Abstracts

Sustained Seizure Control with 2 Years of Ganaxolone Treatment for Cyclin-dependent Kinase-like 5 (CDKL5) Deficiency Disorder (CDD) in the Marigold Study Open-Label Extension (OLE)

Abstract number : 2.263
Submission category : 7. Anti-seizure Medications / 7B. Clinical Trials
Year : 2023
Submission ID : 588
Source : www.aesnet.org
Presentation date : 12/3/2023 12:00:00 AM
Published date :

Authors :
Presenting Author: Elia Pestana-Knight, MD – Cleveland Clinic

Sam Amin, MD – University Hospitals Bristol and Weston; Nadia Bahi-Buisson, MD, PhD – Necker Enfants Malades University Hospital; Orrin Devinsky, MD – New York University Langone Comprehensive Epilepsy Center; Eric Marsh, MD, PhD – University of Pennsylvania Perelman School of Medicine and Division of Neurology; Rajsekar Rajaraman, MD – UCLA Mattel Children's Hospital; Alex Aimetti, PhD – Marinus Pharmaceuticals, Inc.; Eva Rybak, PharmD – Marinus Pharmaceuticals, Inc.; Fanhui Kong, PhD – Marinus Pharmaceuticals, Inc.; Ian Miller, MD – Marinus Pharmaceuticals, Inc.; Joseph Hulihan, MD – Marinus Pharmaceuticals, Inc.; Scott Demarest, MD, MSCS – University of Colorado School of Medicine

Rationale:
In the 17-week double-blind phase 3 Marigold Study (NCT03572933), 101 patients aged 2–19 years with CDD were treated with adjunctive ganaxolone (GNX) oral suspension which significantly reduced median 28-day major motor seizure frequency (MMSF) versus placebo. In the open-label extension (OLE) phase of the study, an additional 24 months of treatment with GNX further reduced MMSF versus baseline in patients who continued treatment. While the primary outcome of the OLE was to evaluate safety, here we also describe secondary endpoints such as responder analyses, reduction in the number of days with seizures, Caregiver Global Impression of Change–Improvement (GCI-I), and CGI–Seizure Intensity and Duration (CGI-CSID) score through 24 months.

Methods:
Patients who continued into the OLE underwent a four week blinded crossover from their maintenance dose of double-blind study medication to open-label GNX. The target dose of open-label GNX was 21 mg/kg TID (for patients <
Anti-seizure Medications