Prediction programs developed for drugs in juvenile myoclonic epilepsy

Neurologists generally expect most patients with JME to get rid of seizures completely with medication

Researchers at the UMC Utrecht Brain Center have developed two prediction programs for medications in juvenile myoclonic epilepsy. These models predict which patient responds well to medications and who has a low risk of re-attacks when tapering the medication. This ensures that patients do not always have to take lifelong medications and that side effects such as fatigue, nausea, behavioral changes and obesity can be limited. The prediction programme, developed together with researchers from eighteen countries, is now available online for healthcare professionals.

Worldwide, approximately four million people suffer from juvenile myoclonic epilepsy (JME). This is a common form of epilepsy that often manifests itself in childhood. Despite existing drug treatments, a third of patients continue to have seizures. Two-thirds don’t, but these patients are often advised to continue using the drugs for life, with unpleasant side effects.

Despite the differences, the same advice
Neurologists generally expect most patients with JME to get rid of seizures completely with medication. But until now it was not possible to say how great this possibility was. What patients can expect in the long run was equally impossible to predict. “When patients have been seizure-free for a few years, they often ask me if they still need the drug,” says trainee neurologist and researcher Remi Stevelink. Most people want to take fewer pills to get rid of any side effects. Because many people with JME have attacks again after tapering the drug, with all the associated risks, neurologists around the world advise against trying this. “Despite the fact that there are many differences among people with JME, nearly everyone gets the same advice,” says Remi.

Look for models
To predict how patients respond to medications and who can safely taper off medications, Remi and colleagues at UMC Utrecht collaborated with researchers from eighteen countries. The researchers collected information about the disease course of 2,518 people with JME. They found that as many as a third of people continue to have seizures despite medication, far more than most neurologists previously thought. Looking for patterns in the data, the researchers found nine different predictors of freedom to attack. For example, having three different types of seizures (triggered by menstruation and psychiatric illness) appears to be predictive of poor treatment outcome. “We combined these predictors into a model that allows us to calculate the person’s chance of being seizure free with treatment,” Remi says.

Lifelong medication unnecessary for every patient
Of the patients who tried to taper off their medications, three-quarters had attacks again. By looking at the differences between people with and without relapses, Remi and his colleagues found three strong predictors, which they combined into a second prediction model. “Elderly people in particular who have been seizure-free for several years and who use only one epilepsy medication have a high probability of remaining seizure-free after tapering their medications gradually,” says Remi. Of the people who experienced seizures again after tapering off, nearly all were found to become seizure-free again after resuming their medications. Remi: “Because we can now predict who can safely taper off their medications, it’s not necessary for everyone with JME to be on their medications for life.”

Personalized treatment
The researchers translated the two models into a handy online forecasting program. This software can calculate the likelihood of someone responding to treatment and the likelihood of remaining seizure-free after tapering medications. The program, intended for healthcare professionals, is available free of charge and easy to consult online, just like previous forecasting models from the same research group. “From now on I can better predict the treatment outcome of the patient sitting in front of me in the office with just a few clicks of the mouse,” continues Remi. “And this goes for all my colleagues. We hope to contribute to tailored care and advice for people with JME around the world.”

The results of this research are recent published in eClinicalMedicinean open access journal from the Lancet.

Expertise center for complex epilepsy
UMC Utrecht has more than 30 centers of expertise for rare diseases (ECZA). One of them is the competence center for complex epilepsy. This is where children, youth and adults can go with different forms of epilepsy. Many different specialists work together in this center. In order to continuously improve patient care, the researchers of this center study the different forms of epilepsy, the evolution of the disease and the use of drugs. At European level, this competence center is part of the EpiCARE European Reference Network (ERN). Together, care providers and researchers ensure a good quality of care according to the latest medical findings. A golden combination for rare diseases.