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

 Hyun Sook Kim  ;  Dong Ik Kim  ;  Byung In Lee  ;  Eun Kee Jeong  ;  Chulhee Choi  ;  Jong Doo Lee  ;  Pyeong Ho Yoon  ;  Eun Ju Kim  ;  Seung Hyung Kim  ;  Yong Kyu Yoon 
 Yonsei Medical Journal, Vol.42(1) : 128-133, 2001 
Journal Title
 Yonsei Medical Journal 
Issue Date
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.
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Nuclear Medicine (핵의학교실)
Yonsei Authors
김동익(Kim, Dong Ik)
김현숙(Kim, Hyun Sook)
윤평호(Yoon, Pyeong Ho)
이병인(Lee, Byung In)
이종두(Lee, Jong Doo)
정은기(Jeong, Eun Kee)
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