The primary purpose of this study is to evaluate the safety and tolerability of ION440.
This is a phase 1-2 randomized, double-blind, sham-controlled, multiple-ascending dose (MAD) study to evaluate ION440 in pediatric and adult participants with MECP2 Duplication Syndrome (MDS) and will be conducted in two parts. During Part 1 (MAD) (36 weeks), participants will be randomized in a 3:1 ratio to receive ION440 or sham. Individuals who complete Part 1 may enter Part 2, an open label long-term extension study (LTE), where they will receive ION440 for up to approximately 156 weeks. Multiple dose cohorts (Dose A, Dose B, and Dose C) will be evaluated in the study. All dosing cohorts will be further subdivided by age. Sub cohort A will include participants 8 through 65 years of age, and sub cohort B will include participants 2 through 7 years of age. Dosing cohorts will be enrolled sequentially with sub cohort A initiating prior to sub cohort B.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
QUADRUPLE
Enrollment
48
ION440 will be administered by intrathecal bolus (ITB) injection.
An LP will be performed with CSF collection but will not be followed by the administration of study treatment by ITB injection.
Rady Children's Hospital
San Diego, California, United States
RECRUITINGUniversity of Colorado Hopsital - Anschutz Medical Campus
Aurora, Colorado, United States
RECRUITINGPart 1: Number of Participants With Treatment-Emergent Adverse Events (TEAEs)
Time frame: Up to approximately 36 weeks
Part 1: Number of Participants With Clinically Significant Change From Baseline in Vital Signs
Time frame: Baseline up to approximately 36 weeks
Part 1: Number of Participants With Clinically Significant Change From Baseline in Physical and Neurological Examination Findings
Time frame: Baseline up to approximately 36 weeks
Part 1: Number of Participants With Clinically Significant Change from Baseline in Laboratory Assessments
Time frame: Baseline up to approximately 36 weeks
Part 1: Number of Participants With Clinically Significant Change From Baseline in Electrocardiogram (ECG)
Time frame: Baseline up to approximately 36 weeks
Part 2: Number of Participants With TEAEs
Time frame: Up to approximately 192 weeks
Part 2: Number of Participants With Clinically Significant Change From Baseline in Vital Signs
Time frame: Baseline up to approximately 192 weeks
Part 2: Number of Participants With Clinically Significant Change From Baseline in Physical and Neurological Examination Findings
Time frame: Baseline up to approximately 192 weeks
Part 2: Number of Participants With Clinically Significant Change from Baseline in Laboratory Assessments
Time frame: Baseline up to approximately 192 weeks
Part 2: Number of Participants With Clinically Significant Change From Baseline in ECG
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Kennedy Krieger
Baltimore, Maryland, United States
RECRUITINGBoston Children's Hospital
Boston, Massachusetts, United States
RECRUITINGGillette Children's Specialty Healthcare
Saint Paul, Minnesota, United States
RECRUITINGChildren's Hospital of Philadelphia
Philadelphia, Pennsylvania, United States
RECRUITINGVanderbilt University Medical Center
Nashville, Tennessee, United States
RECRUITINGBaylor College of Medicine
Houston, Texas, United States
RECRUITINGKepler University Hospital
Linz, Austria
RECRUITINGCHU Dijon Bourgogne
Dijon, France
RECRUITINGTime frame: Baseline up to approximately 192 weeks
Part 1: Maximum Observed Concentration (Cmax) of ION440 in Plasma
Time frame: Pre-dose and at multiple points post-dose up to Week 36
Part 1: Area Under the Concentration-time Curve (AUC) of ION440 in Plasma
Time frame: Pre-dose and at multiple points post-dose up to Week 36
Part 1: Plasma Terminal Elimination Half-life (t½) of ION440
Time frame: Pre-dose and at multiple points post-dose up to Week 36
Part 1: Trough Concentration (Ctrough) of ION440 in Plasma and CSF
Time frame: Pre-dose and at multiple points post-dose up to Week 36
Part 1: Plasma Concentration of ION440
Time frame: Pre-dose and at multiple points post-dose up to Week 36
Part 2: Trough Concentration (Ctrough) of ION440 in Plasma and CSF
Time frame: Up to approximately 192 weeks