Approved LGS therapy may safely reduce convulsive seizures in adults
Benefits seen for 6 UK patients with treatment-resistant epilepsy
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Seizures typically are caused by sudden, uncontrolled surges of electrical activity in the brain. (Image from iStock)
Use of Fintepla (fenfluramine), an approved oral therapy for Lennox-Gastaut syndrome (LGS), was found to safely reduce convulsive seizures in a small group of adults with hard-to-treat developmental and epileptic encephalopathy, a severe type of epilepsy that includes LGS.
That’s according to a new case series from the U.K., which detailed the drug’s use among six people within the LGS spectrum who were given Fintepla for treatment-resistant convulsive seizures. The researchers reported that the therapy was well tolerated and, in addition to cutting seizure frequency, reduced both hospitalizations and the use of rescue medications for these individuals.
Overall, five of the patients “were rated as much/very much improved,” the team wrote.
The researchers noted that this patient population typically faces a severe burden in terms of the disease, its treatments, and the costs they incur, noting these individuals experience “regular prolonged seizures necessitating intensive use of emergency care.”
“There is a clear need for tailoring existing treatments within this diverse and complex population,” the researchers wrote, calling for “larger real-world studies” to test Fintepla’s use for such patients.
Titled “Effect of Fenfluramine on convulsive seizures and rescue medication usage: A case series of adults within the Lennox-Gastaut syndrome spectrum,” the work was published as a short communication in the journal Epilepsy & Behavior Reports.
Developmental and epileptic encephalopathy is characterized by seizures and a delay in development or even loss of acquired skills. In LGS, seizures begin in childhood and may become less frequent during adulthood. However, convulsive seizures, which involve sudden, uncontrolled body movements, can continue despite treatment.
Testing Fintepla as an add-on antiseizure med
Fintepla is approved in the U.S. and Europe to reduce seizures in adults and children ages 2 and older with LGS. Here, the research team looked at whether adding Fintepla to existing antiseizure medications could reduce convulsive seizures in adults with genetically caused developmental and epileptic encephalopathy with symptoms that fit LGS.
Their study involved four women and two men who ranged in age from 23 to 32 when they started Fintepla. All had severe or profound intellectual disability that required continuous daily supervision.
Before starting Fintepla, each had tried at least eight antiseizure medications. Four of the individuals had a vagus nerve stimulator, a device that sends electrical signals to a nerve in the neck to help control seizures.
All but one patient had convulsive seizures every day and regularly needed midazolam, a rescue medication given when a seizure lasts too long or when several seizures occur close together, known as clusters. Three of the five were hospitalized at least once a month because of seizures, while the other two had seizure clusters requiring hospitalization at least every two months.
Altogether, the six took Fintepla for an average of 23 months, or nearly two years. Three had started treatment under a compassionate-use program before it was officially licensed in the U.K.
Half of patients saw at least a 75% drop in hospital admissions
According to the researchers, all six individuals experienced a significant reduction in generalized tonic-clonic seizures, which cause loss of consciousness followed by stiffening and rhythmic shaking of the body.
The reduction in seizures was accompanied by less use of rescue medications. Among the five patients who regularly needed such treatments, use tended to decrease. In four patients, cannabidiol, approved for LGS under the brand name Epidiolex, was gradually reduced after Fintepla was started, while seizures remained under control, per the team.
The researchers noted that three patients had at least a 75% reduction in hospital admissions due to seizures.
Caregivers rated overall change using the Clinical Global Impression of Improvement (CGI-I), a scale that describes the extent to which a patient’s condition has improved. Five of the six patients were rated by caregivers as much or very much improved. The remaining patient was rated as minimally improved. No patient was rated as unchanged or worse, the team noted.
[The] corresponding reduction in use of rescue medications and hospitalisations [with Fintepla] has the potential to impact on both patient and caregiver quality of life.
Fintepla was generally well-tolerated. All of the patients continued taking it, and no serious side effects were observed. However, because only six individuals were studied and there was no comparison group, the researchers noted that larger studies are needed to determine how consistently these findings apply to other adults with developmental and epileptic encephalopathy within the LGS spectrum.
Overall, “there was a significant reduction in seizures at last follow up [after Fintepla treatment] … with a corresponding reduction in use of rescue medications and hospitalisations,” the researchers wrote. The team noted that use of Fintepla as an add-on treatment “was tolerated without significant side effects.”
While the researchers noted the small number of patients followed and the need for further study, they concluded that benefits were seen with the drug’s use.
The team added: “[The] corresponding reduction in use of rescue medications and hospitalisations [with Fintepla] has the potential to impact on both patient and caregiver quality of life.”
