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The case demonstrates typical features of mucopolysaccharidosis type I (enlarged head size, marked ventriculomegaly, diffuse alteration of the white matter (involving the subcortical U-fibres) signal detected as high signal intensity on T2-weighted sequences, multiple dilated perivascular spaces within the bilateral basal ganglia giving a cribriform-like appearance more at the left side, and finally diffuse cerebral atrophy in the form of prominent extra-axial CSF spaces especially at the bilateral temporal region). Other features include mega cisterna magna and an empty sella.