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

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

  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 onseta (years) 64.5 (32–86) 62.0 (32–86) 65.5 (50–79) 72.0* (59–82) 0.023
Survival timea (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 %)  
 othersb 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 %)  
 othersc 29 (30 %) 18 (29 %) 8 (36 %) 3 (27 %)  
  1. ALS amyotrophic lateral sclerosis
  2. aData are expressed as median (range)
  3. 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)
  4. cInfections, gastrointestinal bleeding or sudden death
  5. *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)