Evolution of Albumin on AOA1 Patients Supplemented With Coenzyme Q10
We propose a study on Ataxia with oculomotor apraxia type 1 (AOA1) in which Coenzyme Q10 (CoQ10) deficit has been observed. Main objectives of the study are : To monitor evolution of albumin in patients affected with AOA1 while supplemented with CoQ10 ; To measure with clinical scales and biological markers efficacy of supplementation on disease evolution. AOA1 is characterised by Hypoalbuminemia. Disease duration is negatively correlated with albumin level. This study aims to understand mechanisms of the disease and our hypothesis is that correction or stabilization of albumin level with CoQ10 supplementation could impact disease evolution. The study is planned from 1 to 2 years supplementation. The CoQ10 is classified as a food supplement and has already been tested in other neurological conditions.
• Diagnosis of ataxia with oculomotor apraxia type I (AOA1) confirmed by genetic molecular analysis
• Age ≥ 18 years
• Efficient contraception for women of childbearing potential (with pregnancy test during each visit)
• Signature of the written informed consent form
• Presence of a support person (for patient with cognitive disorders)