Effect of long-term oral treatment with L-arginine and idebenone on the prevention of stroke-like episodes in an adult MELAS patient

• Published in: Revue neurologique Vol. 167; no. 11; pp. 852 - 855
• Main Authors: Lekoubou, A., Kouamé-Assouan, A.-E., Cho, T.-H., Luauté, J., Nighoghossian, N., Derex, L.
• Format: Journal Article
• Language: English
• Published: Paris Elsevier Masson SAS 01-11-2011; Masson
• Subjects: Administration, Oral; Adult; Antioxidants - administration & dosage; Antioxidants - pharmacology; Arginine - administration & dosage; Arginine - pharmacology; Biological and medical sciences; Drug Combinations; Female; Humans; Idebenone; L-arginine; Medical sciences; MELAS syndrome; MELAS Syndrome - complications; MELAS Syndrome - drug therapy; Neurology; Stroke - etiology; Stroke - prevention & control; Stroke-like episodes; Syndrome de MELAS; Time Factors; Treatment Outcome; Ubiquinone - administration & dosage; Ubiquinone - analogs & derivatives; Ubiquinone - pharmacology; Vascular diseases and vascular malformations of the nervous system; Épisodes pseudovasculaires; Idebenone; Stroke-like episodes; MELAS syndrome; Syndrome de MELAS; L-arginine; Épisodes pseudovasculaires; Human; Stroke; Nervous system diseases; Oral administration; Cardiovascular disease; Long term; Cerebral disorder; Vascular disease; Prevention; Treatment; Arginine; Central nervous system disease; Cerebrovascular disease
• ISSN: 0035-3787
• DOI: 10.1016/j.neurol.2011.02.038

Mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a maternally-inherited multisystem disorder. Mitochondrial angiopathy mediated by nitric oxide, a metabolite of L-arginine, is among the proposed pathophysiologic mechanisms of stroke-like episodes (SLEs) in MELAS. There are very few reports on long-term prevention of SLEs with oral L-arginine and idebenone treatment in MELAS adult patients.
A 38-year-old patient with MELAS and SLEs was treated with oral L-arginine and idebenone for 27
months. She remained free of attacks throughout the treatment period except when she stopped her treatment on two occasions during which she had recurrent cerebral metabolic attacks. The patient experienced no side effect of treatment with L-arginine and idebenone.
Our observation suggests long-term safety and potential benefit of oral L-arginine and idebenone in the prevention of recurrence of SLEs in adult MELAS patients.
Le syndrome de MELAS est une maladie multisystémique a été suggéré dans des rapports ponctuels dont le pronostic est sévère. L’effet bénéfique de la L-arginine ou de l’idebenone (analogue du coenzyme Q10) sur la récidive des épisodes pseudovaculaires observée dans cette pathologie a été suggéré dans les rapports ponctuels mais on dispose de peu de données sur la tolérance et l’efficacité à long terme de ce type de traitement.
Il s’agit d’une patiente de 38
ans atteinte du syndrome de MELAS traitée pendant 27
mois par l’association L-arginine et idebenone sans effets secondaires notables et qui a présenté à plusieurs reprises lors de l’interruption temporaire de ce traitement des épisodes d’aggravation neurologique aiguë.
Notre observation suggère une bonne tolérance et un bénéfice potentiel de l’association L-arginine et idebenone dans la prévention des récidives des épisodes pseudovasculaires chez les patients adultes atteints de MELAS.