Heterogeneity of cortical pTDP-43 inclusion morphologies in amyotrophic lateral sclerosis

Table 1 Demographic, clinical and pathological characteristics of ALS-TDP subtypes

	ALS-TDP type B	ALS-TDP type E	ALS-TDP type SC	P value
N (% male)	41 (56%)	11 (36%)	9 (78%)	0.2
Age at death (years)	64 ± 11	65 ± 7	68 ± 12	0.6
Age at disease onset (years)	62 ± 11	63 ± 7	64 ± 12	0.8
Disease duration (years)	3 ± 1	3 ± 2	4 ± 2	0.2
Site of onset UL/LL/Bulbar (n)	15/11/15	6/2/3	4/4/1	0.5
Postmortem interval (hours)	27 ± 14	27 ± 14	29 ± 21	0.9
C9ORF72% (n)	22% (9)	18% (2)	0% (0)	0.3
Mean ALS-TDP stage	2.6 ± 1.2	3.9 ± 0.3*	2.2 ± 1.5	0.003
Intermediate AD or LBD Braak stage IV-VI (%)	5%	0%	45%*	0.001
ACE assessment % (n)	46% (19)	82% (9)	44% (4)^#	0.3
ACE-III total /100	89 ± 10	92 ± 6	76 ± 16*	0.03
Time between cognitive assessment and death (years	1.4 ± 1.3	1.4 ± 0.7	2.8 ± 1.9	0.1

Data is presented as mean ± standard deviation or prevalence. UL upper limb; LL lower limb; LBD lewy body disease; ADNC Alzheimer’s disease neuropathological changes; ACE-III Addenbrooke’s Cognitive Examination third edition [19]. ^#Despite the absence of cognitive scores for 75% of ALS-TDP type SC case with intermediate AD or neocortical LBD (n = 3), a significantly lower overall cognitive performance was identified in this subgroup. *p < 0.05 compared to the two other pathological groups

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