RecruitingPhase 2Phase 3NCT05004129

Safety and Efficacy of Tideglusib in Congenital or Childhood Onset Myotonic Dystrophy

An Open-Label Study to Evaluate the Long-Term Safety and Efficacy of Tideglusib for the Treatment of Congenital or Childhood Onset DM1 (REACH CDM X)


Sponsor

AMO Pharma Limited

Enrollment

76 participants

Start Date

Aug 23, 2021

Study Type

INTERVENTIONAL

Conditions

Summary

This is an open-label phase 2/3 study for individuals with Congenital Myotonic Dystrophy (Congenital DM1) who participated in the preceding AMO-02-MD-2-003 study or individuals with either Congenital or Childhood Onset DM1 who are treatment naïve.


Eligibility

Min Age: 6 YearsMax Age: 45 Years

Inclusion Criteria13

  • Subjects who do not enter this study directly from completing the AMO-02-MD-2-003 study (i.e. subjects who did not complete AMO-02-MD-2-003, subjects who completed AMO-02-MD-2-003 but did not directly rollover or subjects who are re-entering AMO-02-MD-2-004), will not be considered eligible for the study without meeting all of the criteria below:
  • Subjects under study must be individuals with a diagnosis of Congenital or Childhood Onset DM1.
  • Diagnosis must be genetically confirmed
  • Subjects must be male or female aged ≥6 years to ≤45 years at Screening
  • Subjects must have a Clinical Global Impression - Severity (CGI-S) score of 3 or greater at Screening (V-1)
  • Written, voluntary informed consent must be obtained before any study related procedures are conducted. Where a parent or legally authorized representative (LAR) provides consent, there must also be assent from the subject (as required by local regulations)
  • Subject's caregiver must be willing and able to support participation for duration of study
  • Subject must be willing and able to comply with the required food intake restrictions as outlined per protocol
  • Subjects entering directly from completing the antecedent AMO-02-MD-2-003 study will not be considered eligible for the study without meeting all of the criteria below:
  • Subjects who have completed the antecedent AMO-02-MD-2-003 study through V11
  • Written, voluntary informed consent must be obtained before any study related procedures are conducted. Where a parent or LAR provides consent, there must also be assent from the subject (as required by local regulations)
  • Subject's caregiver must be willing and able to support participation for duration of study
  • Subject must be willing and able to comply with the required food intake restrictions as outlined per protocol

Exclusion Criteria7

  • Body mass index (BMI) less than 13.5 kg/m² or greater than 40 kg/m²
  • New or change in medications/therapies within 4 weeks prior to Eligibility/Baseline Visit
  • Use within 4 weeks prior to Eligibility/Baseline Visit of strong CYP3A4 inhibitors (eg.clarithromycin, telithromycin, ketoconazole, itraconazole, posaconazole, nefazodone, idinavir and ritonavir)
  • Concurrent use of drugs metabolized by CYP3A4 with a narrow therapeutic window (e.g. warfarin and digitoxin)
  • Current enrollment in a clinical trial of an investigational drug or enrollment in a clinical trial of an investigational drug in the last 6 months other than the AMO-02- MD-2-003 study
  • Existing or historical medical conditions or complications (eg. neurological, cardiovascular, renal, hepatic, gastrointestinal, endocrine or respiratory disease) that may impact the interpretability of the study results
  • Hypersensitivity to tideglusib or any components of its formulation including allergy to strawberry

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Interventions

DRUGTideglusib

Tideglusib dosing will be weight-adjusted at 400 mg, 600 mg, or 1000 mg dose levels, or weight banded fixed doses of 400 mg, 600 mg, 800 mg or 1000 mg, with each subject starting at a weight-adjusted 400 mg dose level for 2 weeks, then up titrating to a weight-adjusted 600 mg dose level for the next 2 weeks.


Locations(14)

Arkansas Children's Hospital

Little Rock, Arkansas, United States

University of California, Los Angeles (UCLA)

Los Angeles, California, United States

Stanford University

Palo Alto, California, United States

Lurie's Children's Hospital

Chicago, Illinois, United States

University of Iowa Hospitals and Clinics

Iowa City, Iowa, United States

University of Rochester - Medical Center

Rochester, New York, United States

University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, United States

University of Utah Clinical Neurosciences Center

Salt Lake City, Utah, United States

Children's Hospital of The King's Daughters

Norfolk, Virginia, United States

Virginia Commonwealth University-Department of Neurology - Muscular Dystrophy Translational Research Program

Richmond, Virginia, United States

The Bright Alliance

Randwick, New South Wales, Australia

Children's Hospital London Health Sciences Centre (LHSC)

London, Ontario, Canada

Children's Hospital of Eastern Ontario

Ottawa, Ontario, Canada

New Zealand Clinical Research (NZCR)

Auckland, New Zealand

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NCT05004129


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