Figure 3. . The MRI in a severe, early-onset form of LBSL at age two days (A, B, C) and age five months (D, E, F) shows striking progression of abnormalities.

Figure 3.

The MRI in a severe, early-onset form of LBSL at age two days (A, B, C) and age five months (D, E, F) shows striking progression of abnormalities. At two days, sagittal T1-weighted (A) and axial T2-weighted images (B) show hypoplasia of the cerebrum with numerous tortuous blood vesssels on the surface of the brain (A, B). In the medulla, the inferior cerebellar peduncles and pyramids are clearly affected (C). At follow up, the cerebral mantle has been reduced to a thin rim, with degeneration of virtually all cortex and white matter (D, E). The tortuous vessels are no longer present. The posterior limb of the internal capsule is abnormal in signal (E). The axial image through the cervical spinal cord (F) shows signal abnormalities in the corticospinal tracts and dorsal columns.

Images for this figure courtesy of Dr Menno Stellingwerff

From: Leukoencephalopathy with Brain Stem and Spinal Cord Involvement and Lactate Elevation

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