Fig. 15

Neuroradiological imaging findings. Involvement of basal ganglia in an MPS VI patient; the MR exam was acquired when the patient was 17. Axial T2-weighted fast spin-echo (a) brain image obtained at the level of the third ventricle with magnification focused on basal ganglia (b) demonstrating symmetrical focal areas of hyperintensity in the lenticular nucleus (black arrow) and in the thalamus (black arrowhead) because of enlarged perivascular spaces. On corresponding T2-weighted FLAIR images (c and d), these areas (white arrow and white arrowhead) are hypointense. This typical imaging described in the brain of patients with MPS is the “honeycomb-like” appearance. Note also ventriculomegaly and diffuse high signal intensity in the periventricular white matter