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American Journal of Neuroradiology 23:1421-1423, September 2002
© 2002 American Society of Neuroradiology

Case Report
PEDIATRICS

MR Spectroscopic Findings in a Case of Alpers-Huttenlocher Syndrome

Katharina Flemminga, Stefan Ulmera, Barbara Duisbergb, Andreas Hahnb and Olav Jansena

a Section of Neuroradiology, Clinic of Neurosurgery, Christian-Albrechts-Universität Kiel, Kiel, Germany
b Clinic of Neuropediatrics, Christian-Albrechts-Universität Kiel, Kiel, Germany

Address reprint requests to Katharina Flemming, Herzog-Friedrich-Strasse 66, 24103 Kiel, Germany

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.




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