Article Figures & Data
Figures
- fig 1.
4-month-old male patient with severe developmental delay and elevated lactate in blood and CSF.
A, T2-weighted image showing striking hyperintensity in the periventricular frontal white matter and the caput of the dentate nuclei on both sides. Because of the swollen dentate nuclei, the frontal horns of the lateral ventricles are compressed.
B, Unenhanced, axial T1-weighted MR image obtained at the level of the third ventricle (same level as that shown in A). The third and lateral ventricles are compressed by the basal ganglia and thalami. Note the marked symmetrical hyperintensity of the frontal and less pronounced occipitoparietal white matter.
C, The same plane as that shown in B obtained after the injection of contrast medium. Although the striate bodies show dense symmetrical enhancement, the thalami take up contrast material only around the dorsal part of the third ventricle. There is also intensive enhancement of the white matter and the swollen mamillary bodies.
D, Midsagittal T1-weighted contrast-enhanced MR image. No abnormality is seen caudal to the plane of the inferior colliculi. Periaqueductal enhancement, as well as hypothalamic and mamillary body enhancement, is very dense.
