The authors report five elderly men with the fragile X premutation who had
a progressive action tremor associated with executive function deficits and
generalized brain atrophy. These individuals had elevated fragile X mental
retardation 1 gene (FMR1) messenger RNA and normal or borderline levels of
FMR1 protein. The authors propose that elevations of FMR1 messenger RNA ma
y be causative for a neurodegenerative syndrome in a subgroup of elderly me
n with the FMR1 premutation.