefits of cannabidiol (CBD) for patients with seizure disorders and other medical conditions, Summarize the characteristics, efficacy/safety profiles, and current indications of oral CBD in treatment-resistant epilepsies, Review the impact of recent legal and regulatory decisions on the availability, distribution, and payer coverage of an FDA-approved oral CBD formulation, Integrate cannabidiol into treatment plans for appropriate patients with treatment-resistant epilepsies in managed care settings, Educate patients, caregivers, and other members of the managed care team about the distinction between pharmaceutical-quality and dispensary-derived cannabinoid products.

A slightly different podcast episode today to give an authentic human experience of what effect a ketogenic diet could have in certain circumstances - we’re talking with Alex Ash, a colleague of one of my best friends in America, who reached out to me a while ago wanting to share his personal experience with the use of a Ketogenic diet for his son’s medical condition.Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy with multiple types of seizures and as you’ll hear, Alex and his partners experience has not been a straight forward one in the slightest.For any of you listening with children, with or without a chronic condition, I just want to say from the outset that this episode could be triggering so I would exercise some caution if you do not want to listen. Alex’s story has a positive ending but I understand this is not the case for a lot of people struggling with epilepsy and many other conditions. As a medical doctor I witness the daily battle many parents and carers have to endure for the sake of their loved ones.As always, none of the information spoken about on today’s podcast is taken to be medical advice, always speak with your practitioner about changes to medication, dietary or supplement regimen. And there are a few explanations for how the keto diet may yield an effect offered by Alex that are not technically accurate, but for the purposes of this special podcast I’ve chosen to completely unedit our conversation. If you’re interested in the more nuanced opinion of a practitioner I would listen to the previous podcast with Sue Wood that I've linked here for you.I hope you enjoy listening to Alex tell Jachin’s story, it’s certainly hit home a lot of truths to me.Remember to check out The Doctor's Kitchen website where you can find full show notes and social media links for this episode See acast.com/privacy for privacy and opt-out information.

This episode has something for everyone: -People who have contracted a mild strain of COVID -People who have cussed out a mental disorder -All other people See? Literally EVERYONE. In honor of Rich's 30th birthday, Hilary interviews him about the ups & downs of being the dad to a little girl with epilepsy. They talk about the struggles of parenting a non-verbal child, the painful reality of seizures, and the little unexpected moments of joy and beauty found in all of it. IG: @hilaryharris_

We pre-recorded today's conversation via Zoom with neurologist and epileptologist, Dr. Paul Lyons. Dr. Lyons is the medical director of the Virginia Comprehensive Epilepsy Program at Winchester Medical Center, an NAEC Level 4 epilepsy center. [The National Association of Epilepsy Centers (NAEC) accredits epilepsy centers and recognizes four levels of care. Level 4 centers are the highest level centers, offering the most “complex forms of intensive neurodiagnostic monitoring, as well as more extensive medical, neuropsychological, and psychosocial treatment. Level 4 centers also offer a complete evaluation for epilepsy surgery, including intracranial electrodes and a broad range of surgical procedures for epilepsy.”] The Virginia Comprehensive Epilepsy Program at Winchester Medical Center is an NAEC Level 4 adult and pediatric epilepsy center. It is one of only three centers in Virginia to offer level 4 epilepsy care for both children and adults. The program is a collaboration between Winchester Medical Center and two Winchester-based physician practices, Winchester Neurological Consultants (neurology practice) and Virginia Brain and Spine Center | Valley Health (neurosurgery practice). The founders of the program, epileptologist Paul Lyons, MD (Winchester Neurological Consultants) and neurosurgeon Lee Selznick, MD (Virginia Brain and Spine Center | Valley Health) envisioned integrated services for the treatment of epilepsy and pursued a cohesive, comprehensive program for our region. Dr. Lyons defined Epilepsy, explained the role seizures play in diagnosis and talked about how an FDA approved pharmaceutical grade cannabinoid extract called Epidiolex is changing how several severe seizure disorders are being treated, and the dramatic impact it is having on patients' lives locally. Beginning in 2015, Dr. Lyons, The Virginia Comprehensive Epilepsy Program at Winchester Medical Center and the Valley Health Clinical Research department were involved in a series of clinical research trials studying the efficacy of Epidiolex in the treatment of two severe forms of epilepsy; Lennox-Gastaut syndrome (LGS), and Dravet Syndrome. Children and adults with each type of epilepsy were recruited for participation in the controlled trials. For one of the studies, the Virginia Comprehensive Epilepsy Program at WMC was the highest enrolling site in the country. Most recently, in August of 2020, the U.S. Food and Drug Administration (FDA) approved EPIDIOLEX® (cannabidiol) oral solution to treat seizures associated with tuberous sclerosis complex (TSC) in patients one year of age and older. TSC is a rare disease that causes benign tumors to grow in vital organs of the body and is a leading cause of genetic epilepsy. Although tuberous sclerosis complex is a rare condition, Winchester Neurological Consultants currently sees a number of adult and pediatric patients with TSC. Along with this new indication, the age range has been expanded to include patients one year of age and older who experience seizures associated with Lennox-Gastaut syndrome (LGS) or Dravet syndrome. EPIDIOLEX, the first plant-derived cannabinoid prescription medicine and the only FDA-approved form of cannabidiol (CBD), was initially approved by the FDA in June 2018 for the treatment of seizures associated with LGS or Dravet syndrome in patients two years of age and older.

