Table 1.

Studies used to gather data for clinicopathological associations of FTLD-tau and FTLD-TDP

Study	Hodges et al. [38]	Kertesz et al. [67]	MacKenzie et al. [80]/Davidson et al. [25]	Josephs et al. [56]	Forman et al. [31]	Snowden et al. [111]	Grossman et al. [35]	Josephs et al. [57]
Institution	Sydney, Australia and Cambridge, UK	London, Ontario, Canada	Manchester, UK	Rochester, MN, USA	Philadelphia, PA, USA	Manchester, UK	Philadelphia, PA, USA	Jacksonville, FL, USA
Study criteria	Path proven FTLD or CBD from dementia clinics. Excluded PSP	Clinical diagnosis of FTD, CBS or PSP that went to autopsy	Path diagnosis of FTLD-U or FTLD-MND.	Path diagnosis of FTLD, CBD or PSP from movement disorders and dementia clinics	Clinical diagnosis of dementia as well as FTLD, CBD or PSP path diagnosis	Path diagnosis of FTLD or CBD from a cerebral function unit	Path diagnosis of FTLD-U or FTLD-MND	Path diagnosis of FTLD-U or FTLD-MND
No. of cases reported
Total	61	60	37	127	90	65	23	39
Tau	31	21	0	87	53	25	0	0
TDP	30	24	37	39	37	40	23	39
FUS	0	0	0	0	0	0	0	0
Other	0	15	0	1	0	0	0	0
No. of FTLD pathological cases reported that had syndromic diagnoses of bvFTD, PNFA, SD, FTD-MND, CBS or PSPS
Total	61	33a	37	119	0b	62	18d	39
Tau	31	13	0	80	0	22c	0	0
TDP	30	20	37	39	0	40	18	39
FUS	0	0	0	0	0	0	0	0
Fig. 2a	X	X	–	X	X	X	–	–
Fig. 2b	–	–	X	–	–	–	X	X
Fig. 8	X	X	–	X	–	X	–	–
Fig. 9a	X	X	–	X	–	X	–	–
Fig. 9b	–	–	X	–	–	–	X	X

^a22 cases were diagnosed as PPA, and could not be classified as PNFA or SD, and 5 cases were not included in the review since they did not have an FTLD, CBD or PSP pathological diagnosis

^bSpecific clinical diagnoses (bvFTD, PNFA, SD, FTD-MND, CBS or PSPS) were not provided separately for the FTLD-tau and FTLD-TDP cases

^cThree patients were diagnosed with apraxia (PAX), but not CBS

^dThree cases had a clinical diagnosis of AD and two had a clinical diagnosis of dementia with Lewy bodies