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Case Reports
. 1980 Jan;222(3):183-90.
doi: 10.1007/BF00313117.

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

I Ferrer, T Arbizu, J Peña, J P Serra
Case Reports

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

I Ferrer et al. J Neurol. 1980 Jan.

Abstract

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.

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