Table 1.

Table Characteristics of TDP-43 positive Hippocampal Sclerosis Dementia

C9ORF72 mutation	ID	Sex	Age at Onset	Age at death	Years of illness	Initial diagnosis	Final diagnosis	CERAD stage	Braak stage	TDP43 type	UBQ2*	p62*
+	1	F	67.0	74.6	7.6	AD	AD	0	II	A	H+ C+	H+ C+
+	2	F	66.6	79.4	12.8	AD	FTD	0	II	A	H+ C+	H+ C+
+	3	F	71.0	82.2	11.2	Other	AD	0	II	A	H+ C+	H+ C+
−	4	M	47.7	55.0	7.3	AD	AD	0	0	A	H+ C−	H+ C−
−	5	M	--	69.0	--	AD	AD	0	II	C	H+ C−	H+ C−
−	6	M	65.5	73.9	8.4	AD	AD	0	0	Ind.	H+ C−	H+ C−
−	7	M	67.6	81.8	14.3	AD	FTD	0	II	B	H+ C−	H+ C−
−	8	F	83.3	96.9	13.7	AD	AD	0	II	C	H+ C−	H+ C−
−	9	F	72.2	82.2	10.0	AD	AD	A	0	Ind.	H+ C−	H+ C−
−	10	M	78.6	83.3	4.7	AD	FTD	0	III	Ind.	H+ C−	H+ C−

Initial and final clinical diagnoses are shown; case #1 had a family history of neuropsychiatric disorder.

Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) amyloid plaque staging, A = low likelihood of Alzheimer disease (AD), B = intermediate, C = high. Braak neurofibrillary tangle staging, I/II = prodromal, III/IV early-moderate AD, V/VI = late stage.

TDP43 type = indeterminate (Ind.) in cases where neocortical inclusions are absent or sparse and assignment to a type (A, B, C or D) cannot be made.

^*Cytoplasmic ubiquilin-2 (UBQ) and p62 inclusion bodies in hippocampal (H) and cerebellar neurons (C).