Using a liberal criterion, a conservative probability-based criterion,
and a criterion for autosomal dominant inheritance, we classified 36%
, 13.5%, and 6.4% of 311 patients, respectively, as having familial Al
zheimer's disease (FAD). The mean age of onset was over 70 years for a
ll three categories of FAD. FAD and sporadic Alzheimer's disease (SAD)
cases did not differ in clinical features, incidence of risk factors
for dementia, or MRI or PET features. We observed earlier age of onset
of AD to be related positively to longer duration of disease. Except
for the autosomal dominant AD group, there was an earlier age of onset
in FAD probands. The inheritance of AD from mothers was from 1.7 to 3
.6 times more frequent than from fathers. Among SAD patients only, we
found a preponderance of women, who were more frequently affected than
would be expected from the male/female ratio in the general populatio
n of the same average age. Language performance tended to be less affe
cted in FAD than in SAD patients, contrary to some previous reports.