MR Imaging and Proton Spectroscopy of Neuronal Injury in Late-Onset GM2 Gangliosidosis
Matilde Inglesea,
Annette O. Nusbauma,
Gregory M. Pastoresb,
John Gianutsosc,
Edwin H. Kolodnyb and
Oded Gonena
a From the Departments of Radiology, New York University, New York, NY
b Neurology, New York University, New York, NY
c Rehabilitation Medicine, New York University, New York, NY
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FIG 1. A, top left, Axial T2-weighted image from a 54-year-old late-onset GM2G patient superimposed with the 5 x 5 cm2 VOI. Right, 1H spectrum from the cerebellum (arrow).
B, bottom left, Corresponding section from a 52-year-old healthy control superimposed with the 5 x 5 cm2 VOI. Right, Arrow indicates analogous region for metabolite comparison. Both spectra are on the same scale. Note the dramatic atrophy of the cerebellar folia and vermis in the patient and consequent lower metabolites levels reflecting larger partial CSF volume contamination.
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FIG 2. A, top left, T2-weighted image of a 54-year-old patient with late-onset GM2G, superimposed with the 8 x 8 cm2 1H-MR spectroscopy VOI. Right, Two spectra from thalamus and occipital white matter (arrows 1 and 2).
B, bottom left, Corresponding section from a matched control. The arrows indicate analogous regions to A for metabolite spectral comparisons. All spectra are on common intensity and chemical shift (parts per million) scales.
Note the characteristic lower NAA signal intensity in thalamus and NAWM in the patient (A) compared with the control (B), as well as decreased Cr and Cho.
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