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Neurology

Orphan Drug Designation From the U.S. FDA for EPX-100 and EPX-200 in the Treatment of Patients With Dravet Syndrome

The U.S. Food and Drug Administration has granted Orphan Drug Designation to EPX-100 and EPX-200 for the treatment of patients with Dravet syndrome. Dravet Syndrome qualifies as a rare pediatric disease under Section 529 of Food, Drug, and Cosmetic Act. The rare, catastrophic, lifelong form of epilepsy begins in the first year of life with frequent or prolonged seizures. Intellectual disability, behavioral abnormalities, gait and motor dysfunction, and increased mortality are commonly observed as the disease progresses. Patients with Dravet Syndrome also suffer with life-threatening seizures that cannot be adequately controlled by available medications, and face a 15-20 percent mortality rate due to SUDEP (Sudden Unexplained Death in Epilepsy), seizure-related accidents such as drowning, o...

Youth with uncomplicated epilepsy have favorable social outcomes as their siblings

A 15-year follow-up study of young adults with epilepsy found that those with uncomplicated epilepsy who were seizure-free for five years or more did as well as their siblings without epilepsy in measures of education, employment, family arrangements and driving status. Youth with complicated epilepsy had worse social outcomes and were less likely to drive, even if living without seizures. Results were published in the journal Epilepsia.

New Guideline Published on Uncommon Risk of Death in Epilepsy American Academy of Neurology

There is an uncommon risk of death that people with epilepsy and their loved ones may not know about. The risk is called sudden unexpected death in epilepsy, or SUDEP. Now the American Academy of Neurology (AAN) and the American Epilepsy Society have co-developed a new guideline on SUDEP, published in the April 24, 2017, online issue of Neurology®, the medical journal of the American Academy of Neurology, and presented at the 69th AAN Annual Meeting in Boston, April 22 to 28, 2017. The guideline is endorsed by the International Child Neurology Association. SUDEP is when someone with epilepsy who is otherwise healthy dies suddenly with no known cause.

Cannabidiol May Reduce Seizures by Half in Hard-to-treat Epilepsy

Promising results from a large-scale, controlled, Phase 3 clinical study of epilepsy patients being treated with cannabidiol will be presented next week at the American Academy of Neurology’s Annual Meeting in Boston on April 25. GW Pharmaceuticals’ liquid oral formulation of cannabidiol (CBD), called Epidiolex, is one of 500 compounds found in cannabis. Unlike the well-known compound, tetrahydrocannabinol (THC), CBD does not produce a “high” as the psychoactive component is absent. Results from the randomized, double-blind, placebo-controlled study found that almost 40 percent of people with Lennox-Gastaut syndrome (LGS) had at least a 50 percent reduction in drop seizures, compared to 15 percent taking a placebo. LGS is a severe form of epilepsy that often results in impaired intellectua...

Study finds genetic basis for drug response in childhood absence epilepsy

Consider two children who have childhood absence epilepsy (CAE), the most common form of pediatric epilepsy. They both take the same drug—one child sees an improvement in their seizures, but the other does not. A new study in the Annals of Neurology identified the genes that may underlie this difference in treatment outcomes, suggesting there may be potential for using a precision medicine approach to help predict which drugs will be most effective to help children with CAE. The study was funded by the National Institute of Neurological Disorders and Stroke (NINDS) and the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), both part of the National Institutes of Health. “A better understanding of genetic factors underlying a disease and the way t...

First Epilepsy Clinical Trial Patients Implanted with Dose-delivering Prometra II Device

The first refractory epilepsy patients have been implanted with Prometra II programmable infusion pumps for a clinical trial of the dose-delivery devices, according to the product’s developers, Flowonix Medical and Cerebral Therapeutics. Dan Abrams, Cerebral Therapeutics’ CEO, said the multi-center ADDRESS trial is the first study of programmable, implantable pumps delivering medication directly to the brain — what is called intracerebroventricular delivery.

Researchers Working on Non-Invasive Method to Suppress Epileptic Seizures

Researchers at Carnegie Mellon University have developed a new device that detects ongoing epileptic seizures and suppresses them as they start, according to a recent study. This approach may evolve into a non-invasive method to treat epileptic seizures.

Silicon Valley firm’s implant helps stop brain seizures

Imagine a seismograph — the instrument that measures and records earthquakes and volcanic eruptions — for your brain. Except this one has a wireless link to a device implanted in your head that stops epileptic seizures at their source, halting the sudden and violent attacks before they happen. It’s not science fiction.

The gene that turns epilepsy treatment deadly

Drug-induced hypersensitivity reactions (DIHRs) are serious and life threatening. A common example is the use of the antiepileptic drug carbamazepine, but the mechanisms that trigger it are unclear. Current scientific consensus holds that people who have a specific variation of the ‘human leukocyte antigen B’ (HLA-B) gene, which provides the code for making a protein that plays a critical role in the immune system, are more at risk of DIHR. However, the mechanism linking this gene to DIHR is currently unknown. As this specific variation, called HLA-B*15:02, is fairly common in people of South-East Asian descent, this is a serious problem for clinicians in the region.

New Epilepsy Classifications

For the first time in almost three decades, the classification for epileptic seizures has been updated. The new system formally recognizes some seizure types, provides additional information on causes, and replaces obscure or questionable words and terms with more meaningful ones, the authors say.

The DEA just gave a big boost to a cannabis-based seizure drug

We are often judged by the company we keep, even unfairly. For decades, that has been the fate of cannabidiol, a chemical compound that has the bad luck to occur naturally in marijuana, the world’s most controversial plant. Because cannabidiol is subject to the same tight legal restrictions on personal and scientific use as is marijuana, its potential medical benefits have been underappreciated — at least up until now.

Stress may trigger seizure for epilepsy patients

For people suffering with epilepsy, facing stressful events such as the war, trauma or natural disaster, or the death of a loved one, may act as a common trigger for seizures, a study has found. Epilepsy is a disorder in which nerve cell activity in the brain is disturbed, causing seizures. The findings showed that higher anxiety levels in patients with epilepsy reported stress as a seizure trigger.

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