Switch to Fycompa helps seizures due to high body temperature
Researchers recommended discontinuing Zonegra in this instance
Recurrent seizures in a man with Dravet syndrome and high body temperature were managed by treating the recurrent infections and switching from Zonegran (zonisamide) to Fycompa (perampanel), a case study reports.
The researchers recommended that clinicians consider treating infections early and discontinuing Zonegra if the medication causes high body temperature in Dravet patients with repeated seizures.
The case was detailed in “Switching from zonisamide to perampanel improved the frequency of seizures caused by hyperthermia in Dravet syndrome: a case report and published in the Journal of Medical Case Reports.
Dravet syndrome is marked by seizures that last five to 30 minutes or longer that are difficult to control with anti-seizure medications. They may be triggered by elevated body temperature from fever, illness, warm weather, or a warm bath.
Zonegran is an oral medicine approved to treat seizures that may be resistant to other therapies. In several case studies, however, reported side effects included hyperthermia, an abnormally high body temperature, and decreased sweating, called oligohydrosis, which may exacerbate Dravet-related seizures.
Here, researchers in Japan described the case of a 24-year-old man with Dravet who was treated with Zonegran for a long time. He had a repeat pattern of hyperthermia caused by infection, treatment, and persistent seizures, “which caused a vicious cycle of further seizures,” they wrote.
A worsening condition
At 6 months of age, the man had generalized clonic seizures marked by repeated jerking movements of his arms and legs. He had no family history of neuromuscular disease, including epilepsy. He was diagnosed with Dravet due to recurrent seizures triggered by fever or high body temperature before he turned 18 months.
To manage his seizures, he was treated with standard sodium valproate (sold as Depacon), sodium bromide, and Zonegran. During childhood, his seizures were limited to generalized absence seizures, that is, brief, sudden lapses of consciousness, after waking, and partial seizures during sleep. Clonic seizures occurred about once a month when his body temperature rose during a bath.
He displayed developmental delays in motor skills, weakness in his face and mouth muscles, called the bulbar muscles, and his speech was limited to single words. He lived at home under the care of his family.
At age 24, he had aspiration pneumonia, which occurs when food or liquid is breathed into the airways, fever, and generalized clonic seizures, for which he returned to the hospital. His anti-seizure medication regimen had been in place for at least five years.
He was treated with antibiotics for his aspiration pneumonia, but still had clonic seizures with a fever. the frequency of his seizures returned to the pre-admission level after the fever from the infection was resolved.
He returned to the hospital for aspiration pneumonia a year later, but he had respiratory failure and was placed on a breathing and feeding tube.
An infection and seizures caused a high body temperature, sometimes 39 degrees C (102.2 F) or higher, which lasted about three days. His once-a-month seizures worsened to once every two to three days and sometimes every day. This led to life-threatening status epilepticus, marked by prolonged seizures, or a series of shorter epileptic episodes close together. Other treatments were unable to control his ongoing seizures.
The researchers suggested the fever was due to repeated infections and seizures, along with the side effects of Zonegran, which may have contributed to his high body temperature. His family didn’t agree to discontinue Zonegran because it had been used for a long time, however.
Genetic testing showed the man carried a mutation in the SCN1A gene, which is known to cause Dravet in around 80-90% of patients, confirming a Dravet diagnosis. He continued to have aspiration pneumonia, low blood oxygen, fever, and lung, urinary tract, and skin infections, and received various anti-microbial therapies.
Switch to Fycompa to treat seizures
At age 27, his status epilepticus due to hyperthermia continued without infections and he was treated in a hospital with the anticonvulsant sedative thiopental for two weeks. The family agreed to discontinue Zonegran, which was tapered.
At the same time, he began receiving increasing doses of Fycompa, an anti-seizure medication approved in the U.S. for focal seizures, with or without generalized seizures, in children ages 4 and older.
The man’s hyperthermia and associated seizures resolved quickly after discontinuing Zonegran and switching to Fycompa, the dose of which was raised then lowered after he had psychiatric symptoms. The man still has mild seizures a year and a half after the switch to Fycompa, status epilepticus no longer occured. He remained on valproate and sodium bromide.
The man lived in a state of total bed rest without speaking and, while he could raise his upper limbs, his lower limbs were mostly motionless due to lack of use. He lost the ability to swallow.
“In case of repeated seizures with [Dravet syndrome], clinicians should consider treating infections early and discontinuing [Zonegran] if a high body temperature results from [Zonegran],” the researchers wrote.
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