Table 1.

Characteristics of FTD caused by MAPT and PGRN mutations

Characteristic	FTDP-17 (MAPT)	FTLD-U (PGRN)
Mean onset age, years	49 (25–76)	59 (48–83)
Clinical presentation	Behavioral changes or parkinsonism, supranuclear gaze palsy, dystonia	Behavioral changes, language impairment, rarely parkinsonism, CBS, MND
Mean disease duration, years	7 (2–30)	7 (2–17)
Pathology	FTLD, tau-immunoreactive inclusions	FTLD, ubiquitin immuno- reactive/TDP-43-positive intranuclear and cytoplasmic inclusions

Figures in parentheses indicate ranges. CBS = Corticobasal syndrome; FTDP-17 = frontotemporal dementia with parkinsonism linked to chromosome 17; FTLD = fronto-temporal lobar degeneration; MND = motor neuron disease.