Multicentric Trial of the Treatment of Huntington's Disease by Cysteamine (RP103)

Inclusion Criteria:

• Clinically disease-registered for at least one year, leading to consult (abnormalmovements, neuropsychiatric disorders, neuropsychological impairment).

• Unified Huntington's Disease Rating Scale motor ≥ 5

• Total Functional Capacity > 10 (≥ 11)

• Huntington Disease diagnosed with abnormal number of CAG repeats: 38 < nucleotideexpansion (CAG)

• Age between 18 and 65

• Voluntarily Patient Consent

• Patients willing and able to take oral medications, and comply with the specificprocedures of the study

Exclusion Criteria:

• Severe cognitive impairment or neuropsychiatric troubles.

• No drug compliance to previous treatment.

• Patients with contra indication to the realization of imaging studies (includingclaustrophobia ) .

• Patients who have not given their written and informed consent signed .

• No national health insurance affiliation

• Private patients of their liberty by judicial or administrative decision, or patientsunder supervision.

• Pregnant women ( pregnancy test will be carried out systematically for women at risk)or lactating .

• Women who could become pregnant during the study period and with no contraception.

• Patients who have developed hypersensitivity to cysteamine or penicillamine ( againstindication of cysteamine ) .

• Brain Damage intercurrent MRI. Brain morphological abnormalities , other than thosecharacteristic of the disease .

• Disease - associated with neurological repercussions.

• Affection - visceral serious , scalable , involving life-threatening.

• Mental - disorder may disrupt accession to the Protocol , including a history ofspontaneous and / or drug-induced hallucinations history of severe depression thatrequired repeated hospitalizations , history of repeated suicide attempts .

• Participation in progress, or interrupted for less than three months, a therapeuticprotocol of Huntington's disease .

• Patients with a history of surgical interventions to improve the symptoms ofHuntington 's disease such as graft neuron, deep brain stimulation, infusion ofneurotrophic agent