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.
D000328:Adult; D005917:Globus Pallidus / Q000378:metabolism*; D006651:Histocytochemistry; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism* / Q000473:pathology; D013378:Substantia Nigra / Q000378:metabolism*
D007501:Iron