. Author manuscript; available in PMC: 2012 Apr 11.

Published in final edited form as: J Mol Neurosci. 2011 Sep 3;45(3):343–349. doi: 10.1007/s12031-011-9632-1

Table 3.

FTDP-17T/MAPT vs. FTDP-17U/PGRN

	FTDP-17T/MAPT	FTDP-17U/PGRN
Age	Younger, 40s	Older, >50 years
Predominant clinical phenotype	Parkinsonism and personality change are more common	Language abnormalities, parkinsonism less common (except for CBS)^a
Most common presenting deficits	Behavioral/personality changes, semantic impairment	Anomia, apathy (or disinhibition), apraxia
Penetrance	Nearly 100% (false sporadic cases are rare)	Age dependent: 90% reached by 70 years
Disease duration	Mean, 5 years	Mean, 7 years
	Range, 3–10 years	Range, 1–15 years
Common clinical presentations	bvFTD, CBS, PSP, AD	PNFA, CBS, AD, PDD/DLB
Response to levodopa	Commonly present but rarely sustained	Rarely present
Distribution of atrophy	Anteromedial temporal lobe and orbitofrontal region; caudate	Inferior frontal, temporal, and inferior parietal lobe
Symmetry of atrophy	Symmetric	Asymmetric. Asymmetry becomes greater over time

Although pathology of FTDP-17U/PGRN is always FTLD-TDP, MND is exceptionally rare among PGRN carriers

bvFTD behavioral variant of frontotemporal dementia, PNFA progressive nonfluent aphasia, PDD Parkinson’s disease dementia, DLB dementia with Lewy bodies