Restricted accessCase reportFirst published online 2022-10
Response to the Letter to the Editor “The apparent beneficial effect of L-arginine for stroke-like lesions can be accidental” by Josef Finsterer and Sinda Zarrouk
ItoHMoriKKagamiS. Neuroimaging of stroke-like episodes in MELAS. Brain Dev. 2011;33:283-288. doi:10.1016/j.braindev.2010.06.010
2.
IizukaTSakaiFKanSSuzukiN. Slowly progressive spread of stroke-like lesions in MELAS syndrome. Neurology. 2003;61:1238-1244. doi:10.1212/01.wnl.0000091888.26232.fe
3.
IizukaTSakaiFSuzukiN, et al.Neuronal hyperexcitability in strokelike episodes of MELAS syndrome. Neurology. 2002;59:816-824. doi:10.1212/wnl.59.6.816
4.
MiyamotoAOkiJTakahashiSItohJKusonkiYChoK. Serial imaging in MELAS. Neuroradiology. 1997;39:427-430. doi:10.1007/s002340050438
5.
NishiokaJAkitaYYatsugaS, et al.Inappropriate intracranial hemodynamics in the natural course of MELAS. Brain Dev. 2008;30:100-105. doi:10.1016/j.braindev.2007.06.008
6.
OoiwaYUematsuYTeradaT, et al.Cerebral blood flow in Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. Stroke. 1993;24:304-309.
7.
IizukaTSakaiF. Pathogenesis of stroke-like episodes in MELAS: analysis of neurovascular cellular mechanisms. Curr Neurovasc Res. 2005;2:29-45. doi:10.2174/1567202052773544
8.
KogaYPovalkoNNishiokaJKatayamaKYatsugaSMatsuishiT. Molecular pathology of MELAS and L-arginine effects. Biochim Biophys Acta. 2012;1820:608-614. doi:10.1016/j.bbagen
9.
GilchristJMSikiricaMStopaEShanskeS. Evidence for involvement of neurons as well as cerebral vasculature in strokelike episodes. Stroke. 1996;27:1420-1423. doi:10.1161/01.str.27.8.1420
10.
MizukamiKSasakiMSuzukiT, et al.Central nervous systemchanges in mitochondrial encephalomyopathy: light and electron microscopic study. Acta Neuropathol. 1992;83:449-452. doi:10.1007/BF00713541
11.
ZhangZQNiuSTLiangXHJianFWangY. Vascular involvement in the pathogenesis of mitochondrial encephalomyopathies. Neurol Res. 2010;32:403-408. doi:10.1179/016164110X12670144526345
12.
GropenTJProhovikITatemichiTKHiranoM. Cerebral hyperemia in MELAS. Stroke. 1994;25:1873-1876. doi:10.1161/01.str.25.9.1873
13.
IizukaTSakaiFIdeTMiyakawaSSatoMYoshiiS. Regional cerebral blood flow and cerebrovascular reactivity during chronic stage of stroke-like episodes in MELAS—implication of neurovascular cellular mechanism. J Neurol Sci. 2007;257:126-138. doi:10.1016/j.jns.2007.01.040
14.
KodakaRItagakiYMatsumotoMNagaiTOkadaS. A transcranial doppler ultrasonography study of cerebrovascular CO2 reactivity in mitochondrial encephalomyopathy. Stroke. 1996;27:1350-1353. doi:10.1161/01.str.27.8.1350
15.
MolnarMJValikovicsAMolnarS, et al.Cerebral blood flow and glucose metabolism in mitochondrial disorders. Neurology. 2000;55:544-548. doi:10.1212/wnl
16.
NariaiTOhnoKAkimotoH, et al.Cerebral blood flow, vascular response and metabolism in patients with MELAS syndrome—xenon CT and PET study. Keio J Med. 2000;49(suppl 1):A68-A70.
17.
RodanLHPoublancJFisherJA, et al.Cerebral hyperperfusion and decreased cerebrovascular reactivity correlate with neurologic disease severity in MELAS. Mitochondrion. 2015;22:66-74. doi:10.1016/j.mito.2015.03.002
18.
KogaYPovalkoNNishiokaJKatayamaKKakimotoNMatsuishiT. MELAS and L-arginine therapy: Pathophysiology of stroke-like episodes. Ann N Y Acad Sci. 2010;1201:104-110. doi:10.1111/j.1749-6632.2010.05624.x
19.
MorrisSMJr.Arginine metabolism: boundaries of our knowledge. J Nutr. 2007;137:1602S-1609S. doi:10.1093/jn/137.6.1602S
20.
RodanLHPoublancJFisherJASobczykOMikulisDJTeinI. L-arginine effects on cerebrovascular reactivity, perfusion and neurovascular coupling in MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes) syndrome. PLoS One. 2020;15(9):e0238224. doi:10.1371/journal.pone.0238224
21.
SiddiqIWidjajaETeinI. Clinical and radiology reversal of stroke-like episodes in MELAS with high-dose L-arginine. Neurology. 2015;85:197-198. doi:10.1212/WNL.0000000000001726
22.
TeinIDiMauroSXieZWDe VivoDC. Valproic acid impairs carnitine uptake in cultured human skin fibroblasts. An in vitro model for the pathogenesis of valproic acid-associated carnitine deficiency. Pediatr Res. 1993;34(3):281-287. doi:10.1203/00006450-199309000-00008
23.
TeinIXieZW. Reversal of valproic acid-associated impairment of carnitine uptake in cultured human skin fibroblasts. Biochem Biophys Res Commun. 1994;204:753-758. doi:10.1006/bbrc.1994.2523
24.
De VivoDCBohanTPCoulterDL, et al.L-carnitine supplementation in childhood epilepsy: Current perspectives. Epilepsia. 1998;39:1216-1225. doi:10.1111/j.1528-1157.1998.tb01315
25.
TeinI. Role of carnitine and fatty acid oxidation and its defects in infantile epilepsy. J Child Neurol. 2002;17:3S57-3S82.
26.
SalsaaMPereiraBLiuJYuWJadhavSHuttemannMGreenbergML. Valproate inhibitis mitochondrial bioenergetics and increases glycolysis in Saccharomyces cerevisiae. Sci Rep. 2020;10:11785. doi:10.1038/s41598-020-68725-5
27.
LuderASParksJKFrermanFParkerWDJr. Inactivation of beef brain alpha-ketoglutarate dehydrogenase complex by valproic acid and valproic acid metabolites. Possible mechanism of anticonvulsant and toxic actions. J Clin Invest. 1990;86:1574-1581. doi:10.1172/JCI114877
28.
SantosNAGMedinaWSGMartinsNMMingattoFECurtiCSantosAC. Aromatic antiepileptic drugs and mitochondrial toxicity: effects on mitochondria isolated from rat liver. Toxicol in Vitro. 2008;22:1143-1152. doi:10.1016/j.tiv.2008.03.004
