Generalized Lipofuscinosis (Generalized Kufs' Disease)

Abstract

Light microscopic and ultrastructural investigations in a case of systemic storage disease in an adult revealed lipofusein to be the stored substance in all cells involved. No lipid bodies or cytosomes characteristic for any storage disease were found. Similarly there were no structures which could be interpreted as transitional stages of such bodies into lipofusein. Cases like this might be considered as a nosologic entity but the possibility that they might be a manifestation of a yet undiscovered metabolic derangement, in particular a lipidosis, cannot be excluded.