Preferential tau aggregation in von Economo neurons and fork cells in frontotemporal lobar degeneration with specific MAPT variants

Table 1 Subject demographics

Case no.	Clinical Dx	Primary Neuropathological Dx	MAPT variant	Age/Sex	Symptom duration (yr)	PMI (hr)	Thal phase	Braak stage	CERAD score	ADNC	CDR score
FTLD-tau/MAPT
1	bvFTD/PSP-RS	FTLD-tau/MAPT	IVS10 + 16 C > T	60/M	8	12.5	ND	1	absent	Low	2^a
2	bvFTD	FTLD-tau/MAPT	IVS10 + 16 C > T	60/M	13	15.8	1	0	absent	Low	3^b
3	bvFTD	FTLD-tau/MAPT	IVS10 + 16 C > T	63/F	17	19.7	1	0	absent	Low	3
4	bvFTD	FTLD-tau/MAPT	P301L	59/F	4	24.2	1	1	absent	Low	2
5	bvFTD	FTLD-tau/MAPT	P301L	75/M	9	8.6	1	2	absent	Low	3
6	bvFTD	FTLD-tau/MAPT	V337 M	68/F	13	18.0	0	1	absent	Not	3
7	PSP-RS	PSP	A152T	56/F	6	5.7	2	2	absent	Low	3
8	nfvPPA	CBD	A152T	63/M	10	> 48 h	1	3	absent	Low	1
Average				63	10	15	1	1			2.5
Sporadic bvFTD-PiD
1	bvFTD	PiD	None	60/F	6	15.1	2–3	1	moderate	Low	3^c
2	bvFTD	PiD	None	57/M	6	21.7	0	0	absent	Not	2^d
3	bvFTD	PiD	None	70/M	10	10.2	2	0	moderate	Low	n/a
4	bvFTD	PiD	None	72/M	9	24.3	0	0	absent	Not	3
5	bvFTD	PiD	None	69/F	13	6.3	0	2	absent	Not	3
6	bvFTD	PiD	None	76/F	13	25.5	0	0	absent	Not	3
7	bvFTD	PiD	None	57/M	13	12.4	1	1	absent	Low	3
Average				66	10	17	1	1			2.8

^aantemortem, 3 months prior to death, ^bantemortem, 20 months prior to death, ^cantemortem, 18 months prior to death, ^dantemortem, 7 months prior to death, diagnosis (Dx), behvaioral variant FTD (bvFTD), progressive supranuclear palsy-Richardson syndrome (PSP-RS), nonfluent variant primary progressive aphasia (nfvPPA), corticobasal degeneration (CBD), Pick’s disease (PiD), Postmortem interval (PMI)

ISSN: 2051-5960