Loss of pigment-laden stellate cells: a severe alteration of the isocortex in juvenile neuronal ceroid-lipofuscinosis.

Juvenile neuronal ceroid-lipofuscinosis is associated with a moderate numerical decrease of isocortical neurons. From Nissl preparations, it is not apparent, whether this neuronal rarefication occurs at random or affects specific types of cortical neurons. Lipopigment preparations facilitate the distinction between pyramidal and stellate cells. In juvenile neuronal ceroid-lipofuscinosis, they reveal an almost complete to total loss of small pigment-laden stellate cells. This type of local circuit neurons, scattered throughout the corpuscular and pyramidal layer in varying numbers and patterns of distribution, is common to all isocortical regions. Its pronounced and selective vulnerability in this lysosomal disease may be causally related to the marked functional impairment of the brain.