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Phase 2 N=80 Randomized Quadruple-blind Treatment

Phase II, Randomized, Placebo-controlled Trial in Patients With Charcot-marie-tooth Disease Type 1A

Charcot-Marie-Tooth Disease · Hereditary Neuropathy With Liability to Pressure Palsies · Genetic Disorders

Enrolled (actual)
80
Serious AEs
2.5%
Results posted
Feb 2017
Primary outcome: Primary: Safety and Tolerability of PXT3003 — 21; 21; 19; 19 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
PXT3003 Low dose (Drug); PXT3003 Intermediate Dose (Drug); PXT3003 High Dose (Drug); Placebo (Other)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Pharnext S.C.A.
Primary completion
Oct 2011

Outcome Measures

OutcomeResultp-value
PRIMARY
Safety and Tolerability of PXT3003
21; 21; 19; 19
SECONDARY
To Obtain Preliminary Data on the Efficacy of PXT3003 on Clinical Scores and Functional Tests
SECONDARY
To Assess the Pharmacodynamic Effect of PXT3003 on PMP22 mRNA Levels and Intra-epidermal Axon Density in Cutaneous Biopsy
SECONDARY
To Assess the Pharmacodynamic Effect of PXT3003 on Selected Neurophysiological Parameters
SECONDARY
To Assess the Pharmacodynamic Effect of PXT3003 on a Series of Biochemical Biomarkers
SECONDARY
To Assess the Plasma Concentrations of PXT3003

Summary

The present trial is a randomized, placebo-controlled study evaluating 3 different doses of PXT3003 in patients with CMT1A disease.

Eligibility Criteria

Inclusion Criteria

  • DNA proven CMT1A
  • Muscle weakness in at least foot dorsiflexion (clinical assessment)
  • Age between 18 and 65 years
  • Male or non pregnant, non breastfeeding female
  • CMT neuropathy score at screening ≤ 20
  • Agrees to perform electrorophysiological studies and two cutaneous biopsies for determination of PMP22 expression and histology
  • Providing signed written informed consent to participate in the study and willing and able to comply with all study procedures and scheduled visits

Exclusion Criteria

  • Patients with another neurological disease
  • Patients using unauthorized concomitant treatments, ascorbic acid, opioids, levothyroxine and potentially neurotoxic drugs. Patients who can/agree to stop these medications 4 weeks before randomization can be included
  • Patients who have participated in another trial of investigational drug within the past 30 days
  • Concomitant major systemic disease
  • Clinically significant history of unstable medical illness over the last 30 days (unstable angina…)
  • History of significant hematologic, kidney, liver disease, or insulin-dependent diabetes
  • Clinically significant abnormalities on the prestudy laboratory evaluation, physical evaluation, electrocardiogram (ECG)
  • ASAT/ALAT levels above the upper limit of normal (ULN). However, patients with an isolated elevation of either ASAT or ALAT (<1.5 ULN) can be included at investigators" discretion if the remaining liver function tests are normal and if ASAT or ALAT value is stable at 2 distinct evaluations in the month prior to inclusion
  • Serum creatinine levels above the upper limit of normal
  • Limited mental capacity or psychiatric disease rendering the subject unable to provide written informed consent or comply with evaluation procedures
  • History of recent alcohol or drug abuse or non-adherence with treatment or other experimental protocols
  • Female of childbearing potential (apart of patient using adequate contraceptive measures), pregnant or breast feeding
  • Suspected inability to complete the study follow-up (foreign workers, transient visitors, tourists or any others for whom follow-up evaluation is not assured)
  • Limb surgery in the six months before randomization or planned before completion of the trial
  • Known hypersensitivity to any of the individual components of PXT3003
  • Porphyria
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01401257). 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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