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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
Issue Date
Adolescent ; Aspartic Acid/analogs & derivatives* ; Aspartic Acid/metabolism ; Brain/metabolism ; Creatine/metabolism ; Diffusion ; Female ; Humans ; MELAS Syndrome/diagnosis* ; MELAS Syndrome/metabolism ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy
Magnetic resonance imaging ; diffusion study ; spectroscopy ; MELAS ; brain metabolism
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.
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1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Nuclear Medicine (핵의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Dong Ik(김동익)
Kim, Hyun Sook(김현숙)
Yoon, Pyeong Ho(윤평호)
Lee, Byung In(이병인)
Lee, Jong Doo(이종두)
Jeong, Eun Kee(정은기)
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