[HTML][HTML] Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study
I Nestrasil, A Ahmed, JM Utz, K Rudser… - Molecular genetics and …, 2018 - Elsevier
Background GM1-gangliosidosis and GM2-gangliosidosis (Tay-Sachs disease and
Sandhoff disease) are unrelenting heritable neurodegenerative conditions of lysosomal
ganglioside accumulation. Although progressive brain atrophy is characteristic, longitudinal
quantification of specific brain structures has not been systematically studied. Objectives The
goal of this longitudinal study has been to quantify and track brain MRI volume changes,
including specific structure volume changes, at different times in disease progression of …
Sandhoff disease) are unrelenting heritable neurodegenerative conditions of lysosomal
ganglioside accumulation. Although progressive brain atrophy is characteristic, longitudinal
quantification of specific brain structures has not been systematically studied. Objectives The
goal of this longitudinal study has been to quantify and track brain MRI volume changes,
including specific structure volume changes, at different times in disease progression of …