Lennox-Gastaut syndrome (LGS) is an epilepsy syndrome that can develop over time from childhood seizures that remain uncontrolled by treatments. It's common for children with genetic epilepsy to develop LGS which can then further exacerbate their symptoms. To better understand LGS, we speak to Dr Tracy Dixon-Salazar, Director of Research and Strategy for the Lennox-Gastaut Foundation.  You can listen to and subscribe to the SCN2A Insights podcast via Apple Podcasts,  Spotify, and Google Podcasts or in your podcast app. Links:  Lennox-Gastaut Foundation

What are terpenes and how important are they to the cannabis plant? This week we're joined by Dr. Ethan Russo, physician, researcher, and leading expert in the field of Medicinal Cannabis. In this episode, we discuss all things terpenoid, including how they are produced, varieties commonly found in cannabis, and their therapeutic benefits. Episode SummaryDr. Russo was classically trained as a neurologist, but turned back to a teenage interest in medicinal plants when he realised he was prescribing increasingly toxic drugs to his patients. In the early 90's he became interested in treating headaches and migraines, and took research trips to the Amazon rainforest in Peru to work with native tribes who treated the condition with botanicals.When he returned to the USA he knew that he wanted to change his career path, and became interested in the medicinal properties of essential oils, terpenes and terpenoids.Dr. Russo became interested in using cannabis medicinally and started the Journal of Cannabis Therapeutics. He quickly became an expert in the field, and worked as a consultant for GW Pharmaceuticals who had obtained a permit to grow cannabis for medicinal purposes in 1998.Later on he became a Senior Medical Advisor and was heavily involved with the development of Sativex and Epidiolex.The first cannabis derived pharmaceutical approved by the FDA was Epidiolex, which contains a 98% pure cannabidiol compound and is used to treat Lennox-Gastaut syndrome and Dravet syndrome.To get a drug approved by the FDA often requires between 10 and 12 years of research and between $700m and $1.2bn of expenditure.Dr. Russo has recently founded CReDO Science, who create innovative products and services related to cannabis and the endocannabinoid system, outside the realm of THC. Projects include over the counter treatments, novel extraction techniques, nutritional cannabis products, cannabis based disinfectant, and diagnostic tests. Terpenes are the aromatic components of plants which can be found in the leaves, bark, flowers and sometimes roots. They are used in aromatherapy which is the science of essential oils. An example of this is lemon oil, which denotes cleanliness and is a cleaning agent. The terpene responsible for this aroma is Limonene, which can be found in citrus fruits.Selective breeding for high THC strains means that many of the new strains have a dominant terpene of Myrcene, which interacts with THC to produce sedative effects.Dr. Russo is currently conducting a piece of research which looks into the effects of using THC with differing terpenoids, using memory tests as a measure.There are over 20,000 identified terpenoids, none of which (so far) are unique to cannabis. Alpha Pinene and Limonene are the most abundant in nature but are not found in cannabis very often. In cannabis, Myrcene and Caryophyllene are often found, which interact with CB2 receptors. Caryophyllene is both a terpene and a cannabinoid.In any cannabis sample, there are on average 3-5 prominent terpenoids along with trace amounts of many more.Terpenes act as a defence mechanism for plants, their strong smells either promote pollination, deter predation by insects or to prevent grazing by mammals. Cannabinoids and Terpenoids are both produced in Capitate Glandular Trichomes which are most abundant in unfertilised female plants. Many terpenes have pain reducing effects which work through distinct mechanisms. Quotables‘Terpenes are critical components to the medicinal effects of cannabis' 04:20‘Essential oils are extremely potent, so the amounts involved are tiny' 22:25

