Pigmentoarchitectonic pathology of the isocortex in juvenile neuronal ceroid-lipofuscinosis: axonal enlargements in layer IIIab and cell loss in layer V.

Pigment preparations of 800 micrometer thickness and Golgi studies of the isocortex in juvenile neuronal ceroid-lipofuscinosis, morphologically proven by electron microscopy, revealed: 1. giant axonal dilatations of IIIab-pyramids. These expansions exceeded by far those found in pyramidal cell axons of other isocortical layers. 2. severe numerical reduction of neurons in the ganglionic layer.