A clinicopathologic examination of pallidonigroluysian atrophy

In people presenting with early senility, who show gradual progression of akinesia and postural instability, it should be considered together with the disease whose lesion would be in the pallidonigroluysian system, When a patient is a poor responder to the dopaminergic agents and who develops no supranuclear ophthalmoplegia or nuchal dystonia, which is characteristic of PSP, even 4-5 years after onset, PNLA (pallidonigroluysian atrophy) should be considered as a possible diagnosis, The neuropathological findings show that globose-type tangles, glial tangles and neuropil threads are very rarely observed in PNLA even on the specimens stained by Gallyas-Braak method, while severe neuronal cell loss and gliosis are present in the pallidonigroluysian system, PNLA should be considered as a disease distinguishable from atypical PSP.