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Recurrent Stroke-Like Episodes in a Patient with Elderly Onset Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS)  

Song, Pamela (Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
Roh, Jee-Hoon (Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
Noh, Hyun-Jin (Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
Seo, Sang-Won (Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
Kim, Kwang-Ki (Department of Neurology, Dongguk University International Hospital, Dongguk University College of Medicine)
Na, Duk-L. (Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
Publication Information
Dementia and Neurocognitive Disorders / v.9, no.2, 2010 , pp. 69-73 More about this Journal
Abstract
Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) uncommonly develops after the age of 40. We describe a patient with MELAS whose first strokelike episode presented at age 65, followed by two more episodes. The brain MR imaging for each stroke-like episode demonstrated hyperintense lesion in diffusion weighted image (DWI), and variable signal intensities in apparent diffusion coefficient (ADC) maps in temporo-parietal and occipital regions. We diagnosed the patient as MELAS with 3243A>G point mutation.
Keywords
MELAS; Elderly; Stroke; MR imaging;
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Times Cited By KSCI : 2  (Citation Analysis)
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1 Scaglia F, Northrop JL. The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (melas) syndrome: a review of treatment options. CNS Drugs 2006; 20: 443-64.   DOI   ScienceOn
2 Iizuka T, Sakai F. Pathogenesis of stroke-like episodes in melas: analysis of neurovascular cellular mechanisms. Curr Neurovasc Res 2005; 2: 29-45.   DOI   ScienceOn
3 James AM, Sheard PW, Wei YH, Murphy MP. Decreased atp synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial trna mutations. Eur J Biochem 1999; 259: 462-9.   DOI   ScienceOn
4 Iizuka T. Pathogenesis and treatment of stroke-like episodes in melas. Rinsho Shinkeigaku 2008; 48: 1006-9.   DOI   ScienceOn
5 Kolb SJ, Costello F, Lee AG, White M, Wong S, Schwartz ED, et al. Distinguishing ischemic stroke from the stroke-like lesions of melas using apparent diffusion coefficient mapping. J Neurol Sci 2003; 216: 11-5.   DOI   ScienceOn
6 Tzoulis C, Bindoff LA. Serial diffusion imaging in a case of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. Stroke 2009; 40: e15-7.   DOI   ScienceOn
7 Pavlakis SG, Phillips PC, DiMauro S, De Vivo DC, Rowland LP. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann Neurol 1984; 16: 481-8.   DOI   ScienceOn
8 Holt IJ, Harding AE, Morgan-Hughes JA. Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies. Nature 1988; 331: 717-9.   DOI   ScienceOn
9 Wallace DC, Singh G, Lott MT, Hodge JA, Schurr TG, Lezza AM, et al. Mitochondrial DNA mutation associated with leber's hereditary optic neuropathy. Science 1988; 242: 1427-30.   DOI
10 Goto Y, Nonaka I, Horai S. A mutation in the trna(leu)(uur) gene associated with the melas subgroup of mitochondrial encephalomyopathies. Nature 1990; 348: 651-3.   DOI   ScienceOn
11 Hirano M, Pavlakis SG. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (melas): current concepts. J Child Neurol 1994; 9: 4-13.   DOI   ScienceOn
12 Goto Y, Horai S, Matsuoka T, Koga Y, Nihei K, Kobayashi M, et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (melas): a correlative study of the clinical features and mitochondrial DNA mutation. Neurology 1992; 42: 545-50.   DOI
13 Lee TY, Hong DK, Lim SR, Heo K, Cho HK. A case report of melas syndrome. J Korean Neurol Assoc 1993; 11: 254-60.
14 Minamoto H, Kawabata K, Okuda B, Shibuya N, Tachibana H, Sugita M, et al. Mitochondrial encephalomyopathy with elderly onset of strokelike episodes. Intern Med 1996; 35: 991-5.   DOI   ScienceOn
15 Dickerson BC, Holtzman D, Grant PE, Tian D. Case records of the massachusetts general hospital. Case 36-2005. A 61-year-old woman with seizure, disturbed gait, and altered mental status. N Engl J Med 2005; 353: 2271-80.   DOI   ScienceOn
16 Yu HJ, Roh SY, Lee KE, Koh SH. A case of melas with maternally transmitted type ii diabetes mellitus and deafness. J Korean Neurol Assoc 2007; 25: 416-8.
17 Lee JY, Jo YS, No SJ, Ki CS, Lee KE, Kim YD. A case of melas with schizophrenia-like psychiatric symptoms. J Korean Neurol Assoc 2005; 23: 830-2.
18 Kim H, Na DL. Paradise-Korean version-the western aphasia battery. Seoul: Paradise welfare foundatrion, 2001.
19 Kang YW, Na DL, Hahn S. A validity study on the korean mini-mental state examination (k-mmse) in dementia patients. J Korean Neurol Assoc 1997; 15: 300-8.
20 Hirano M, Ricci E, Koenigsberger MR, Defendini R, Pavlakis SG, DeVivo DC, et al. Melas: an original case and clinical criteria for diagnodiagnosis. Neuromuscul Disord 1992; 2: 125-35.   DOI   ScienceOn
21 Bosbach S, Kornblum C, Schroder R, Wagner M. Executive and visuospatial deficits in patients with chronic progressive external ophthalmoplegia and kearns-sayre syndrome. Brain 2003; 126: 1231-40.   DOI   ScienceOn
22 Johns DR, Stein AG, Wityk R. Melas syndrome masquerading as herpes simplex encephalitis. Neurology 1993; 43: 2471-3.   DOI   ScienceOn
23 Sharfstein SR, Gordon MF, Libman RB, Malkin ES. Adult-onset melas presenting as herpes encephalitis. Arch Neurol 1999; 56: 241-3.   DOI   ScienceOn