In response to criticism that epilepsy care for children has little impact, healthcare professionals and administrators have developed various service models and strategies to address perceived inadequacies.
An advisory panel from the United States Food and Drug Administration (FDA) has recommended the approval of a novel epilepsy drug that is made up of ingredients from marijuana. The agency normally follows the recommendations of the advisory panels regarding approvals and rejections of applications of new drugs. The recommendation statement came yesterday (19th April 2018).
If this drug gets a green light, it is expected to become the first cannabis-derived prescription medicine to be available in the US. The drug is named Epidiolex and is made by GW Pharmaceuticals from Britain. It contains cannabidiol or CBD that is derived from cannabis. However the drug is not seen to cause any intoxication among the users.
The use of only one of the components of cannabis also makes it different from medical marijuana that is approved for pain management and other conditions around the world and in the United States. Synthetic forms of chemicals in the cannabis plant are also used to treat nausea among cancer patients and in AIDS patients to prevent weight loss.
Dr. Igor Grant, director of the Center for Medicinal Cannabis Research at the University of California San Diego welcomed this new recommendation from the panel saying, “This is a very good development, and it basically underscores that there are medicinal properties to some of the cannabinoids… I think there could well be other cannabinoids that are of therapeutic use, but there is just not enough research on them to say.”
As of now the panel has recommended the use of this new drug for two types of epilepsy only – Lennox-Gastaut syndrome and Dravet syndrome. These are notoriously difficult to treat and most people continue to have seizures despite treatment. Multiple seizures may occur in a day and this makes the children with these conditions vulnerable for developmental and intellectual disabilities. Lennox-Gastaut syndrome can appear in toddlers at around ages 3 to 5 and Dravet syndrome is usually diagnosed earlier. Nearly 30,000 children and adults suffer from Lennox-Gastaut syndrome and similar numbers of people are diagnosed with Dravet syndrome. Due to the small population of diagnosed patients Epidiolex was filed and classified under orphan drug status.
An orphan drug is one that is developed for a relatively rare disease condition. The FDA provides special subsidies and support for development of orphan drugs and often speed tracks their approval process.
The recommendation from the advisory panel is based on the results of three randomized, double-blind, placebo-controlled trials that included patients of both these disease conditions. The agency statement says, “The statistically significant and clinically meaningful results from these three studies provide substantial evidence of the effectiveness of CBD for the treatment of seizures associated with LGS and DS.” They drug causes liver damage but the report says that this could be managed effectively.
The FDA will conduct a final vote for approval of this drug in June. Oral solution of the drug for a small group of patients with these conditions would be allowed.
According to a new study from the Columbia University however, brain cells are continuously added to our brains even when we reach our 70s. This is a process called neurogenesis. Their work is published in a study that appeared in the latest issue of the journal Cell Stem Cell this week.
Lead author Dr. Maura Boldrini, a research scientist at the department of psychiatry, Columbia University and her colleagues investigated the brains of 28 dead people aged between 14 and 79 years. They were studying the effects of aging on the brain’s neuron production. The team examined the brains that were donated by the families of the deceased at the time of death. The brains were frozen immediately at minus-112 degrees Fahrenheit before they could be examined. This preserved the tissues.
Neurogenesis has been shown to decline with age in lab mice and rats as well as in experimental primates. The team wanted to explore if same rates of decline are seen in human brains as well. So they checked the brains samples for developing neurons. These developmental stages included stem cells, intermediate progenitor cells, immature neuronal cells and finally new mature neurons. They focused on the hippocampus region of the brain that deals with memory and emotional control and behavior.
The results revealed that for all age groups, the hippocampus shows new developing neurons. The researchers concluded that even during old age, the hippocampus continues to make new neurons. The differences that they noted with age include reduction in the development of new blood vessels as people got older. The proteins that help the neurons to make new connections are reduced with age. This was a finding that differentiated ageing brains from younger ones, they explained. Boldrini said the new neurons are there in older brains but they make fewer connections than younger brains. This explains the memory losses and decrease in emotional resiliency in older adults she said.
An earlier study last month came from another set of researchers led by University of California San Francisco researcher Arturo Alvarez-Buylla. The study titled, “Human hippocampal neurogenesis drops sharply in children to undetectable levels in adults,” was published first week of March this year in the journal Nature.
