Long-Term Advantages of Fenfluramine for Lennox-Gastaut Syndrome: Kelly Knupp, MD

The pediatric neurologist and epilepsy specialist at Colorado Children’s Hospital discussed new data presented at AAN 2022 on the use of recently approved fenfluramine in patients with Lennox-Gastaut syndrome. [WATCH TIME: 3 minutes]

WATCH TIME: 3 minutes

"What I’ve learned in my experience with this medication is that sometimes it’s helpful to make an adjustment and then watch for 4 weeks before making the next adjustment. That’s how the open-label extension was designed, but a number of my patients saw ongoing improvement over that 4-week period without us making an adjustment in their medication. Perhaps we do need slower titration to see the full benefit of this medication."

After originally receiving approval to treat seizures associated with Dravet syndrome in June 2020, the FDA recently approved fenfluramine (Fintepla; Zogenix) to include another rare childhood epileptic encephalopathy, Lennox-Gastaut syndrome (LGS). The approval decision was made based on data from the phase 3 Study 1601 (NCT03355209), which showed that treatment with fenfluramine at a dose of 0.7 mg/kg per day was superior to placebo in reducing monthly drop seizure frequency. At the 2022 American Academy of Neurology (AAN) Annual Meeting, April 2-7, in Seattle, Washington, lead investigator Kelly Knupp, MD, and colleagues presented an interim analysis from the ongoing open-label extension of Study 1601.

Each patient included in the analysis initially started on 0.2 mg/kg/day fenfluramine and were titrated to effectiveness/tolerability after 1 month. After 12 months of treatment, fenfluramine was well tolerated, showed sustained reductions in drop seizure frequency, and did not result in any cases of valvular heart disease or pulmonary arterial hypertension.1 Knupp, associate professor of neurology and epilepsy specialist at Children’s Hospital Colorado, told NeurologyLive® that her personal experiences with titrating patients have proven to be more effective uses of the treatment.

At AAN 2022, Knupp sat down to break down the open-label data in detail, highlight some of the most notable findings, and share some of the positive aspects of fenfluramine the LGS community may not yet be aware of regarding its use as a therapy.

Click here for more coverage of AAN 2022.

REFERENCES
1. Knupp K, Scheffer I, Ceulemans B, et al. Interim analysis of long-term safety and efficacy of Fintepla (fenfluramine) in patients with Lennox-Gastaut syndrome. Presented at: 2022 AAN Annual Meeting; April 2-7; Seattle, Washington. Abstract 3305