Seizures, Febrile

Viral infections trigger more cases of intussusception, the common cause of bowel blockages in young children, than previously thought, according to a new study. Viral infections trigger more cases of intussusception, the common cause of bowel blockages in young children, than previously thought, according to a new study. The research, led by Murdoch Children's Research Institute (MCRI) and published in Clinical Infectious Diseases, found during the COVID-19 lockdowns hospital admissions for intussusception, a medical emergency involving obstruction of the intestine, among young children significantly decreased. For the study, 12 years of data was analysed across Victoria, NSW and Queensland. A total of 5,589 intussusception cases were recorded between January, 2010 and April, 2022. Of those, 3,179 were children under the age of two. During the lockdown periods, Victoria and NSW experienced a decline in hospital admissions for intussusception among children under two by 62.7 per cent and 40.1 per cent, respectively. The rate of intussusception cases has now returned to normal levels. MCRI and Monash University researcher Dr Ben Townley said the magnitude of the decline supported that common respiratory diseases such as colds, the flu and respiratory syncytial virus (RSV), were behind a significant proportion of intussusception cases. "Reductions in intussusception hospital admissions were seen in all age groups, however most occurred in children less than two years of age," he said. "Intussusception is the leading cause of acute bowel obstruction in infants and young children and without prompt diagnosis and management, can be fatal. "Countries with prolonged COVID-19 lockdowns and suppression strategies saw reductions in common respiratory viruses, which influenced the drop in intussusception admissions." Victoria experienced the greatest lockdown duration, with Melbourne having six lockdown periods, for a total of 263 days. Greater Sydney had 159 days and Brisbane had 18 days in lockdown. MCRI Professor Jim Buttery said the decrease in intussusception cases was greater than expected given previous research into the causes of the condition. "Our analysis found commons viruses play a larger role than previously recognised in triggering intussusception," he said. Infectious triggers were thought to comprise only a minority, about 30 per cent, of cases." Professor Buttery said the findings raised the possibility that emerging vaccines like the new RSV vaccines may help prevent intussusception. "When a new vaccine against common childhood respiratory viruses is introduced, we may find there are some unexpected benefits, like protecting more children from intussusception," he said. We last saw this in 2007, when introducing the rotavirus vaccine against gastroenteritis, also reduced febrile convulsions in young children." Researchers fromSydney Children's Hospital Network, University of Melbourne and Queensland Health also contributed to the findings.

NEW YORK, Jan. 4, 2024 /PRNewswire/ -- In a study designed to better understand sudden, unexpected deaths in young children, which usually occur during sleep, researchers have identified brief seizures, accompanied by muscle convulsions, as a potential cause. Experts estimate in excess of 3,000 families each year in the United States lose a baby or young child unexpectedly and without explanation. Most are infants in what is referred to as sudden infant death syndrome, or SIDS, but 400 or more cases involve children aged 1 and older, and in what is called sudden unexplained death in children (SUDC). Over half of these children are toddlers. The study findings come from a registry of more than 300 SUDC cases, set up a decade ago by researchers at NYU Grossman School of Medicine. Researchers used extensive medical record analysis and video evidence donated by families to document the inexplicable deaths of seven toddlers between the ages of 1 and 3 that were potentially attributable to seizures. These seizures lasted less than 60 seconds and occurred within 30 minutes immediately prior to each child's death, say the study authors. For decades, researchers have sought an explanation to sudden death events in children, noticing a link between those with a history of febrile seizures (seizures accompanied by fever). Earlier research had reported that children who died suddenly and unexpectedly were 10 times more likely to have had febrile seizures than children who did not die suddenly and unexpectedly. Febrile seizures are also noted in one-third of SUDC cases registered at NYU Langone Health. Publishing in the