Assessing Long Term Safety and Tolerability of PXT3003 in Patients With Charcot-Marie-Tooth Disease Type 1A

Inclusion Criteria after September 18th 2017:

• Patients previously randomized to study CLN-PXT3003-02 under placebo and dose 1 andhaving completed 15 months of double-blind treatment in that study, including allprocedures required at the Study Termination visit (V6) or
• Patients previously randomized to the initial study CLN-PXT3003-02 under dose 2,prematurely discontinued following sponsor decision, and having performed allprocedures required at the Study Termination visit (V6)
• Patients whose V6 was performed within 4 weeks before entering the extension study orif not done must have a new baseline visit (VB)
• Female patients must agree to continue using an approved method of birth controlthroughout the extension study
• Patients must sign a written informed consent, specific to the extension study, inorder to participate in this study. In case of minor children aged 16 to 18 years,both parent' and children's consents should be collected Inclusion Criteria until September 18th 2017:
• Patients must have completed 15 months of double-blind treatment in the primary studyCLN-PXT3003-02, including all procedures required at the Study Termination visit (V6)
• Female patients must agree to continue using an approved method of birth controlthroughout the extension study
• Patients must sign a written informed consent, specific to the extension study, inorder to participate in this study. In case of minor children aged 16 to 18 years,both parent' and children's consents should be collected

Exclusion Criteria:

• Any clinically significant change in health status that, in the opinion of theInvestigator, would prevent the subject from participating in this study orsuccessfully completing this study
• Any unauthorized concomitant treatments, as study CLN-PXT3003-02 (e.g. including butnot limited to baclofen, naltrexone,sorbitol (pharmaceutical form), opioids,levothyroxin, and potentially neurotoxic drugs such as amiodarone, chloroquine, cancerdrugs susceptible to induce peripheral neuropathy)