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Therapeutic regimen of L-arginine for MELAS: 9-year, prospective, multicenter, clinical research.
- Source :
-
Journal of neurology [J Neurol] 2018 Dec; Vol. 265 (12), pp. 2861-2874. Date of Electronic Publication: 2018 Sep 29. - Publication Year :
- 2018
-
Abstract
- Objective: To examine the efficacy and safety of the therapeutic regimen using oral and intravenous L-arginine for pediatric and adult patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).<br />Methods: In the presence and absence of an ictus of stroke-like episodes within 6 h prior to efficacy assessment, we correspondingly conducted the systematic administration of oral and intravenous L-arginine to 15 and 10 patients with MELAS in two, 2-year, prospective, multicenter clinical trials at 10 medical institutions in Japan. Subsequently, patients were followed up for 7 years. The primary endpoint in the clinical trial of oral L-arginine was the MELAS scale, while that for intravenous L-arginine was the improvement rates of headache and nausea/vomiting at 2 h after completion of the initial intravenous administration. The relationships between the ictuses of stroke-like episodes and plasma arginine concentrations were examined.<br />Results: Oral L-arginine extended the interictal phase (p = 0.0625) and decreased the incidence and severity of ictuses. Intravenous L-arginine improved the rates of four major symptoms-headache, nausea/vomiting, impaired consciousness, and visual disturbance. The maximal plasma arginine concentration was 167 μmol/L when an ictus developed. Neither death nor bedriddenness occurred during the 2-year clinical trials, and the latter did not develop during the 7-year follow-up despite the progressively neurodegenerative and eventually life-threatening nature of MELAS. No treatment-related adverse events occurred, and the formulations of L-arginine were well tolerated.<br />Conclusions: The systematic administration of oral and intravenous L-arginine may be therapeutically beneficial and clinically useful for patients with MELAS.
- Subjects :
- Administration, Intravenous
Administration, Oral
Adolescent
Adult
Arginine adverse effects
Arginine blood
Central Nervous System Agents adverse effects
Central Nervous System Agents blood
Female
Follow-Up Studies
Humans
MELAS Syndrome blood
Male
Prospective Studies
Treatment Outcome
Young Adult
Arginine administration & dosage
Central Nervous System Agents administration & dosage
MELAS Syndrome drug therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 265
- Issue :
- 12
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 30269300
- Full Text :
- https://doi.org/10.1007/s00415-018-9057-7