journal Neurology online Jan. 4, the new study involved an analysis by a team of eight physicians of the rare SUDC cases for which there were also home video recordings, from either security systems or commercial crib cameras, made while each child was sleeping on the night or afternoon of their death. Five of seven recordings were running nonstop at the time and showed direct sound and visible motion indicative of a seizure happening. The remaining two recordings were triggered by sound or motion, but only one suggested that a muscle convulsion, a sign of seizure, had occurred. As well, only one toddler had a documented previous history of febrile seizures. All children in the study had previously undergone an autopsy that revealed no definitive cause of death. "Our study, although small, offers the first direct evidence that seizures may be responsible for some sudden deaths in children, which are usually unwitnessed during sleep," said study lead investigator Laura Gould, M.Sc., MA, PT, a research assistant professor at NYU Langone. Gould lost her daughter, Maria, to SUDC at the age of 15 months in 1997, a tragedy that prompted her successful lobby for establishment of the NYU SUDC Registry and Research Collaborative. Gould points out that if not for the video evidence, the death investigations would not have implicated a seizure. "These study findings show that seizures are much more common than patients' medical histories suggest, and that further research is needed to determine if seizures are frequent occurrences in sleep-related deaths in toddlers, and potentially in infants, older children, and adults," said study senior investigator and neurologist Orrin Devinsky, MD. Devinsky, a professor in the Departments of Neurology, Neurosurgery, and Psychiatry at NYU Langone, as well as chief of its epilepsy service, adds that "convulsive seizures may be the 'smoking gun' that medical science has been looking for to understand why these children die. "Studying this phenomenon may also provide critical insight into many other deaths, including those from SIDS and epilepsy," said Devinsky, who cofounded the SUDC Registry and Research Collaborative at NYU Langone with Gould. Further research, Devinsky notes, is also needed to determine precisely how seizures with or without fever may induce sudden death. Previous research in epilepsy patients, he says, points to difficulty breathing that is known to occur immediately after a seizure and that can lead to death. This has been found to happen more frequently in epilepsy patients, as it does in the children involved in the study, while they are sleeping face down on the stomach and without anyone witnessing the death. Continuous monitoring of child deaths and improvements in health records to track how often these convulsive seizures precede death, he explains, will be needed for this to be confirmed. Seizure-related deaths are underreported in both people with and without epilepsy. For the study, experts in forensic pathology, neurology, and sleep medicine analyzed each recording for video quality, sound, and motion. From this, they were able to determine which toddlers showed signs of muscle convulsions as a sign of seizures prior to their death and when. Access to the videos was and remains strictly limited to the researchers involved in the study. Funding support for this study was provided by SUDC UK, FACES at NYU Langone Health, and the SUDC Foundation. Additional funding support was provided by the National Center for Advancing Translational Sciences and National Institutes of Health grant UL1TR001445. Besides Gould and Devinsky, other NYU Langone researchers involved in this study are Codi-Ann Reid, BS; and Alcibiades Rodriguez, MD. Co-investigators of the SUDC video study group are Alison Krywanczyk, MD, at the Cuyahoga County Medical Examiner's Office in Cleveland; Kristen Landi, MD, at the New York City Office of the Chief Medical Examiner; Melissa Guzzetta, DO, at the Office of the County Medical Examiner in Middlesex, N.J.; Heather Jarrell, MD, at the Office of the Medical Investigator, University of New Mexico, in Albuquerque; Kelly Lear, MD, at the Arapahoe County Coroner's Office in Centennial, Colo.; Tara Mahar, MD, and Katherine Maloney, MD, at the Erie County Medical Examiner's Office in Buffalo, N.Y.; Declan McGuone, MBBCh, at Yale University in New Haven, Conn.; Alex Williamson, MD, at Zucker School of Medicine at Hofstra/Northwell in Hempstead, N.Y.; Katheryn Pinneri, MD, at the Montgomery County Forensic Service in Conroe, Texas; and Victoria Delavale, MPH, and Daniel Friedman, MD, at NYU Grossman School of Medicine. Media Inquiries: David March 212-404-3528 [email protected] SOURCE NYU Langone Health System

