Pallido-nigro-luysial atrophy with massive appearance of corpora amylacea in the CNS.
A case of pallido-nigro-luysial atrophy, characterized by symptoms typical of progressive supranuclear palsy, presented (1) severe symmetrical abiotrophic degeneration of the globus pallidus, substantia nigra, and subthalamic nucleus and (2) widespread accumulations of corpora amylacea in the CNS. The former is thought to be consistent with pallido-nigro-luysial atrophy and we regard it as a combined system degeneration of both the pallido-nigral and the pallido-luysial systems. The significance of the latter is quite obscure. As far as we know, only three cases of pallido-nigro-luysial atrophy have been reported. However, there is no reported case of this disorder with such widespread and massive accumulations of corpora amylacea as in our case.
D000368:Aged; D001284:Atrophy; D001927:Brain Diseases / Q000473:pathology*; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D008297:Male; D013378:Substantia Nigra / Q000473:pathology*