Cerebral GM1-gangliosidosis: chemical pathology of visceral organs.

The livers and spleens from three patients with cerebral G(M1)-gangliosidosis contained greatly increased concentrations of a mucopolysaccharide tentatively identified as keratan sulfate. The concentration of a very soluble sialomucopolysaccharide was also increased. Concentrations of these compounds were not increased in the viscera of patients with Tay-Sachs disease (G(M2)-gangliosidosis). G(M1)-gangliosidosis appears to be a combined cerebral gangliosidosis and visceral mucopolysaccharidosis.