Data from nine abstracts to be presented, including real-world outcomes of Epidiolex® (cannabidiol) in treatment-resistant focal epilepsies Presentations include data on U.S. caregiver-reported seizure and non-seizure outcomes in patients with tuberous sclerosis complex prescribed Epidiolex DUBLIN, Dec. 1, 2023 /PRNewswire/ -- Jazz Pharmaceuticals, Inc. (Nasdaq: JAZZ) today announced that nine company-sponsored presentations, including five late-breaking abstracts, will be shared at the 2023 American Epilepsy Society (AES) annual meeting, being held December 1-5 in Orlando, Florida. Late-breaking presentations include a post-hoc analysis examining real-world outcomes of Epidiolex® (cannabidiol) in treatment-resistant focal epilepsies within the Expanded Access Program (EAP), as well as a post-hoc analysis of the GWPCARE6 Open-Label Extension (OLE) trial evaluating the effectiveness of Epidiolex as add-on therapy against focal seizures in tuberous sclerosis complex (TSC). Two additional late-breaking abstracts will feature interim results from the BECOME-TSC (BEhavior, COgnition, and More with Epidiolex) Survey of caregivers of patients with TSC. These abstracts will report seizure and non-seizure outcomes with real-world use of Epidiolex in TSC and further expand the results of the original BECOME survey conducted among caregivers of people with Dravet syndrome (DS) or Lennox-Gastaut syndrome (LGS) that were presented at the 2022 AES meeting. "Multiple presentations at the 2023 AES meeting continue to build the scientific evidence in support of Epidiolex and improve understanding around the treatment's full impact," said Kelvin Tan, MBBCh, MRCPCH, senior vice president and chief medical officer of Jazz Pharmaceuticals. "In addition to seizure outcomes, the BECOME studies are also designed to assess non-seizure benefits of Epidiolex, such as cognition and executive function, emotional and social function, and language and communication. We are determined to not only address seizure outcomes for patients living with rare epilepsies, but also improve the day-to-day lives for both patients and their loved ones." Data highlights at the 2023 AES annual meeting include: A post-hoc analysis of patients diagnosed with treatment-resistant focal epilepsy within the EAP that demonstrated Epidiolex was associated with a sustained reduction in focal-onset seizures through 144 weeks. Notably, a second post-hoc analysis investigated the effectiveness and safety results of Epidiolex in adult patients from the EAP which found similar outcomes among patients with treatment-resistant epilepsies, including focal seizures. The EAP was an initiative launched in 2014 to provide add-on Epidiolex to patients with treatment-resistant epilepsy at 35 U.S. epilepsy centers. A post-hoc analysis of the GWPCARE6 OLE trial evaluating the effectiveness of Epidiolex as add-on therapy against focal seizures in TSC. At least 50% reduction was reported by the majority of patients across focal seizure types through 144 weeks. Additionally, responder rates for focal seizure subtypes were consistent with the overall focal seizure responder rates, and the safety pro in the analysis was consistent with that observed in its overall clinical development program. Interim results of the BECOME-TSC survey, in which the majority of caregivers reported patient improvements in overall seizure frequency and severity, as well as improvements in patients' cognition, emotional functioning, and communication domains. The 2023 AES abstracts are available online at the following link: . A full list of Jazz Pharmaceuticals' presentations follows below: About Tuberous Sclerosis Complex Tuberous sclerosis complex (TSC) is a rare genetic condition.1 The condition causes mostly benign tumors to grow in vital organs of the body including the brain, skin, heart, eyes, kidneys and lungs2 and is a leading cause of genetic epilepsy.3 People with TSC may experience a variety of seizure types. One of the most common is infantile spasms that typically present in the first year of life; focal (or partial) seizures are also very common.4 TSC is associated with an increased risk of autism and intellectual disability5 and the severity of the condition can vary widely. In some children the disease is very mild, while others may experience life-threatening complications.4 Epilepsy is present in about 85 percent of patients with TSC and may progress to become intractable to medication.4,6,7 More than 60 percent of individuals with TSC do