Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks.

Hyonsuk Kim, D. I. Kim, B. I. Lee, E. K. Jeong, C. Choi, J. D. Lee, P. H. Yoon, E. J. Kim, SeHoon Kim, Y. K. Yoon

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Abstract

We report the clinical and MR manifestations of an 18 year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Recurrent status epilepticus caused reversible cytotoxic edema on diffusion-weighted images (DWI). Initial and one month follow-up MR spectroscopy, after seizure control, showed some discrepancies in the ratio of metabolites. N-acetylaspartate (NAA) partially recovered (NAA/creatine (Cr) ratio: 1.27-->1.84). This was because of a normalization of decreased NAA due to cellular dysfunction as a result of status epilepticus. A low ratio of NAA/Cr due to abnormal mitochondria remained in the decreased state. Reversible NAA/Cr ratios in the acute lesion suggested that NAA reflects the neuronal function as well as the level of neuronal structural damage. The altered NAA/Cr ratio better correlated with the abnormal signal intensity area of T2-weighted images (T2WI) and DWI than the lactate (Lac)/Cr ratio. With conservative treatment with anti-epileptics not accompanied by coenzyme Q or sodium dichloroacetate, lactate persistently increased (Lac/Cr ratio: 1.01-->1.21) because of the continued production of lactate in cells with respiratory deficiency, which is the main pathology of MELAS.

Original languageEnglish
Pages (from-to)128-133
Number of pages6
JournalYonsei medical journal
Volume42
Issue number1
DOIs
Publication statusPublished - 2001 Jan 1

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Lactic Acidosis
Brain Diseases
Muscular Diseases
Creatine
Stroke
Lactic Acid
Status Epilepticus
Dichloroacetic Acid
MELAS Syndrome
Sodium Lactate
Ubiquinone
N-acetylaspartate
Edema
Mitochondria
Seizures
Magnetic Resonance Spectroscopy
Pathology

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Kim, Hyonsuk ; Kim, D. I. ; Lee, B. I. ; Jeong, E. K. ; Choi, C. ; Lee, J. D. ; Yoon, P. H. ; Kim, E. J. ; Kim, SeHoon ; Yoon, Y. K. / Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks. In: Yonsei medical journal. 2001 ; Vol. 42, No. 1. pp. 128-133.
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Kim, H, Kim, DI, Lee, BI, Jeong, EK, Choi, C, Lee, JD, Yoon, PH, Kim, EJ, Kim, S & Yoon, YK 2001, 'Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks.', Yonsei medical journal, vol. 42, no. 1, pp. 128-133. https://doi.org/10.3349/ymj.2001.42.1.128

Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks. / Kim, Hyonsuk; Kim, D. I.; Lee, B. I.; Jeong, E. K.; Choi, C.; Lee, J. D.; Yoon, P. H.; Kim, E. J.; Kim, SeHoon; Yoon, Y. K.

In: Yonsei medical journal, Vol. 42, No. 1, 01.01.2001, p. 128-133.

Research output: Contribution to journalArticle

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