The team found that after adolescence there is little or no neurogenesis in the brain. They examined the brains of 17 deceased individuals and 12 patients with epilepsy part of whose brains had been surgically resected. The debate between the two teams continues. Boldrini explained that Buylla’s team had examined different types of samples that were not preserved as her samples had been.
Further the other team examined three to five sections of the hippocampus and not the whole of it she explained. More studies on this needed to make concrete conclusions regarding neurogenesis in the elderly say experts.
Despite this, becoming more independent is a crucial part of growing up for any child. It is important that you are not overly controlling with your child so that, in time, they can become good decision-makers in their own right and will be able to manage their epilepsy themselves.
In this blog post, we give you some helpful tips on letting your child be independent in spite of their epilepsy
Firstly, to encourage and support your child in their epilepsy journey, you must learn as much as you can about the condition. Then you can discuss epilepsy openly and honestly with your child, and establish where boundaries need to be set.
However, it’s important not to let your child’s epilepsy define them. Try not to think of your child as sick or fragile because of their condition – hopefully you will soon reach the stage where their epilepsy is manageable.
Your child’s achievements in life and their general well-being are far more important than their epilepsy, and it is important to remember this when deciding what they can and can’t do.
You might think that certain sports and activities such as cycling or swimming are off limits to your child living with epilepsy. However, it’s always worth consulting your child’s doctor or paediatrician before ruling them out completely – sports can be a great opportunity for your child to gain independence and to make friends.
While some activities may have to be limited (particularly when seizures are unpredictable), it’s important for your child to have as many of the same opportunities as their peers as possible.
Thankfully, technology is available that can make day-to-day living much easier. There are now products thatcan really help to settle your mind about your child’s safety, and allow them to gain the independence they need.
For example, seizure alarms make it possible to always know when your child has a seizure and be able to react accordingly even if you aren’t with them.
Another great solution is the anti-suffocation pillow. These ensure that, if your child has a seizure in the night, they will still be able to breathe. This allows you to be at ease, and your child to have more independence.
If you are a parent of a child living with epilepsy, why not let other parents know any tips or advice that you have in the comments below…
Telerehabilitation, defined as the method by which communication technologies are used to provide remote rehabilitation, although still underused, could be as efficient and effective as the conventional clinical rehabilitation practices. In the literature, there are descriptions of the use of telerehabilitation in adult patients with various diseases, whereas it is seldom used in clinical practice with child and adolescent patients. We have developed a new audiovisual telerehabilitation (AVT) system, based on the multisensory capabilities of the human brain, to provide a new tool for adults and children with visual field defects in order to improve ocular movements toward the blind hemifield. The apparatus consists of a semicircular structure in which visual and acoustic stimuli are positioned. A camera is integrated into the mechanical structure in the center of the panel to control eye and head movements. Patients can use this training system with a customized software on a tablet. From hospital, the therapist has complete control over the training process, and the results of the training sessions are automatically available within a few minutes on the hospital website. In this paper, we report the AVT system protocol and the preliminary results on its use by three adult patients. All three showed improvements in visual detection abilities with long-term effects. In the future, we will test this apparatus with children and their families. Since interventions for impairments in the visual field have a substantial cost for individuals and for the welfare system, we expect that our research could have a profound socio-economic impact avoiding prolonged and intensive hospital stays.
Telerehabilitation, defined as the method by which communication technologies are used to provide remote rehabilitation, although still underused, could be as efficient and effective as the conventional clinical rehabilitation practices (1). In the literature, we can find some descriptions of the use of telerehabilitation in adult patients for various types of disorder, whereas it is seldom used in clinical practice with children and adolescents (2).
The development and use of telerehabilitation program are slow because they are affected by many logistical factors, such as regional economic resources, medical technical support systems, and population quality, but their potential is very high, as they are conceived and studied to improve patients’ ability to perform activities from daily life, thereby increasing their independence (3). For example, for adult post-stroke patients, telerehabilitation is widely used with the main goal of giving disabled people the same quality of motor, cognitive, and neuropsychological rehabilitation at home as they would have in-home visit and day-care rehabilitation (4, 5–7).
