Long-Term Safety and Tolerability of Idebenone in Friedreich's Ataxia Patients (MICONOS Extension)
Primary Purpose
Freidreich's Ataxia
Status
Completed
Phase
Phase 3
Locations
International
Study Type
Interventional
Intervention
idebenone
Sponsored by
About this trial
This is an interventional treatment trial for Freidreich's Ataxia focused on measuring FRDA, idebenone, FRDA disease
Eligibility Criteria
Inclusion Criteria:
- Completion of 52 weeks in study SNT-III-001
- Body weight ≥ 25 kg
- Negative urine pregnancy test
- Eligibility to participate in the present extension study as confirmed by investigator
Exclusion Criteria:
- Safety or tolerability issues arising during the course of SNT-III-001 which in the opinion of the investigator preclude further treatment with idebenone
- Clinically significant abnormalities of haematology or biochemistry including, but not limited to, elevations greater than 1.5 times the upper limit of SGOT, SGPT or creatinine
- Parallel participation in another clinical drug trial
- Pregnancy or breast-feeding
- Abuse of drugs or alcohol
Sites / Locations
- Universitätsklinik Innsbruck
- Hôpital Erasme - Univeristé Libre de Bruxelles
- Hôpital de la Salpétrière - INSERM U679, Neurologie et Thérapeutique expérimentale
- HELIOS Klinikum Berlin
- Neurologische Universitätsklinik und Ploklinik - Universitätsklimikum Bonn
- Klinik II, Neuropädiatrie und Muskelerkrankungen - Universitätsklinik Freiburg
- Zentrum für Neurologische Medizin
- UKE Hamburg Neuropädiatrie - Zentrum für Frauen, Kinder und Jugendmedizin
- Neurologische Klinik - Klinikum Grosshadern
- Neurologische Universitätsklinik und Poliklinik
- University medical Center Groningen
Arms of the Study
Arm 1
Arm Type
Experimental
Arm Label
Idebenone
Arm Description
1350 mg/day or 2250 mg/day for patients weighing ≤45 kg or >45 kg, respectively.In case of poor tolerability, dose reduction to 450 mg/day or 900 mg/day, respectively, were allowed.
Outcomes
Primary Outcome Measures
Measures of Safety and Tolerability: Nature and Frequency of Adverse Events (AEs)
Global Overvbiew of accurance of AEs-Safety population. The Safety population included all subjects who received at least one dose of the study medication.
Absolute Change in The International Cooperative Ataxia Rating Scale (ICARS)
The International Cooperative Ataxia Rating Scale (ICARS) is a commonly used evaluation and is composed of four clinical sub-scores involving the following: posture and gait, limb coordination, speech and oculomotor function.The ICARS score is the total sum of the sub scores and ranges from 0 to 100, with 100 indicative of the most severely affected outcome.
Secondary Outcome Measures
Full Information
NCT ID
NCT00993967
First Posted
October 13, 2009
Last Updated
August 21, 2017
Sponsor
Santhera Pharmaceuticals
1. Study Identification
Unique Protocol Identification Number
NCT00993967
Brief Title
Long-Term Safety and Tolerability of Idebenone in Friedreich's Ataxia Patients (MICONOS Extension)
Official Title
A Phase III Open-Label, Single-Group, Extension Study to Obtain Long-Term Safety and Tolerability Data of Idebenone in the Treatment of Friedreich's Ataxia Patients.
Study Type
Interventional
2. Study Status
Record Verification Date
August 2017
Overall Recruitment Status
Completed
Study Start Date
June 2007 (undefined)
Primary Completion Date
June 2012 (Actual)
Study Completion Date
June 2012 (Actual)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Santhera Pharmaceuticals
4. Oversight
Data Monitoring Committee
Yes
5. Study Description
Brief Summary
This is an Extension study of the MICONOS main randomised placebo-controlled trial (NCT00905268), and open to those patients completing the main study. The scientific aim of this extension study is to monitor safety and tolerability of idebenone over two years in patients with Friedreich's Ataxia.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Freidreich's Ataxia
Keywords
FRDA, idebenone, FRDA disease
7. Study Design
Primary Purpose
Treatment
Study Phase
Phase 3
Interventional Study Model
Single Group Assignment
Masking
None (Open Label)
Allocation
N/A
Enrollment
200 (Actual)
8. Arms, Groups, and Interventions
Arm Title
Idebenone
Arm Type
Experimental
Arm Description
1350 mg/day or 2250 mg/day for patients weighing ≤45 kg or >45 kg, respectively.In case of poor tolerability, dose reduction to 450 mg/day or 900 mg/day, respectively, were allowed.
