Achromatic neurons (AN) in the cerebral cortex are a characteristic fe
ature of several neurodegenerative conditions including Pick's disease
, corticobasal degeneration and some cases of primary progressive apha
sia. Although AN are occasionally seen in some other diseases, their p
resence in progressive supranuclear palsy (PSP) has not been previousl
y documented. We found significant numbers of AN in the cerebral corte
x of five out of seven cases which had been diagnosed pathologically a
s PSP The identification of AN was greatly facilitated by the use of n
eurofilament immunohistochemistry. The entorhinal and transentorhinal
cortices were most frequently involved, but in several cases AN were a
lso seen throughout the neocortex. The presence and number of AN rough
ly correlated with a history of clinical dementia. This suggests that
cortical AN may be a common and important pathological finding in PSP.