In this episode, Jonathan & Kristy McKinney talk about raising a child with Lennox-Gastaut Syndrome and how it has challenged and shaped their entire family.   Jonathan & Kristy Mckinney Share Their Story Jonathan & Kristy McKinney were young marrieds at 24 when they became pregnant with their first child. They share the journey of infantile seizures which led to Lennox-Gastaut. When a doctor tells you that your child has a "catastrophic" condition and that it will be lifelong and incurable, what do you do? This is a snippet of their story. What is Lennox-Gastaut Syndrome Lennox-Gastaut Syndrome is a severe condition marked by recurrent epileptic seizures that begin early in life. Most children with Lennox-Gastaut Syndrome have severe intellectual disability or learning problems which often worsen over time.  Most people with this condition require help with the usual activities of daily living. Raising a Child With Lennox-Gastaut Syndrome is Challenging Parents with a disabled child face definite challenges. Jonathan and Kristy share a few that they have faced in raising their son with Lennox-Gastaut Syndrome. Multiple hospital visits. Extended stays in hospital. Multiple brain procedures. Multiple prescription drugs with high cost. A need for medical equipment not covered by insurance. Support From Others Is Life Changing Jonathan and Kristy share a few of the many ways they and their family have been blessed by the help of others in their family, church, and community. They share how even at their lowest points, help came in some form~whether through family, church members, or a well-equipped school. Further Information Related to this Podcast Suffer Strong: How to Survive Anything By Redefining Everything by Katherine Wolf is a book Kristy recommends to those facing hardships. Kristy may be reached by email at mckinneykristy@gmail.com Jonathan may be reached by email at jonathancmckinney@gmail.com

Friend of the podcast, Hilary Harris, is back sharing with us her family’s journey as special needs parents. Hilary & Rich are the parents of three beautiful girls, Millie (1), Haven, (3) and Jane (6) who lives with Lennox-Gastaut syndrome, a complex, rare, and severe childhood-onset epilepsy. Hilary graciously invites us into her world and offers sound, practical, and needed advice on how we can interact, love, and approach those with special needs. Click here for a great list of tips from Hilary!  Connect with Hilary on Instagram here. Be sure to follow her blog here as well: www.hilaryharris.org Connect with Rachel: hello@honestmomspodcast.com Subscribe to Honest Moms email list to get the latest info from us! (scroll down to where the page says Let’s Connect) Follow Rachel on Instagram  Music from this episode provided by Joseph McDade.

Dr. Jerzy Szaflarski talks about the FDA's approval of the first cannabis-derived pharmaceutical for the treatment of epilepsy and Lennox-Gastaut syndrome in particular. For more, read the full article, "The US Food and Drug Administration's Authorization of the First Cannabis-Derived Pharmaceutical: Are We Out of the Haze?" by Dr. Szaflarski in JAMA Neurology: https://jamanetwork.com/journals/jamaneurology/article-abstract/2714720. 

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

There is an increased interest in cannabis-derived products for the treatment of human illness. Most recently, randomized and double blinded clinical trials have shown efficacy of plant-derived extract for the treatment of seizures associated with Lennox-Gastaut and Dravet Syndromes. Jerzy Szaflarski, MD, PhD discusses how concurrent open label studies showed potential efficacy of cannabidiol for the treatment of other seizure types and the utility of cannabis-derived products (FDA approved and artisanal) for the treatment of epilepsy.

