There have been no previous three-dimensional volumetric studies of regiona
l brain atrophy in patients with pathologically confirmed progressive supra
nuclear palsy (PSP). Postmortem cortical and subcortical volumes were compa
red with neuropathology in 9 patients with PSP, 15 patients with 'Parkinson
's disease, 10 patients with dementia with Lewy bodies, and 23 controls. Ca
ses with the neuritic pathology of Alzheimer's disease were excluded. The t
opography of brain atrophy differed according to clinicopathological phenot
ype. Patients with Parkinson's disease had atrophy confined to the amygdala
. Atrophy of the frontal lobe was found in both PSP and dementia with Lewy
bodies and correlated with increasing neurofibrillary tangle or Lewy body d
ensities, respectively. Patients with PSP could be differentiated by their
marked atrophy of the internal globus pallidus. Further analysis of varianc
e revealed that trends for greater frontal lobe atrophy correlated with cli
nical dementia in PSP, whereas both greater frontal and hippocampal atrophy
and higher densities of Lewy bodies and Lewy neurites correlated with clin
ical dementia in cases with Lewy bodies, The present study provides evidenc
e for selective regional atrophy that correlates with the underlying pathol
ogy of PSP and Lewy body disease.