GM-2 GANGLIOSIDOSIS (SANDHOFFS DISEASE) - 2-YEAR FOLLOW-UP BY MRI

Two children with GM-2 gangliosidosis type 0 (Sandhoff's disease) foll owed up by MRI at 1.5 Tesla for 1.8 years are reported. One was presym ptomatic at the first MRI examination. As her neurological status dete riorated, MRI showed low signal in bilaterally, on T2-weighted images the white matter with involvement of the optic radiations. In the seco nd, MRI correlated well with the clinical progression of the disease, showing in the different stages involvement of thalamus and basal gang lia. There was no contrast enhancement and the grey matter remained no rmal.