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

Go online to PeerView.com/XYB860 to view the entire program with slides. In this activity, an expert in the management of Lennox-Gastaut syndrome discusses the challenges of identifying, diagnosing, and treating patients with this condition. Upon completion of this activity, participants should be better able to: Identify patients with Lennox-Gastaut syndrome based on the presence of cardinal symptoms and hallmark features, Cite current data on approved pharmacologic therapies for the treatment of seizures associated with Lennox-Gastaut syndrome, Develop personalized seizure management regimens for patients with Lennox-Gastaut syndrome.

Did you know that 1/3 of all kids with autism develop seizures?I've got a lot of mom guilt and "shoulda, coulda, woulda" surrounding my son's seizure condition. Here's what to look for and where to get more information.What you need to know if your child is at risk for developing seizures.Read the blog post: 6 Things I wish I'd been told about my son's seizure risk.Support the show (https://paypal.me/ADayInOurShoes)

Daniel Barber and Ken Marshall talk about the recent FDA approval of Sympazan™ for Lennox-Gastaut syndrome (LGS) and Aquestive Therapeutics’s new focus on delivering innovative drugs over “Listerine-like” dissolvable films named PharmFilm® to patients with diseases of the nervous system, such as epilepsy. Listen to more health-related stories and research updates at www.hpr.fm

Brittany’s son Lane was a healthy, developmentally on target 18 month old when he started having seizures and was diagnosed with Lennox-Gastaut Syndrome.  Despite trying multiple pharmaceutical drugs Lane's seizures continued and even seemed to worsen with every medication.  After months of frustration and watching her son regress into an almost vegetative state and failing to qualify for a CBD study at Texas Children's Hospital, Brittany packed up her family and moved to Colorado so she could treat Lane's condition with medical cannabis.  Lane has been seizure free for 18 months and is flourishing.   Relevant Links: https://rarediseases.org/rare-diseases/lennox-gastaut-syndrome/ https://www.leafly.com/news/cannabis-101/what-is-rick-simpson-oil Check out today's Sponsor The Grow CFO www.thegrowcfo.com   --- This episode is sponsored by · Anchor: The easiest way to make a podcast. https://anchor.fm/app

This approval is the culmination of GW’s many years of partnership with patients, their families, and physicians in the epilepsy community to develop a much needed, novel medicine,” GW CEO Justin Gover said in a statement. “These patients deserve and will soon have access to a cannabinoid medicine that has been thoroughly studied in clinical trials, manufactured to assure quality and consistency, and available by prescription under a physician’s care.” A company representative said the list price for the medication had not been set yet. Patients with Lennox-Gastaut and Dravet syndromes, which typically emerge in the first few years of life, can suffer from debilitating and recurrent seizures, sometimes dozens a day. One in five patients is estimated to die before they are 20 years old. There are six other drugs approved to treat seizures associated with Lennox-Gastaut, but none approved for Dravet.

Dr Shane, Dr Ailie and Dr Ray discuss coral reefs adapting to sea level rise, snowshoe hares and using fiber-optic cables already on the ocean floor as seismic detectors.First guest: Associate Professor, Department of Paediatrics, University of Melbourne tells us about her work in the use of antibiotics in children.Second guest: David Balding, Melbourne Integrative Genomics, University of Melbourne discusses his work in genomics.Third guest: Aaron Warren, Department of Medicine, University of Melbourne talks about his research into Lennox-Gastaut syndrome, a rare but severe form of epilepsy that typically begins in childhood.Twitter: Einstein A Go Go