So far, the application of telerehabilitation during childhood has been primarily limited to preterm babies (8) and children with hemiplegia (9, 10), with autism spectrum disorders (11), with speech and language disorders (12, 13), and with learning difficulties (14–16). Despite the well-known impact of visual defects on cognitive functioning and neurological recovery (17), no study has yet investigated the application of telerehabilitation with children with visual impairments.
Here, we describe an innovative telerehabilitation platform, which consists in an audiovisual telerehabilitation (AVT) system, developed for children and adults with visual field defects caused by post-chiasmatic brain lesions. The AVT system allows patients to exercise independently, in an intensive, active, and functional way and in a familiar environment, under remote supervision; it consists of a mobile device platform with remote control, which is accessible directly from home and suitable both for adults, adolescents, and children from the age of 8.
The AVT system is based on a very promising multisensory audiovisual therapy, originally developed for the treatment of adults and children with visual field defects caused by brain lesions (18, 19). Basically, this training aims to stimulate multisensory integration mechanisms in order to reinforce visual and spatial compensatory functions (i.e., implementation of oculomotor strategies). In this first phase of the study, we tested the feasibility and efficacy of AVT in three adult patients with chronic visual field defects, in order to explore how the apparatus can be implemented at home.[…]
July 19, 2017
The number of available anti-seizure medications has exploded in the past two decades, going from just a handful of medicines available in the 1990s to more than 20 now. Once the Food and Drug Administration (FDA) approves each new medicine based on trials in adults, it’s available for clinicians to prescribe off-label to all age groups. However, says William D. Gaillard, M.D., division chief of Child Neurology and Epilepsy, Neurophysiology and Critical Care Neurology at Children’s National Health System, trials that lead to FDA approval for adults do not provide any information about which medications are best for children.
“With so many medications and so little data,” Dr. Gaillard says, “one might think doctors would choose a wider variety of medicines when they prescribe to children with epilepsy.”
However, the results from a recent study by Dr. Gaillard and colleagues, published online in Pediatric Neurology on June 27, 2017, show otherwise. The study indicates that doctors in the United States appear to have reached an unexpected consensus about which medication to prescribe for their pediatric patients.
The study is part of a broader effort to collect data on the youngest epilepsy patients — those younger than 3 years old, the age at which epilepsy most often becomes evident. As part of this endeavor, researchers from 17 U.S. pediatric epilepsy centers enrolled in the study 495 children younger than 36 months old who had been newly diagnosed with non-syndromic epilepsy (a condition not linked to any of the commonly recognized genetic epilepsy syndromes).
The researchers mined these patients’ electronic medical records for information about their demographics, disease and treatments. About half of the study participants were younger than 1 year old when they were diagnosed with epilepsy. About half had disease marked by focal features, meaning that their epilepsy appeared to originate from a particular place in the brain. Nearly all were treated with a single medication, as opposed to a cocktail of multiple medicines.
Of those treated with a single medication, nearly all were treated with one of five medicines: Levetiracetam, oxcarbazepine, phenobarbital, topiramate and zonisamide. However, the data showed a clear prescribing preference. About 63 percent of the patients were prescribed levetiracetam as a first choice. By contrast, oxcarbazepine and phenobarbital, the next most frequently prescribed medicines, were taken by patients as a first choice by a mere 14 percent and 13 percent respectively.
Even more striking, of the children who were not prescribed levetiracetam initially but required a second medication due to inadequate efficacy or unacceptable side effects, 62 percent also received this medication. That made levetiracetam the first or second choice for about 74 percent of all the children in the study, despite the availability of more than 20 anti-seizure medications.
It’s not clear why levetiracetam is such a frequent choice in the United States, says Dr. Gaillard. However, in its favor, the drug is available in a liquid formulation, causes no ill effects medically and can be started intravenously if necessary. Studies have shown that it appears to be effective in controlling seizures in about 40 percent of infants.
Yet, levetiracetam’s market dominance appears to be a North American phenomenon, the study authors write. A recent international survey that Dr. Gaillard also participated in suggests that outside of this continent, carbazepine and oxcarbazepine were the most frequently prescribed medications to treat focal seizures.
What’s really necessary, Dr. Gaillard says, is real data on efficacy for each of the medications commonly prescribed to pediatric epilepsy patients–a marked vacuum in research that prevents doctors from using evidence-based reasoning when making medication choices.
