Presentation
Learning difficulty at school. Visual and hearing impairments. Intermittent strabismus. Overall developmental regression.
Patient Data
MRI brain demonstrate symmetrical, confluent T1 hypointense and T2 hyperintense signal abnormality involving the peritrigonal parieto-occipital white matter with extension into the subcortical U-fibers. (Figure 1, 2 and 3)
In the craniocaudal direction signal abnormality is seen in the corticospinal tracts extending from the centrum semioavle to the level of the pyramids of the medulla (Figure 3 and Figure 4). Both auditory (Figure 5) and visuals (figure 6) pathways are also involved.
The leading edge of the white matter signal abnormality demonstrate enhencement (Figure 7 and 8). No involvement of the cerebellum is evident.
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