Lank and I had a great time with Ricardo and Poe. We are in an important time in history. Take a listen. If you are a parent you will understand Ricardo's story even more. This is #TuffysFight8-year-old Camden resident Tatyana “Tuffy” Rivera.At 10 months old, Tuffy was diagnosed with Lennox-Gastaut syndrome, a rare form of epilepsy that caused upwards of 300 seizures a day at its worst. As a result of her disorder, Tuffy was left unable to speak or feed herself.However, since June 2014, Tuffy’s father, Ricardo Rivera of South Jersey, has been administering medical cannabis to Tuffy in edible candy form. Since then, Rivera says, his daughter’s seizures have “decreased by about 90 percent.” Prior to that, the Riveras had been medicating Tuffy with dozens of different pharmaceuticals to little success.“She was pretty much dying in front of us,” Rivera, who was recently honored by Jersey freeholders for his advocacy, says of his daughter’s condition. “We decided to try something that has a lot fewer side effects, or more mild ones.”However, despite medical marijuana’s success with Tuffy, Rivera and his family have faced an uphill battle at the hands of Gov. Chris Christie’s continued hamstringing of the program. Now, the next phase in the battle is having Tuffy’s school allow her to medicate during the school day, all while dealing with the $500-per-ounce price point and limited dispensary system New Jersey currently runs.“Her brain is so dependent on being able to get this that she seizes more in school than at home,” Rivera says.As for Poe, Tuffy’s Fight is as much a massive failing of New Jersey’s medical marijuana system as it is a cautionary tale for us in Pennsylvania, where we’re currently mulling over a medical marijuana bill.“Without a comprehensive medical marijuana program in place here, most patients and caregivers will face the same uphill battle as the Riveras,” Poe says. “I won’t let that happen here.”Article Via-Philly.com **Update: Since this article was posted Tuffy has now learned to say No and push around on a scooter** 

