TY - JOUR T1 - MR Spectroscopic Findings in a Case of Alpers-Huttenlocher Syndrome JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 1421 LP - 1423 VL - 23 IS - 8 AU - Katharina Flemming AU - Stefan Ulmer AU - Barbara Duisberg AU - Andreas Hahn AU - Olav Jansen Y1 - 2002/09/01 UR - http://www.ajnr.org/content/23/8/1421.abstract N2 - 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. ER -