Intervention Type
Drug
Intervention Name(s)
idebenone
Intervention Description
Idebenone 1350 mg/d, patients < or equal 45 kg Idebenone 2250 mg/d, patients > 45 kg
Primary Outcome Measure Information:
Title
Measures of Safety and Tolerability: Nature and Frequency of Adverse Events (AEs)
Description
Global Overvbiew of accurance of AEs-Safety population. The Safety population included all subjects who received at least one dose of the study medication.
Time Frame
overall study, up to 24 months
Title
Absolute Change in The International Cooperative Ataxia Rating Scale (ICARS)
Description
The International Cooperative Ataxia Rating Scale (ICARS) is a commonly used evaluation and is composed of four clinical sub-scores involving the following: posture and gait, limb coordination, speech and oculomotor function.The ICARS score is the total sum of the sub scores and ranges from 0 to 100, with 100 indicative of the most severely affected outcome.
Time Frame
Baseline, Month 12 and month 24
Other Pre-specified Outcome Measures:
Title
Measures of Safety and Tolerability: Physical Examinations and Vital Signs
Description
Assessment of the head, eyes, ears, nose, throat, heart, chest, lungs, abdomen, extremities, peripheral pulses, skin and any other physical conditions of note.
Time Frame
Month 1, 3, 6, 12, 18 and 24
Title
Measures of Safety and Tolerability: Electrocardiograms (ECGs)
Description
12-lead ECG recordings were performed at every visit. Each ECG was measured using 3 complexes: PR interval in lead II or V2, QRS and QT intervals and heart rate in lead II, corrected QT intervals QTcB and QTcF.
Time Frame
Month 1, 3, 6, 12, 18 and 24
Title
Measures of Safety and Tolerability: Haematological and Biochemical Laboratory Parameters
Description
Safety haematological analysis were done at every visit. Analyses included red blood cell count, haemoglobin, haematocrit, red cell indices, white blood cell count including differential, platelet count Safety biochemistry were done at every visit. Analyses included sodium, potassium, chloride, bicarbonate, urea, creatinine, calcium, inorganic phosphate, glucose, total bilirubin, total protein, albumin, aspartate amiotransferase (AST), alanine aminotransferase (ALT), alkaline phosphotase, Gamma GT, creatine kinase (CK)^, cholesterol, triglycerides, uric acid.
Time Frame
Month 1, 3, 6, 12, 18 and 24
10. Eligibility
Sex
All
Minimum Age & Unit of Time
9 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
Inclusion Criteria:
Completion of 52 weeks in study SNT-III-001
Body weight ≥ 25 kg
Negative urine pregnancy test
Eligibility to participate in the present extension study as confirmed by investigator
Exclusion Criteria:
Safety or tolerability issues arising during the course of SNT-III-001 which in the opinion of the investigator preclude further treatment with idebenone
Clinically significant abnormalities of haematology or biochemistry including, but not limited to, elevations greater than 1.5 times the upper limit of SGOT, SGPT or creatinine
Parallel participation in another clinical drug trial
Pregnancy or breast-feeding
Abuse of drugs or alcohol
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Nick Wood, Professor
Organizational Affiliation
Dept of Molecular Neuroscience, Institute of Neurology. The National Hospital, University College London
Official's Role
Principal Investigator
Facility Information:
Facility Name
Universitätsklinik Innsbruck
City
Innsbruck
Country
Austria
Facility Name
Hôpital Erasme - Univeristé Libre de Bruxelles
City
Bruxelles
ZIP/Postal Code
1070
Country
Belgium
Facility Name
Hôpital de la Salpétrière - INSERM U679, Neurologie et Thérapeutique expérimentale
City
Paris
ZIP/Postal Code
75651
Country
France
Facility Name
HELIOS Klinikum Berlin
City
Berlin
ZIP/Postal Code
13125
Country
Germany
Facility Name
Neurologische Universitätsklinik und Ploklinik - Universitätsklimikum Bonn
City
Bonn
ZIP/Postal Code
53105
Country
Germany
Facility Name
Klinik II, Neuropädiatrie und Muskelerkrankungen - Universitätsklinik Freiburg
City
Freiburg
ZIP/Postal Code
79106
Country
Germany
Facility Name
Zentrum für Neurologische Medizin
City
Göttingen
ZIP/Postal Code
37073
Country
Germany
Facility Name
UKE Hamburg Neuropädiatrie - Zentrum für Frauen, Kinder und Jugendmedizin
City
Hamburg
ZIP/Postal Code
20246
Country
Germany
Facility Name
Neurologische Klinik - Klinikum Grosshadern
City
München
ZIP/Postal Code
81377
Country
Germany
Facility Name
Neurologische Universitätsklinik und Poliklinik
City
Tübingen
ZIP/Postal Code
72076
Country
Germany
Facility Name
University medical Center Groningen
City
Groningen
ZIP/Postal Code
9700 RB
Country
Netherlands
12. IPD Sharing Statement
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Long-Term Safety and Tolerability of Idebenone in Friedreich's Ataxia Patients (MICONOS Extension)
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