Table 1.

Clinical and molecular characteristics of ten Iberian FTLD-MAPT-P301L cases

Case	Sex	Age at death	Duration (years)	Signs at onset	Initial diagnosis	MAPT haplotype	ApoE	PMI (hrs)	Frontal cortex	Dentate nucleus of the cerebellum		Hippocampus		Parahippocampus
									Neuronal loss/AT8	D/N	Neuronal loss/AT8	D/N	Neuronal loss/AT8	Neuronal loss/AT8
1	M	52	6	Behavior	bvFTD	H1/H1	e3/e3	13.5	++/+++	3.6 ± 0.2	−/−	1.0 ± 0.0	−/+++	++/+++
2	M	58	5	Behavior	bvFTD	H1/H1	e3/e4	13	+++/+++	4.1 ± 0.1	+/-	1.0 ± 0.1	−/+++	++/+++
8	M	58	7	Behavior	bvFTD	H1/H1	e3/e4	10	+++/++	4.9 ± 0.3	−/+	1.0± 0.1	−/+++	+++/+++
7a	F	61	5	Behavior	bvFTD	H1/H1	e3/e3	14.8	++/+++	4.6 ± 0.1	−/+	1.7 ± 0.1	−/+++	++/++
4	M	72	13	Memory	AD	H1/H1	e3/e3	5.8	+++/+++	3.7 ± 0.3	−/+	1.0 ± 0.1	+/+++	+++/+++
6	M	53	7	Memory	AD	H1/H1	e3/e3	16.7	+++/+++	12.6 ± 1.2	−/−	1.2 ± 0.1	−/+++	+++/+++
9	M	75	7	Behavior	AD	H1/H2	e3/e3	5.9	++/+++	5.9 ± 0.1	−/−	3. ± 0.1	−/+++	++/+++
5	F	63	4	Language	AD or svPPA	H1/H2	e3/e3	7.3	++/++	4.6 ± 0.1	−/+	1.2 ± 0.2	−/+++	++/+++
3	M	56	10	Language	svPPA	H1/H1	e3/e3	6.3	+++/+++	10.3 ± 0.9	+/−	1.0 ± 0.1	−/+++	+++/+++
12	M	49	6	Language	svPPA	H1/H1	e3/e3	7.4	++/+++	8.2 ± 0.5	−/+	3.3 ± 0.1	ND	ND

Table after [7], arranged by clinical diagnosis: bvFTD behavioral variant of FTD, svPPA semantic variant of primary progressive aphasia, AD Alzheimer disease, PMI post mortem interval. All presented cases were FUS-negative, TDP43-negative. D/N denatured/native ratio in CDI assay, ± standard deviation

Age at onset was 51.3 ± 4, 60.7 ± 11.9, 50.0 ± 7 years for bvFTD, AD and svPPA, respectively. Age at death was 57.3 ± 3.8, 66.7 ± 11.9 and 56.0 ± 7 years, respectively. None of the differences in ages between the different initial diagnoses were significant

^aAlso features globular glial tauopathy affecting oligodendrocytes