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Phase 3 N=232 Randomized Quadruple-blind Treatment

A Study of Efficacy, Safety and Tolerability of Idebenone in the Treatment of Friedreich's Ataxia (FRDA) Patients

Friedreich's Ataxia

Enrolled (actual)
232
Serious AEs
11.2%
Results posted
Jun 2016
Primary outcome: Primary: Absolute Change in International Cooperative Ataxia Rating Scale (ICARS) Scores From Baseline Assessment to Week 52 — 1.6; 1.7; 1.2; 1.1 units on a scale

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
idebenone (Drug); Placebo (Drug)
Age
Pediatric, Adult, Older Adult · 8+ yrs
Sex
All
Sponsor
Santhera Pharmaceuticals
Primary completion
Jan 2010

Outcome Measures

OutcomeResultp-value
PRIMARY
Absolute Change in International Cooperative Ataxia Rating Scale (ICARS) Scores From Baseline Assessment to Week 52
1.6; 1.7; 1.2; 1.1
SECONDARY
Absolute Change in Friedreich's Ataxia Rating Scale (FARS) Scores From Baseline Assessment to Week 52
0.9; 1.2; 1.4; 0.9
SECONDARY
Proportion of Patients Improving (Responding) on ICARS by a Clinically Relevant Margin
18.2; 23.6; 23.7; 31
SECONDARY
Proportion of Patients Improving on Left Ventricular Peak Systolic Strain Rate or Showing a Reduction in Left Ventricular Mass Index (LVMI) With no Worsening in Strain Rate
50; 51.4; 30.3; 44.1
SECONDARY
Change in Peak Systolic Strain Rate From Baseline to Week 52
0.007; -0.004; 0.085; 0.024
SECONDARY
Change in Peak Workload From Baseline to Week 52
1.15; -7.41; -6.91; -1.54

Summary

The purpose of this trial is to study the efficacy, safety and tolerability of idebenone in 12 months of treatment in children and adults with Friedreich's Ataxia. This is a randomised placebo-controlled double-blind trial conducted in Europe. Efficacy outcomes include measures of neurological impairment and function, and measures of the heart.

Eligibility Criteria

Inclusion Criteria

  • Documented diagnosis of FRDA with confirmed FRDA mutations
  • Patients 8 years of age or older at baseline
  • Patients with body weight ≥ 25kg
  • Patients who in the opinion of the investigator are able to comply with the requirements of the study, including swallowing the medication
  • Negative urine pregnancy test at screening and at baseline (women of childbearing potential)

Exclusion Criteria

  • Treatment with idebenone or Coenzyme Q10 within the past 1 month
  • Pregnancy and/or breast-feeding
  • Clinically significant abnormalities of clinical haematology or biochemistry including, but not limited to, elevations greater than 1.5 times the upper limit of normal of SGOT, SGPT, or creatinine
  • Past or present history of abuse of drugs or alcohol
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00905268). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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