Table 1.

Demographic, clinical, radiologic, and neuropathologic findings of affected individuals

Case	I.1	I.2	II.1	II.2	II.3	II.4	II.5 (p)	III.1	III.2	III.3
Age at Onset	69	83	79	*	75	80	69	66	55	61
Age at Death	74	86	86	82	77	87	76	Alive 71	65	70
Duration	5	3	7	*	2	7	7	> 5	10	9
Initial Diagnosis	AD	Dem NOS	AD	Dem NOS	AD	AD	aMCI	md-MCI + a	FTD + DLB	PPA
Final Diagnosis	AD	Dem NOS	AD	Dem NOS	AD + park	FTD	AD	md-MCI + a	FTDP	PPA + FTD
Memory Impairment	present	*	early	present	early	early	early	early	mid	late
Personality Change	*	*	*	present	present	early	late	*	early	late
Executive Dysfunction	*	*	*	*	present	mid	late	early	early	late
Language Dysfunction	*	*	*	*	*	mid	late	early	present	early
Parkinsonism	*	*	*	*	mid	late	late/term	absent	late	absent
MRI Topography of Atrophy						L>R F,T	mild L>R T	mild gen, falcine	mod gen, sev R T	L T>F
Pathology			FTLD-U + NII + AD + VD		FTLD-U + NII	FTLD-U + NII	FTLD-U + NII		FTLD-U + NII	FTLD-U + NII

Abbreviations: AD=Alzheimer's disease; aMCI=amnestic mild cognitive impairment;B=bilateral; Dem NOS=dementia not otherwise specified; DLB=dementia with Lewy bodies; F=frontal; FTD=frontotemporal dementia; FTDP=frontotemporal dementia and parkinsonism; FTLD-U + NII=frontotemporal lobar degeneration with ubiquitin positive neuronal cytoplasmic and intranuclear inclusions; gen=generalized; L=left; md-MCI + a=multi-domain mild cognitive impairment with amnesia; mod=moderate; (P)=proband; park=parkinsonism; PPA=primary progressive aphasia; R=right; T=temporal; VD=vascular dementia. early=onset within first year of symptoms; mid=onset 1-2 years after symptom onset; late=onset 3 or more years after onset; absent=no evidence in clinical records of this feature being present. Asterisks represent unavailable information