Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes

Table 1 Demographics and clinical features in three types of sporadic ALS

	All	Type 1	Type 2a	Type 2b	P-value
	(n = 96)	(n = 63)	(n = 22)	(n = 11)
Sex (male: female)	58:38	38:25	13:9	7:4	0.968
Age at onset^a (years)	64.5 (32–86)	62.0 (32–86)	65.5 (50–79)	72.0* (59–82)	0.023
Survival time^a (months)	22 (6–204)	33 (7–204)	20 (6–64)	12** (9–24)	<0.0001
Initial symptoms					<0.0001
limb	61 (64 %)	50 (79 %)***	7 (32 %)	4 (36 %)
bulbar	32 (33 %)	13 (21 %)	13 (59 %)	6 (55 %)
others^b	3 (3 %)	0	2 (9 %)	1 (9 %)
Cognitive impairment	15 (16 %)	1 (2 %)****	7 (32 %)	7 (64 %)	<0.0001
Cause of death					0.793
respiratory failure	67 (70 %)	45 (71 %)	14 (64 %)	8 (73 %)
others^c	29 (30 %)	18 (29 %)	8 (36 %)	3 (27 %)

ALS amyotrophic lateral sclerosis
^aData are expressed as median (range)
^bThree patients developed cognitive impairment or character change before the appearance of motor symptoms. Motor symptoms became evident seven months after disease onset in one patient (Type 2a), and within a month in the other two patients (Type 2a and Type 2b, respectively)
^cInfections, gastrointestinal bleeding or sudden death
*P = 0.024 vs. Type 1 (Kruskal-Wallis test with post-hoc Steel-Dwass test), **P <0.0001 vs. Type 1 and P = 0.003 vs. Type 2a (Kaplan-Meier method and log-rank test), ***P <0.0001 vs. Type 2a and P <0.01 vs. Type 2b (Fisher’s exact test with Bonferroni-corrected multiple comparison test), ****P <0.001 vs. Type 2a and P <0.00001 vs. Type 2b (Fisher’s exact test with Ryan’s multiple comparison test)

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