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
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FIG 1. Diffusion-weighted (5100/137 [TR/TE]; acquisition time, 20 s; number of acquisitions, one) images show cytotoxic edema in the right frontal and left parieto-occipital cortices as an expression of an acute phase of encephalopathy. Proton spectra were acquired from the right frontal cortex (A) and from the normal-appearing right parietal cortex (B).
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FIG 2. Spectrum (1500/135; acquisition time, 6 minutes 31 seconds; number of acquisitions, 256; voxel size, 8 mL) acquired from voxel A of the diffusion-weighted image (Fig 1) (abnormal right frontal cortex), reveals a distinct lactate peak and a reduced NAA/Cr ratio.
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FIG 3. Spectrum (same parameters as those in Fig 2) acquired from voxel B of the diffusion-weighted image (Fig 1) (normal-appearing right parietal cortex, which also appeared normal on T2-weighted MR images), displays no lactate but a reduced NAA/Cr ratio, although not as distinctly as for voxel A.
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FIG 4. T2-weighted (3000/120; acquisition time, 7 minutes 16 seconds; number of acquisitions, one) MR image shows cortical thickening and edema in the right frontal and left parieto-occipital cortices. The right parietal cortex appears normal in the T2-weighted images.
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