Korean Circulation Journal

The Korean Society of Cardiology (대한심장학회)
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A Case of Myopathy, Encephalopathy, Lactic Acidosis and Stroke-Like Episodes (MEALS) Syndrome with Intracardiac Thrombus

  • Published : 2013.03.30
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Abstract

Myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a multisystem clinical syndrome manifested by mitochondrial myopathy, encephalopathy, lactic acidosis and recurrent stroke-like episodes. A 27-year-old female with MELAS syndrome presented with cerebral infarction. Echocardiography revealed a thrombus attached to the apex of the hypertrophied left ventricle, with decreased systolic function. The embolism of the intracardiac thrombus might have been the cause of stroke. There should be more consideration given to the increased possibility of intracardiac thrombus formation when a MELAS patient with cardiac involvement is encountered.

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References

  1. Lam CW, Lau CH, Williams JC, Chan YW, Wong LJ. Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy. Eur J Pediatr 1997;156:562-4. https://doi.org/10.1007/s004310050663
  2. 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. https://doi.org/10.1038/348651a0
  3. Chae JH, Hwang H, Lim BC, Cheong HI, Hwang YS, Kim KJ. Clinical features of A3243G mitochondrial tRNA mutation. Brain Dev 2004;26:459-62. https://doi.org/10.1016/j.braindev.2004.01.002
  4. Börner GV, Zeviani M, Tiranti V, et al. Decreased aminoacylation of mutant tRNAs in MELAS but not in MERRF patients. Hum Mol Genet 2000; 9:467-75. https://doi.org/10.1093/hmg/9.4.467
  5. Oguro H, Iijima K, Takahashi K, et al. Successful treatment with succinate in a patient with MELAS. Intern Med 2004;43:427-31. https://doi.org/10.2169/internalmedicine.43.427
  6. Kim HS, Kim DI, Lee BI, et al. Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks. Yonsei Med J 2001;42:128-33. https://doi.org/10.3349/ymj.2001.42.1.128
  7. Hirano M, Pavlakis SG. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS): current concepts. J Child Neurol 1994;9:4-13. https://doi.org/10.1177/088307389400900102
  8. Vydt TC, de Coo RF, Soliman OI, et al. Cardiac involvement in adults with m.3243A>G MELAS gene mutation. Am J Cardiol 2007;99:264-9. https://doi.org/10.1016/j.amjcard.2006.07.089
  9. Okajima Y, Tanabe Y, Takayanagi M, Aotsuka H. A follow up study of myocardial involvement in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). Heart 1998; 80:292-5. https://doi.org/10.1136/hrt.80.3.292
  10. Sproule DM, Kaufmann P, Engelstad K, Starc TJ, Hordof AJ, De Vivo DC. Wolff-Parkinson-White syndrome in Patients With MELAS. Arch Neurol 2007;64:1625-7. https://doi.org/10.1001/archneur.64.11.1625

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  1. Mitochondrial DNA mutation “m.3243A>G”-Heterogeneous clinical picture for cardiologists (“m.3243A>G”: A phenotypic chameleon) vol.13, pp.5, 2018, https://doi.org/10.1111/chd.12634