Vacuolating Megalencephalic Leukoencephalopathy with Mild Clinical Course Validated by Diffusion Tensor Imaging and MR Spectroscopy
Yi-Fang Tua,
Cheng-Yu Chenc,
Chao-Ching Huangb and
Chang-Shin Leec
a Department of Emergency Medicine, National Cheng Kung University Hospital, Tainan, Taiwan
b Department of Pediatrics, National Cheng Kung University Hospital, Tainan, Taiwan
c Department of Radiology, Tri-Service General Hospital, Taipei, Taiwan
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FIG 1. MR images obtained when the patient was 2 years 10 months old.
A, Axial fast spin-echo T2-weighted image (TR/TE, 3500/93; two signals acquired) at the level of the basal ganglia, showing diffuse demyelination involving mainly the frontal lobe sparing the occipital subcortical regions and internal capsule.
B, Coronal T1-weighted contrast-enhanced image (700/14), showing periventricular linear enhancement next to bilateral frontal horns.
C, Axial T1-weighted contrast-enhanced image (700/14), demonstrating enhanced lesions in bilateral globus pallidi (arrows).
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FIG 2. Follow-up axial T2-weighted MR image (4000/97.6), obtained when the patient was 5 years old, at the level of the basal ganglia, showing consistent demyelination at the same regions on Fig 1A with thinning of the genu of the corpus callosum and dilatation of the cavum septi pellucidi.
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FIG 3. MR and DT images obtained when the patient was 5 years 8 months old.
Axial T2-weighted MR image (A) at the level just above the body of the corpus callosum, showing diffuse high-signal-intensity white matter abnormalities. On FA (B and D) and vector maps (96 x 128 matrix size) (C and E), the white matter anisotropy and directionality were only mildly impaired (arrows) in our patient (B and C) as compared with a healthy control subject (D and E).
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FIG 4. Depiction of regions of interest used in data analysis obtained when the patient was 5 years old. Each region of interest was placed symmetrically and bilaterally except for region of interest 4.
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FIG 5. Multivoxel 1H MR spectra, obtained when the patient was 5 years old showing a significant decrease of NAA/Cr + phosphocreatine. Left spectrum, which is a magnified voxel indicated with thick frame in multivoxel 1H MR spectroscopy, shows the presence of lactate peaks in the temporoparietal white matter regions.
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