Diffusion-weighted image and MR spectroscopic analysis of a case of MELAS with repeated attacks.
10.3349/ymj.2001.42.1.128
- Author:
Hyun Sook KIM
1
;
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
Author Information
1. Department of Diagnostic Radiology, Yonsei University College of Medicine, Seoul, Korea. dikim@yumc.yonsei.ac.kr
- Publication Type:Case Report
- Keywords:
Magnetic resonance imaging;
diffusion study;
spectroscopy;
MELAS;
brain metabolism
- MeSH:
Adolescence;
Aspartic Acid/metabolism;
Aspartic Acid/analogs & derivatives*;
Brain/metabolism;
Case Report;
Creatine/metabolism;
Diffusion;
Female;
Human;
MELAS Syndrome/metabolism;
MELAS Syndrome/diagnosis*;
Magnetic Resonance Imaging;
Magnetic Resonance Spectroscopy
- From:Yonsei Medical Journal
2001;42(1):128-133
- CountryRepublic of Korea
- Language:English
-
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.