not achieve seizure control8 with standard treatments such as antiepileptic drugs, epilepsy surgery, ketogenic diet, or vagus nerve stimulation8 compared to 30-40 percent of individuals with epilepsy who do not have TSC who are drug resistant.9,10 About Dravet Syndrome Dravet syndrome (DS) is a rare genetic condition that appears during the first year of life with frequent fever-related seizures (febrile seizures). Later, other types of seizures typically arise, including myoclonic seizures (involuntary muscle spasms).11 Additionally, status epilepticus, a potentially life-threatening state of continuous seizure activity requiring emergency medical care, may occur. Children with DS typically experience poor development of language and motor skills, hyperactivity and difficulty relating to others. About Lennox-Gastaut Syndrome Lennox-Gastaut syndrome (LGS) begins in childhood. It is characterized by multiple types of seizures. People with LGS begin having frequent seizures in early childhood, usually between ages 3 and 5.12 More than three-quarters of affected individuals have tonic seizures, which cause the muscles to contract uncontrollably. Almost all children with LGS develop learning problems and intellectual disability. Many also have delayed development of motor skills such as sitting and crawling. Most people with LGS require help with usual activities of daily living. About Epidiolex®/Epidyolex® (cannabidiol) EPIDIOLEX/EPIDYOLEX is a prescription, plant-derived cannabis-based medicine administered as an oral solution which contains highly purified cannabidiol (CBD). Cannabidiol, the active ingredient in EPIDIOLEX, is a cannabinoid that naturally occurs in the Cannabis sativa L. plant. The precise mechanisms by which EPIDIOLEX exerts its anticonvulsant effect in humans are unknown. Epidiolex, approved by the U.S. Food and Drug Administration (FDA) for use in the U.S., the European Commission (EC) for use in Europe, the Medicines and Healthcare products Regulatory Agency (MHRA) for use in Great Britain, the Therapeutic Goods Administration for use in Australia, Swissmedic for use in Switzerland, and the Food & Nutrition Services of the Israel Ministry of Health for use in Israel, is an oral solution which contains highly purified cannabidiol. In the U.S., Epidiolex is indicated for the treatment of seizures associated with Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS) or tuberous sclerosis complex (TSC) in patients one year of age and older. Epidiolex received Orphan Drug Designation from the U.S. FDA for the treatment of seizures associated with LGS, DS and TSC. Epidiolex has received approval in the European Union under the tradename Epidyolex for adjunctive use in conjunction with clobazam to treat seizures associated with LGS and DS in patients two years and older, and for adjunctive use to treat seizures associated with TSC, in patients two years of age and older. Epidyolex has received Orphan Drug Designation from the European Medicines Agency (EMA) for the treatment of seizures associated LGS, DS and TSC. A Phase 3 study is ongoing in Japan evaluating cannabidiol in patients with LGS, DS and TSC. Important Safety Information & Indications CONTRAINDICATION: HYPERSENSITIVITY EPIDIOLEX (cannabidiol) oral solution is contraindicated in patients with a history of hypersensitivity to cannabidiol or any ingredients in the product. WARNINGS & PRECAUTIONS Hepatocellular Injury: EPIDIOLEX can cause dose-related transaminase elevations. Concomitant use of valproate and elevated transaminase levels at baseline increase this risk. Transaminase and bilirubin levels should be obtained prior to starting treatment, at one, three, and six months after initiation of treatment, and periodically thereafter, or as clinically indicated. Resolution of transaminase elevations occurred with discontinuation of EPIDIOLEX, reduction of EPIDIOLEX and/or concomitant valproate, or without dose reduction. For patients with elevated transaminase levels, consider dose reduction or discontinuation of EPIDIOLEX or concomitant medications known to affect the liver (e.g., valproate or clobazam). Dose adjustment and slower dose titration is recommended in patients with moderate or severe hepatic impairment. Consider not initiating EPIDIOLEX in patients with evidence of significant liver injury. Somnolence and Sedation: EPIDIOLEX can cause somnolence and sedation that generally occurs early in treatment and may diminish over time; these effects occur more commonly in patients using clobazam and may be potentiated by other CNS depressants. Suicidal Behavior and Ideation: Antiepileptic drugs (AEDs), including EPIDIOLEX, increase the risk of suicidal thoughts or behavior. Inform patients, caregivers, and families of the risk and advise to monitor and report any signs of depression, suicidal thoughts or behavior, or unusual changes in mood or behavior. If these symptoms occur, consider if they are related to the AED or the underlying illness. Withdrawal of Antiepileptic Drugs: As with most AEDs, EPIDIOLEX should generally be withdrawn gradually because of the risk of increased seizure frequency and status epilepticus. Adverse Reactions: The most common adverse reactions in patients receiving EPIDIOLEX (≥10% and greater than placebo) include transaminase elevations; somnolence; decreased appetite; diarrhea; pyrexia; vomiting; fatigue, malaise, and asthenia; rash; insomnia, sleep disorder and poor-quality sleep; and infections. Hematologic abnormalities were also observed. Pregnancy: EPIDIOLEX should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus. Encourage women who are taking EPIDIOLEX during pregnancy to enroll in the North American Antiepileptic Drug (NAAED) Pregnancy Registry. Drug Interactions: Strong inducers of CYP3A4 and CYP2C19 may affect EPIDIOLEX exposure. EPIDIOLEX may affect exposure to CYP2C19 substrates (e.g., clobazam, diazepam, stiripentol), orally administered P-gp substrates, or other substrates (see full Prescribing Information). Consider dose reduction of orally administered everolimus, with appropriate therapeutic drug monitoring, when everolimus is combined with EPIDIOLEX. A lower starting dose of everolimus is recommended when added to EPIDIOLEX therapy. Concomitant use of EPIDIOLEX and valproate increases the incidence of liver enzyme elevations. Pneumonia was observed more frequently with concomitant use of EPIDIOLEX and clobazam. Dosage adjustment of EPIDIOLEX or other concomitant medications may be necessary. Indications: EPIDIOLEX (cannabidiol) oral solution is indicated for the treatment of seizures associated with Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), or tuberous sclerosis complex (TSC) in patients 1 year of age and older. Please refer to the EPIDIOLEX full Prescribing Information for additional important information here. About Jazz Pharmaceuticals plc Jazz Pharmaceuticals plc (NASDAQ: JAZZ) is a global biopharmaceutical company whose purpose is to innovate to transform the lives of patients and their families. We are dedicated to developing life-changing medicines for people with serious diseases—often with limited or no therapeutic options. We have a diverse portfolio of marketed medicines and novel product candidates, from early- to late-stage development, in neuroscience and oncology. Within these therapeutic areas, we are identifying new options for patients by actively exploring small molecules and biologics, and through innovative delivery technologies and cannabinoid science. Jazz is headquartered in Dublin, Ireland and has employees around the globe, serving patients in nearly 75 countries. Please visit for more information. Investors: Andrea N. Flynn, Ph.D. Vice President, Head, Investor Relations Jazz Pharmaceuticals plc InvestorInfo@jazzpharma.com Ireland +353 1 634 3211 U.S. +1 650 496 2717 References 1 TS Alliance. What is TSC? . Accessed November 2023. 2 National Institute of Neurological Disorders and Stroke. Tuberous Sclerosis Complex. . Accessed November 2023. 3 TS Alliance. What is tuberous sclerosis complex? . Accessed November 2023. 4 Kingswood JC, d'Augères GB, Belousova E, et al. TuberOus SClerosis registry to increase disease Awareness (TOSCA) - baseline data on 2093 patients. 2017;12(1):2. 5 de Vries PJ, Belousova E, Benedik MP, et al. TSC-associated neuropsychiatric disorders (TAND): findings from the TOSCA natural history study. Orphanet J Rare Dis. 2018;13(1):157. 6 Tuberous Sclerosis Alliance. Diagnosis, Surveillance and Management for Healthcare Professionals. . Accessed October 2023. 7 Jeong A, Wong M. Systemic disease manifestations associated with epilepsy in tuberous sclerosis complex. Epilepsia. 2016;57(9):1443-1449. 8 Chu-Shore CJ, Major P, Camposano S, Muzykewicz D, Thiele EA. The natural history of epilepsy in tuberous sclerosis complex. Epilepsia. 2010;51(7):1236-1241. 9 Kwan P, Brodie MJ. Early identification of refractory epilepsy. N Engl J Med. 2000;342(5):314-319. 10 French JA. Refractory epilepsy: clinical overview. Epilepsia. 2007;48 Suppl 1:3-7. 11 Epilepsy Foundation. Dravet Syndrome. . Accessed November 2023. 12 National Organization for Rare Disorders, Inc. Lennox-Gastaut Syndrome: Signs & Symptoms. . Accessed November 2023. Company Codes: NASDAQ-NMS:JAZZ