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PT  - JOURNAL ARTICLE
AU  - Flemming, Katharina
AU  - Ulmer, Stefan
AU  - Duisberg, Barbara
AU  - Hahn, Andreas
AU  - Jansen, Olav
TI  - MR Spectroscopic Findings in a Case of Alpers-Huttenlocher Syndrome
DP  - 2002 Sep 01
TA  - American Journal of Neuroradiology
PG  - 1421--1423
VI  - 23
IP  - 8
4099  - http://www.ajnr.org/content/23/8/1421.short
4100  - http://www.ajnr.org/content/23/8/1421.full
SO  - Am. J. Neuroradiol.2002 Sep 01; 23
AB  - Summary: Alpers-Huttenlocher syndrome, considered a mitochondrial disease, combines encephalopathy and liver failure. An 11-year-old boy with Alpers-Huttenlocher syndrome underwent conventional MR imaging, diffusion-weighted imaging, and proton MR spectroscopy. Diffusion-weighted imaging showed cytotoxic edema interpreted as acute-phase encephalopathy. MR spectroscopy revealed a lactate peak in the cortex that appeared abnormal on diffusion-weighted images, possibly representing respiratory deficiency with anaerobic metabolism. MR spectroscopy proved to be more sensitive regarding lactate detection than did neurometabolic examination of serum and CSF. A reduced N-acetylaspartate-creatine ratio was detected in both the cortex that appeared abnormal and the cortex that appeared normal on the diffusion-weighted images, indicating neuronal damage that was widespread, even beyond the boundaries of conventional MR imaging changes.