A patient with a rapidly developing fluent progressive aphasia was tes
ted prospectively up to the time of death and examined neuropathologic
ally. Severe impairment in accessing semantic skills with substantiall
y intact phonological, syntactic and discourse skills was found. Some
social behavioural difficulties were also noted. This case presented a
unique opportunity to relate this significant language impairment to
the pattern of neurodegeneration, a difficult task in most neuropathol
ogical studies of severe end-stage dementia. A detailed neuropathologi
cal examination revealed focal atrophy with neuronal loss without neur
onal inclusions (Pick bodies, Lewy bodies, neurofibrillary tangles or
senile plaques) or neuronal changes (shrinkage or swelling). In additi
on, spongiform degeneration (confined to layer two of the cortex) and
gliosis were detected at atrophic sites. To establish the amount of ti
ssue loss and pathology associated with the focal language deficit, vo
lume analyses were performed and compared with two age- and sex-matche
d, neurologically normal controls. Both the left and right angular gyr
i and Brodmann's area 37 showed marked volume reduction compared with
controls. The predominant language impairment seen in this case is lik
ely to reflect these marked changes in the posterior parieto-temporal
areas. The milder unilateral atrophy was concentrated in the right tem
poral lobe as well as the right hemisphere homologue of Broca's area.
Recent work suggests a relationship between such unilateral changes an
d the social behavioural difficulties which were noted in this case. T
he hippocampus and other gyri such as the supramarginal gyrus showed n
o volume loss compared with controls correlating with the relative pre
servation of other language skills.