Pediatric airway management is a skill that integrates the three types of knowledge as described by the ancient Greeks: episteme, or theoretical knowledge, techne, or technical knowledge, and phronesis, or practical wisdom, also called prudence. Here we’ll invoke each type of knowledge and understanding as we go beyond the anatomical issues in pediatric airway management – to the advanced decision-making aspect of RSI and the what-to-do-when the rubber-hits-the road. Case 1: Sepsis Laura is a 2-month-old baby girl born at 32 weeks gestational age who today has been “breathing fast” per mother.  On arrival she is in severe respiratory distress with nasal flaring and intercostal retractions.   Her heart rate is 160, RR 50, oxygen saturation is 88% on RA.  She has fine tissue-paper like rales throughout her lung fields.  Despite a trial of a bronchodilator, supplemental oxygen, even nasal CPAP and fluids, she becomes less responsive and her heart rate begins to drop relatively in the 80s to 90s – this is not a sign of improvement, but of impending cardiovascular collapse. She is in respiratory failure from bronchiolitis and likely viral sepsis.  She needs her airway taken over. Is this child stable enough for intubation? We have a few minutes to optimize, to resuscitate before we intubate. Here’s an easy tip: use the sterile flushes in your IV cart and push in 20, 40, or 60 mL/kg NS.  Just keep track of the number of syringes you use – it is the fastest way to get a meaningful bolus in a small child. Alternatively, if you put a 3-way stop-cock in the IV line and attach a 30 mL syringe, you can turn the stop cock, draw up stream from the IV bag into the syringe, turn te stop cock, and push the fluid in the IV. Induction Agent in Sepsis The consensus recommendation for the induction agent of choice for sepsis in children is ketamine. Etomidate is perfectly acceptable, but ketamine is actually a superior drug to etomidate in the rapid sequence intubation of children in septic shock.  It rapidly provides sedation and analgesia, and supports hemodynamic stability by blocking the reuptake of catecholamines. Paralytic Agent in Sepsis The succinylcholine versus rocuronium debate… Succinylcholine and its PROS 82% of RSI in the ED used succinylcholine (According to the National Emergency Airway Registry, in 2005).  We know it, we are comfortable with it. Succinylcholine produces superior intubating conditions when comparing 1 mg/kg succinylcholine versus 0.6 mg/kg rocuronium, succinylcholine is that at 45 seconds. Succinylcholine and its CONs Raises serum potassium in everyone, typically 0.5 to 1 mEq/L.  That is not usually a problem, but for those with preexisting or inducible hyperkalemia, it can precipitate an arrest, as in renal failure, underlying neurologic or myopathic conditions like multiple sclerosis, muscular dystrophy, ALS, or those who had a stroke or a burn more than 72 hours prior. We often have limited information in critical situations. Succinylcholine gives us a false sense of security.  In children, there really is no “safe apnea” period. Succinylcholine’s effect on the nicotinic receptors results in mydriasis, tachycardia, weakness, twitching and hypertension, and fasciculations (Think nicotine overdose: M/T/W/Th/F). Succinylcholine’s effect on muscarinic receptors manifest (as in organophosphate overdose): SLUDGE – salivation, lacrimation, urination, defecation, GI upset or more apropos here: DUMBBELLS – diarrhea, urination, miosis, bradycardia, emesis, lacrimation, lethargy, salivation. Second dose of succinylcholine – beware of the muscarinic effects and bradycardia. Co-administer atropine, 0.01 mg/kg, up to 0.5 mg IV. Coda: succinylcholine is not that bad – we would not have had such great success with it during the early years of our specialty if it were such a terrible drug.  The side effects are rare, but they can be deadly.  So, what’s the alternative? Rocuronium and its PROs It has none of the side-effects of succinylcholine Rocuronium and its CONs Argument 1: the duration is too long if there is a difficult airway, since rocuronium can last over an hour. Still need to intubate, and now your patient is potentially worse. Argument 2: succinylcholine produces better intubating conditions at 45 seconds compared to rocuronium. At 0.6 mg/kg, rocuronium is inferior to succinylcholine at all time intervals. At 1.0 mg/kg, rocuronium is still inferior at 45 seconds.  At 1.2 mg/kg rocuronium – the dose now commonly recommended – there was no difference in intubating conditions, per a study by Heier et al. in Anethesia and Analgesia in 2000. Case 2: Multitrauma Joseph is a 3-year-old boy who is excited that there are so many guests at his house for a family party and when it’s starting to wind down and the guests begin to leave, he is unaccounted for. An unsuspecting driver of a mini-van backs over him. He is brought in by paramedics, who are now bagging him. Induction Agent in Trauma Need something that is hemodynamically stable – agents such as midazolam or propofol would cause too many problems. Etomidate is a short-acting imidazole derivative that acts on GABA-A receptors to induce loss of consciousness in 5-15 seconds. It can cause apnea, pain on injection, and myoclonus. Etomidate reduces cerebral blood flow, reduces intracranial pressure, and reduces cerebral oxygen consumption, all while maintaining arterial blood pressure and cerebral perfusion pressure. Ketamine is reasonable as well: there is no contraindication to ketamine except for known hydrocephalus. It is safe in head trauma. It is a good choice for the hypotensive trauma patient.  TBI is not a contraindication. In the case of the critically injured child who is normotensive, ketamine will raise his blood pressure and perhaps foster further bleeding.  The goal is a good general perfusion and a balanced resuscitation, ensuring enough cerebral perfusion without disrupting nascent clots.  On the other side of the spectrum, permissive hypotension is not described in children, as hypotension is a late and dangerous sign of shock. Paralytic Agent in Trauma Are your surgeons in an uproar about a long-acting agent and the pupillary response?  Relax, it’s a myth. Caro et al in Annals in 2011 reported that the majority of patients undergoing RSI preserved their pupillary response.  