Effects of
            <scp>l</scp>
            -arginine on the acute phase of strokes in three patients with MELAS

Koga, Yasutoshi; Ishibashi, Masatoshi; Ueki, Ikuko; Yatsuga, Shuichi; Fukiyama, Ryo; Akita, Yukihiro; Matsuishi, Toyojiro

doi:10.1212/wnl.58.5.827

Cited by 95 publications

(61 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A MELAS patient treated with L-arginine showed significant improvements in lactate and pyruvate levels, and symptoms of headache, nausea and clinical disability were dramatically improved (7). In contrast, lactate and pyruvate concentrations in CSF remained high even following the succinate therapy in the present case.…”

Section: Discussioncontrasting

confidence: 47%

See 1 more Smart Citation

Successful Treatment with Succinate in a Patient with MELAS

et al. 2004

View full text Add to dashboard Cite

Section: Discussioncontrasting

confidence: 47%

“…Among them, idebenone is a synthetic analogue of coenzyme Q (6). L-arginine is a potent donor of nitric oxide, reducing ischemic damage in brain ischemia (7). Creatine enhances adenosine triphosphatase synthesis and serves as an activator of glycolysis (8).…”

Section: Introductionmentioning

confidence: 99%

Successful Treatment with Succinate in a Patient with MELAS

et al. 2004

View full text Add to dashboard Cite

“…Some have studied the benefits of administering L-arginine, a precursor to the vasodilator NO in patients with MELAS stroke-like episodes Fig. 2 (a, b) (Koga et al 2002(Koga et al , 2005(Koga et al , 2007. It has been found that intravenously administered L-arginine improved acute stroke-like symptoms in MELAS patients (Koga et al 2005).…”

Section: Similarity To Stroke In Melasmentioning

confidence: 99%

Stroke and Stroke-Like Symptoms in Patients with Mutations in the POLG1 Gene

et al. 2011

View full text Add to dashboard Cite

Introduction/Methods Mutations in POLG1, the gene encoding mitochondrial polymerase gamma (Polg), have been associated with a number of well-characterized phenotypes. In this study, we report two cases of patients with biallelic POLG1 mutations and stroke. We also performed a review of the literature and report on all clinical studies of patients with POLG1 mutations in which stroke was described in the phenotype. For each patient, genotype and phenotype are reported.Results Including our two patients, a total of 22 patients have been reported with POLG1 mutations and stroke. The average age of onset of stroke in these patients was 9 years with a range of 1-23 years. In cases where localization was reported, the occipital lobes were the primary location of the infarct. Mutations in the linker-linker or linker-polymerase domains were the most frequent genotype observed. Seizures (16/22) and hepatic dysfunction/failure (8/22) were the most commonly reported symptoms in the stroke cohort.Conclusion This article raises an underrecognized point that patients with POLG1 mutations may suffer a cerebrovascular accident at a young age. The most common location of the infarction is in the occipital lobe. The presentation may be similar to MELAS and can be misdiagnosed as a migrainous stroke.

show abstract

“…In fact, both compounds have already been tried in clinical settings [8,33]. Furthermore, based on the assumption that stroke-like episodes in MELAS are caused by segmental impairment of vasodilatation in intracerebral arteries, Koga et al [10] investigated the effect of l-arginine on the acute phase of strokes in three MELAS patients and reported an improvement in most symptoms. The observation of a positive effect of l-arginine on blood flow in MELAS patients implies that there may indeed be a deficiency of NO production in the endothelial or smooth muscle cells of arterial walls and would, in fact, be consistent with the lower citrulline levels that we observe.…”

Section: Implications For Treatmentmentioning

confidence: 99%

“…More recently, Parfait et al [8] have found hypocitrullinemia in four patients with neurogenic weakness, ataxia, and retinitis pigmentosa (NARP) syndrome, a maternally inherited mitochondrial disorder [9]. Furthermore, a clinical improvement was noted in three patients with another mitochondrial disease, mitochondrial encephalomyopathy, lactic acidosis with stroke-like episodes syndrome (MELAS) [9] after intravenous administration of l-arginine, an amino acid closely related to citrulline [10].…”

Section: Introductionmentioning

confidence: 99%