MELAS Syndrome Clinical Trial
Official title:
Clinical Long Term Evaluation of Glutamine Supplement in MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes) Syndrome in Order to Prevent Neurological Damage.
The purpose of this study is to assesses the clinical efficacy of oral supplementation with glutamine over 3 years.
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a genetically heterogeneous disorder. The most common mutation is in the mtDNA gene MT-TL1 encoding the mitochondrial tRNALeu (UUR). For understanding the development of seizures in patients with mitochondrial disease, a study has recently emphasized the deficiency of astrocytic glutamine synthetase, creating a disinhibited neuronal network for seizure generation. The investigators propose to evaluate nine patients with mitochondrial DNA mutation and MELAS. Patients will receive oral supplementation with 12-18 g/day of glutamine (adjusted for weight and plasma concentrations). The primary outcome measures modification in clinical scales. ;
Status | Clinical Trial | Phase | |
---|---|---|---|
Completed |
NCT01831934 -
Responses to Influenza Vaccine in Patients With Mitochondrial Disorders (MELAS)
|
Phase 4 | |
Not yet recruiting |
NCT06013397 -
Effectiveness of Ketogenic Diet in MELAS Syndrome
|
N/A | |
Completed |
NCT04165239 -
The KHENERGYZE Study
|
Phase 2 | |
Completed |
NCT01603446 -
L-arginine Therapy on Endothelium-dependent Vasodilation & Mitochondrial Metabolism in MELAS Syndrome
|
Phase 2 | |
Completed |
NCT00887562 -
Study of Idebenone in the Treatment of Mitochondrial Encephalopathy Lactic Acidosis & Stroke-like Episodes
|
Phase 2 | |
Active, not recruiting |
NCT00068913 -
Evaluating the Effectiveness of a Dichloroacetate in MELAS Syndrome
|
Phase 2 | |
Completed |
NCT03888716 -
A Phase Ia/Ib, SAD and MAD Study of of KL1333 in Healthy Subjects and Patients With Primary Mitochondrial Disease
|
Phase 1 | |
Completed |
NCT04948138 -
Glutamine Supplement in MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes) Syndrome
|
N/A | |
Completed |
NCT03056209 -
Safety, Tolerability and Pharmacokinetic Study of KL1333 in Healthy Male Volunteers
|
Phase 1 | |
Completed |
NCT01339494 -
Nitric Oxide Production in MELAS Syndrome
|
Phase 0 | |
Recruiting |
NCT02114554 -
Mitochondrial nt3243 A>G Mutation in Taiwan
|
N/A | |
Recruiting |
NCT05554835 -
Global Registry and Natural History Study for Mitochondrial Disorders
|
||
Completed |
NCT01252979 -
Ketones & Mitochondrial Heteroplasmy
|
Phase 0 | |
Completed |
NCT00004353 -
Study of the Metabolism of Pyruvate and Related Problems in Patients With Lactic Acidemia
|
N/A | |
Recruiting |
NCT03952234 -
L-Citrulline Dose Finding Safety Study in MELAS
|
Phase 1 |