A case of Kufs disease with abnormal pallidonigral iron deposit

The clinical, neuropathological and ultrastructural features of a case of Kufs disease (Meyer basal ganglia form) are reported. In the present case, besides the typical lesions of Neuronal Ceroid-Lipofuscinosis, and increased iron reaction at pallidonigral level was detected histochemically. The significance of the last finding is discussed.