Proton MR Spectroscopy of Mitochondrial Diseases: Analysis of Brain Metabolic Abnormalities and Their Possible Diagnostic Relevance
M. Cristina Bianchia,b,
Michela Tosettib,
Roberta Battinib,
Maria L. Mancac,
Michelangelo Mancusoc,
Giovanni Cionib,d,
Raffaello Canapicchib and
Gabriele Sicilianoc
a U.O. di Neuroradiologia, Ospedale S. Chiara, Pisa, Italy
b Istituto Scientifico Stella Maris, Pisa, Italy
c Dipartimento di Neurologia, Università di Pisa, Italy
d Divisione di Neuropsichiatria Infantile, Università di Pisa, Italy
View larger version (112K):
[in a new window]
|
FIG 1. Patient 14 with Leigh syndrome.
A and B, Axial FLAIR images show bilateral and symmetrical hyperintensity of corpora striata. The VOIs used for spectroscopy are also shown.
C, Spectrum from the VOI containing the right affected putamen demonstrates resonances at 1.33 and 0.9 ppm coming respectively from lactate (Lac) and lipids or branched amino-acids (Lip/aa).
D, Abnormally high brain lactate (Lac) is also revealed in the spectrum from the VOI located in paraventricular white matter that appeared normal on the MR image.
Cho indicates choline; Cr, creatine.
| |
View larger version (111K):
[in a new window]
|
FIG 2. Patient 15 with MELAS.
A and B, Axial FLAIR images demonstrate multiple cortical strokelike lesions, the largest of which was located along the parieto-occipital cortex (not shown). Tiny frontal and parietal cortical hyperintensities and the VOIs used for spectroscopy are visible.
C and D, Spectra reveal high lactate (Lac) and the 0.9 ppm signal (Lip/aa) not only into the damaged cortex (C) but also into the normal-appearing parietal cortex (D). A significant decrease of NAA may also be appreciated in the spectrum in C. Cho indicates choline; Cr, creatine.
| |
View larger version (12K):
[in a new window]
|
FIG 3. A–C, Single-voxel proton spectra from a normal-appearing cerebellar volume (A, patient 12), a normal-appearing parieto-occipital white matter volume (B, patient 3), and a normal-appearing parieto-occipital cortex (C, patient 1). Spectra reveal a significant decrease of choline (Cho) and the presence of a low amplitude signal at 0.9 ppm (Lip/aa) in each anatomic location. NAA is decreased in cerebellar (A) and parieto-occipital cortex (C) locations. Cr indicates creatine.
| |
View larger version (17K):
[in a new window]
|
FIG 4. A–C, Plots of the metabolite ratios reported in Table 3 for cerebellum (A), white matter (B), and parieto-occipital cortex (C). Vertical bars are the range of each ratio in 15 control subjects. mI indicates myo-inositol; Cho, choline; Lac, lactate
| |
