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AJNR - American Journal of Neuroradiology

Published ahead of print on December 7, 2007
doi: 10.3174/ajnr.A0890

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Nearly Completely Reversible Brain Abnormalities in a Patient with Incontinentia Pigmenti

H. Lou^a, L. Zhang^b, W. Xiao^a, J. Zhang^a and M. Zhang^a

^a Department of Radiology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
^b Department of Neurology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China

View larger version (39K): [in this window] [in a new window]   Fig 1. MR imaging and spectroscopy at 19 days of age. T1-weighted imaging (TR = 400 ms, TE = 15 ms, section thickness = 6 mm) shows the foci signals of hemorrhage in the left parietal lobe (arrow, A). Spectroscopy with a 2 x 2 x 2 cm3 voxel located on the contralateral region of the parietal lobe, by using a point-resolved spectroscopic sequence technique (TE = 35 and 144 ms), shows a prominent Lac doublet and decreased NAA peak (B and C).

View larger version (49K): [in this window] [in a new window]   Fig 2. MR imaging and spectroscopy at 34 days of age. Cutaneous hyperpigmented macules become more apparent along Blaschko lines in the inguinal regions (A). Fluid-attenuated inversion recovery image (B) demonstrates low signal intensity of multiple patchy softening of the subcortical white matter in the bilateral hemisphere. MR spectroscopy (multivoxel point-resolved spectroscopic sequence technique with TE = 144 ms) (C and D) in different regions shows evident reduction in NAA resonance peak amplitude at 2.02 ppm in cavities of white matter (D) (No. 4, 5 represented) and increase in the Cho peak in the adjacent parenchyma (D) (Nos. 2 and 3, 8 represented).

View larger version (142K): [in this window] [in a new window]   Fig 3. MR imaging at 4.5 months of age. Fluid-attenuated inversion recovery image reveals the disappearance of the previously observed abnormalities. However, multiple small cystic lesions exhibiting low intensity in the gray matter are present (arrow), and bilateral cerebral atrophy is also observed.