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AJNR - American Journal of Neuroradiology

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Proton MR Spectroscopic Imaging in Pelizaeus-Merzbacher Disease

Francesca Pizzini^a, Ali. S. Fatemi^a, Peter B. Barker^a,b, Lidia M. Nagae-Poetscher^a,b, Alena Horská^b, Andrew W. Zimmerman^a,c, Hugo W. Moser^a,c, Genila Bibat^a and Sakkubai Naidu^a,c

^a Kennedy Krieger Institute
^b Department of Radiology, the Johns Hopkins University School of Medicine, Baltimore, MD
^c Department of Neurology, the Johns Hopkins University School of Medicine, Baltimore, MD

View larger version (96K): [in a new window]   FIG 1. Regions of interest (squares) in the frontal-lobe white matter, centrum semiovale, and parietal white matter and gray matter were chosen for analysis.

View larger version (51K): [in a new window]   FIG 2. Severe PMD. T2-weighted MR images (T2); metabolic images of Cho, Cr and NAA; and selected spectra from the parietal white matter (WM) and parieto-occipital gray matter (GM). T2-weighted MR images show diffuse hyperintensity throughout the white matter, which also has markedly decreased NAA levels. Cho and Cr levels are also mildly elevated in the centrum semiovale. Gray matter spectra also show a lower ratio of NAA/Cho and NAA/Cr in the patient compared with that in the control subject. A, Patient 1, aged 2 years 5 months. B, Control subject 1, aged 1 year 9 months.

View larger version (49K): [in a new window]   FIG 3. Mild PMD. T2-weighted MR imaging; metabolic images of Cho, Cr, and NAA; and selected spectra from the parietal white matter and parieto-occipital gray matter. T2-weighted MR images show patchy hyperintensity with greatest involvement in the centrum semiovale. NAA levels are bilaterally decreased in the parietal white matter. Gray-matter spectra are normal. A, Patient 2, aged 3 years 8 months. B, Control subject 2, aged 3 year 2 months.