Neuropathology of late onset gangliosidoses. A review

Neuropathological features of late onset gangliosidoses are reviewed. Although neuropathological studies are carried out on limited numbers of late onset cases, it appears that electron-dense heterogeneous conglomerates of neuronal inclusions increased with age admixed with more typical membranous cytoplasmic inclusions of gangliosidosis. Unlike early onset cases, which show extensive storage in cerebral cortical neurons, cortical neurons are involved less in late onset cases. In chronic (or adult) GM1 gangliosidosis, neuronal storage is almost exclusively limited to the basal ganglia, while in chronic (or adult) GM2 gangliosidosis storage neurons are more widely distributed in the thalamus, substantia nigra and other brainstem nuclei, and cerebellum is significantly affected.