[A case of stroke-like episode of MELAS of which progressive spread would be prevented by edaravone].
A thirty-two-year-old woman who had been diagnosed MELAS with 3243A > G mutation presented headache, nausea, decreased bilateral visual acuity, and topographical disturbance on January 1 in 2002. Although brain CT showed no fresh lesion, recurrence of stroke-like episode was considered. Immediately, she was treated with ubiquinone (210 mg/day, p.o.) and tocopherol nicotinate (300 mg/day, p.o.). She became confused on the fifth day. Diffusion weighted- and T2 weighted-MRI revealed appearance of hyperintense lesion at the right occipital lobe. We started edaravone infusion (30 mg, twice a day, div.) for two weeks with informed consent from her family. On 13th day her consciousness was improved. Edema and signal intensity of the lesion were decreased on MRI with minimal spread to the parietal lobe. She discharged on the 30th day with marked visual field loss, hemispatial neglect, and topographical amnesia. MRI after four months showed remarkable atrophy of the right occipital region. In our department, five stroke-like episodes including this case were treated with ubuiquinone and tocopherol nicotinate. This regimen was effective in prevention of progressive spread of lesions only in two episodes. Edaravone is radical scavenger used in acute cerebral infarction. Progressive spread into the neighboring regions is one of characteristics of MELAS, although its precise mechanisms are not well known. Oxidative stress induced by released free radicals through mitochondrial dysfunction might be one of factors and edaravone would make an effect through blockage of the free radicals. Edaravone could not rescue neurons in the initial lesion. Although more numbers of cases are needed to establish the effect of edaravone on MELAS, it could minimize the neurological deficits after stroke-like episode of MELAS.
Maeda
Kengo
K
Division of Neurology, Department of Medicine, Shiga University of Medical Science.
Tatsumi
Mariko
M
Tahara
Masayuki
M
Murata
Yoshiko
Y
Kawai
Hiromichi
H
Yasuda
Hitoshi
H
D000328:Adult; D000983:Antipyrine / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D001921:Brain / Q000473:pathology*; D018450:Disease Progression; D000077553:Edaravone; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use*; D006801:Humans; D017241:MELAS Syndrome / Q000188:drug therapy* / Q000473:pathology*; D008279:Magnetic Resonance Imaging
D016166:Free Radical Scavengers; D000077553:Edaravone; D000983:Antipyrine