Magnetic resonance imaging (MRI) and Tc99m-ECD-single-photon emission computed tomography (SPECT) of the patient. a-c MRI at age 12. There was mild atrophy of the frontal cortex and slightly low intensity in the globus pallidus on T2-weighted images (T2WI) (a, b). The saggital section of the T1WI exhibited cerebellar atrophy (arrow) (c). d-f MRI at age 18. Low signal intensity in the globus pallidus (arrowhead) and atrophy of the temporal lobes was clear on the T2WI (d). The frontal lobes showed severe atrophy (e). The saggital section of the T1WI exhibited severe cerebellar atrophy (arrow) and thinness of the corpus callosum (f). An ECD-SPECT, at age 18, revealed hypoperfusion of the frontotemporal lobes and cerebellum (g).