Succinylcholine actually performed worse than rocuronium. In the rocuronium group, all patients preserved their pupillary response. In the critically ill, we rethink your dosing of both the sedative and the paralytic. In a critically ill child or adult, perfusion suffers and it affects how we administer medications.  The patient’s arm-brain time or vein-to-brain time is less efficient; additionally, as the patient’s hemodynamic status softens, he becomes very sensitive to the effects of sedatives. We need to adjust our dosing for a critically ill patient: Decrease the sedative to avoid falling over the hemodynamic compensation cliff. Increase the paralytic to account for prolonged arm-brain time. Case 3: Cardiac/myocarditis/congenital heart disease Jacob is a 6-year-old-boy with tricuspid atresia s/p Fontan procedure who’s had one week of runny nose, cough, and now 2 days of high fever, vomiting, and difficulty breathing. The Fontan procedure is the last in a series of three palliative procedures in a child with complex cyanotic congenital heart disease with a single-ventricle physiology. The procedure reroutes venous blood to flow passively into the pulmonary arteries, because the right ventricle has been surgically repurposed to be the systemic pump.  The other most common defect with an indication for a Fontan is hypoplastic left heart syndrome. Typical “normal” saturations are 75 and 85% on RA.  Ask the parents or caregiver. Complications of the Fontan procedure include heart failure, superior vena cava syndrome, and hypercoagulable state, and others. A patient with a Fontan can present in cardiogenic shock from heart failure, distributive shock from an increased risk of infection, hypovolemic shock from over-diuresis or insensible fluid loss – or just a functional hypovolemia from the fact that his venous return is all passive – and finally obstructive shock due to a pulmonary thromboembolism. Types of shock mnemonic: this is how people COHDe – Cardiogenic, Obstructive, Hypovolemic, Distributive. Do we give fluids? Children after palliative surgery for cyanotic heart disease are volume-dependent.  Even if there is a component of cardiogenic shock, they need volume to drive their circuit.  Give a test dose of 10 mL/kg NS. Pressors in Pediatric Shock Children compensate their shock state early by increasing their SVR. Epinephrine (adrenaline) is great at increasing the cardiac output (with minimal increase in systemic vascular resistance; tachycardia)  In children the cardiac deleterious effects are not pronounced as in adults.  Later when the child is stabilized, other medication such as milrinone (ionotrope and venodilator) can be used. Epinephrine is also fantastic for cold shock when the patient is clamped down with cold extremities – the most common presentation in pediatric septic shock. Norepinephrine (noradrenaline) is best used when you need to augment systemic vascular resistance, such as in warm shock, where the patient has loss of peripheral vascular tone. Induction Agent in Cardiogenic Shock A blue baby – with a R –> L shunt – needs some pinking up with ketamine A pink baby – with a L –> R shunt – is already doing ok – don’t rock the boat – give a neutral agent like etomidate. Myocarditis or other acquired causes of cardiogenic shock – etomidate. Case 4: Status Epilepticus Jessica is a 10-year-old girl with Lennox-Gastaut syndrome who arrives to your ED in status epilepticus. She had been reasonably controlled on valproic acid, clonazepam, and a ketogenic diet, but yesterday she went to a birthday party, got into some cake, and has had stomach aches – she’s been refusing to take her medications today. On arrival, she is hypoventilating, with HR 130s, BP 140/70, SPO2 92% on face mask. She now becomes apneic. Induction Agent in Status Epilepticus Many choices, but we can use the properties of a given agent to our advantage. She is normo-to-hypertensive and tachycardic. She has been vomiting. A nice choice here would be propofol. Propofol as both a sedative and anti-epileptic agent works primarily on GABA-A and endocannabinoid receptors to provide a brief, but deep hypnotic sedation.  Side effects can include hypotension, which is often transient and resolves without treatment.  Apnea is the most common side-effect. Ketamine would be another good choice here, for its anti-epileptic activity. Paralytic Agent in Status Epilepticus Rocuronium (in general), as there are concerns of a neurologic comorbidity. Housekeeping in RSI What size catheter doe I use?  If you know your ETT size, then it is just a matter of multiplication by 2, 3, 4, or 5. Remember this: 2, 3, 4 – Tube, Tape, Tap The NG/OG/Foley is 2 x the ETT – tube The ETT should be taped at a depth of 3 x the ETT size – tape A chest tube size 4 x the ETT – tap In summary, in these cases of sepsis, multitrauma, cardiogenic shock, and status epilepticus: Resuscitate before you intubate Use the agent’s specific properties and talents to your benefit Adjust the dose in critically ill patients: decrease the sedative, increase the paralytic Have post-intubation care ready: sedation, verification, NG/OG/foley

Pippa Hull sits on her mother’s lap across the kitchen table in their Parkville home. She is an outgoing and talkative seven-year-old girl, who just happens to have a rare and severe form of epilepsy called Lennox-Gastaut syndrome. Pippa’s mother, Megan, said this form of epilepsy is characterized by its lack of response to treatments. Hull said they have tried different medications, they have had a VNS or Vagus Nerve Stimulation device implanted in Pippa’s chest, and they have even tried a special diet to try and reduce the number of seizures Pippa experiences.

Vorlesung im TR 3, Mesiale Temporallappen-Epilepsien

Recently, the US Federal Drug Administration (FDA) approved a new medication for seizures and epilepsy, Rufinamide. Rufinamide will soon be available for us in the US. In this episode of Hallway Conversations, Dr. Joseph Sirven, Professor of Neurology at Mayo Clinic Arizona and Editor-in-Chief of Epilepsy.com/Professionals, interviews Dr. Greg Krauss an international renowned epilepsy specialist from Johns Hopkins University. This show will be taped live but no questions will be answered live on the air.