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

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