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 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 %) |
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)