A golgi and ultrastructural study of a dominant form of Kufs' disease

A cerebral biopsy from a patient with inherited dominant autosomic Kufs' disease was studied with Golgi's method and ultrastructurally. A marked PAS positive, sudanophile, autofluorescent deposit was observed in the cytoplasm and in the proximal region of the axon of neurons from the third layer. Ultrastructurally this is a granular, membrane-bound product, sometimes with with a dense, compact rectilinear pattern in which the typical clear component of adult lipofuscin is scanty. Sections stained with Golgi's method show a prominent, sometimes double, axon hillock swelling without dendritic spines. These facts are compared with additional samples of Alzheimer's disease and Huntington's chorea processed in a similar way.