“This study identifies current practices, but whether those practices are correct is a separate question,” he explains. “Just because a medication is used commonly doesn’t mean it is the best medication we should be using.”
To answer that question, he says, researchers will need to perform a head-to-head clinical trial comparing the top available epilepsy medications in children. This study sets the stage for such a trial by identifying which medications should be included.
“Uncontrolled pediatric epilepsy can have serious consequences, from potential problems in development to a higher risk of death,” Dr. Gaillard says. “You want to use the optimal medicine to treat the disease.”
By the time epilepsy patient Erika Fleck came to Loyola Medicine for a second opinion, she was having three or four seizures a week and hadn’t been able to drive her two young children for five years.
“It was no way to live,” she said.
Loyola epileptologist Jorge Asconapé, MD, recommended surgery to remove scar tissue in her brain that was triggering the seizures. Neurosurgeon Douglas Anderson, MD, performed the surgery, called an amygdalohippocampectomy. Ms. Fleck hasn’t had a single seizure in the more than three years since her surgery.
“I’ve got my life back,” she said. “I left my seizures at Loyola.”
Surgery can be an option for a minority of patients who do not respond to medications or other treatments and have epileptic scar tissue that can be removed safely. In 60 to 70 percent of surgery patients, seizures are completely eliminated, and the success rate likely will improve as imaging and surgical techniques improve, Dr. Anderson said.
Traditionally, patients would have to try several medications with poor results for years or decades before being considered for surgery, according to the Epilepsy Foundation. “More recently, surgery is being considered sooner,” the foundation said. “Studies have shown that the earlier surgery is performed, the better the outcome.” (Ms. Fleck is a service coordinator for the Epilepsy Foundation North/Central Illinois Iowa and Nebraska.)
Dr. Asconapé said Ms. Fleck was a perfect candidate for surgery because the scar tissue causing her seizures was located in an area of the brain that could be removed without damaging critical structures.
Ms. Fleck experienced complex partial seizures, characterized by a deep stare, unresponsiveness and loss of control for a minute or two. An MRI found the cause: A small area of scar tissue in a structure of the brain called the hippocampus. The subtle lesion had been overlooked at another center.
Epilepsy surgery takes about three hours, and patients typically are in the hospital for two or three days. Like all surgery, epilepsy surgery entails risks, including infection, hemorrhage, injury to other parts of the brain and slight personality changes. But such complications are rare, and they pose less risk to patients than the risk of being injured during seizures, Dr. Asconapé said.
Loyola has been designated a Level Four Epilepsy Center by the National Association of Epilepsy Centers. Level Four is the highest level of specialized epilepsy care available. Level Four centers have the professional expertise and facilities to provide the highest level of medical and surgical evaluation and treatment for patients with complex epilepsy.
Loyola’s comprehensive, multidisciplinary Epilepsy Center offers a comprehensive multidisciplinary approach to epilepsy and seizure disorders for adults and children as young as two years old. Pediatric and adult epileptologist consultation and state-of-the-art neuroimaging and electrodiagnostic technology are used to identify and assess complex seizure disorders by short- and long-term monitoring.
XOOM is a novel interactive tool that allows non ICT-specialists to create web-based applications of Wearable Immersive Virtual Reality (WIVR) technology that use 360° realistic videos as interactive virtual tours. These applications are interesting for various domains that range from gaming, entertainment, cultural heritage, and tourism to education, professional training, therapy and rehabilitation. 360° interactive videos are displayed on smart-phones placed on head-mounted VR viewers. Users explore the virtual environment and interact with active elements through head direction and movements. The virtual scenarios can be seen also on external displays (e.g., TV monitors or projections) to enable other users to participate in the experience, and to control the VR space if needed, e.g., for education, training or therapy purposes. XOOM provides the functionality to create applications of this kind, import 360° videos, concatenate them, and superimpose active elements on the virtual scenes, so that the resulting environment is more interactive and is customized to the requirement of a specific domain and user target. XOOM also supports automatic data gathering and visualizations (e.g., through heat-maps) of the users’ experience, which can be inspected for analytics purposes, as well as for user evaluation (e.g., in education, training, or therapy contexts). The paper describes the design and implementation of XOOM, and reports a case study in the therapeutic context.