TABLE 4.
Clinical characteristics of familial and apparently sporadic cases.
| ALS (n = 118) |
ALS/FTD (n = 37) |
|||
| AS cases | Familial | AS cases | Familial | |
| Gender: M/F (ratio) | 4/5 (0.8) | 53/56 (0.9) | 2/2 (1) | 20/13 (1.5) |
| Bulbar onset (%) | 22 | 41 | 50 | 36 |
| UL onset (%) | 0 | 20 | 25 | 16 |
| LL onset (%) | 78 | 39 | 25 | 42 |
| Dementia onset (%) | – | – | – | 6 |
| Age of onseta | 45.5 ± 6.5b | 58.8 ± 9.9 | 64.6 ± 6.9 | 59.8 ± 7.0 |
| n | 9 | 85 | 4 | 30 |
| Age at deatha | 49.4 ± 7.6 | 62.0 ± 9.5 | 65.8 ± 8.3 | 63.4 ± 6.7 |
| n | 9 | 77 | 3 | 27 |
| Disease durationa | 47.1 ± 21.1 | 39.5 ± 24.8 | 16.9 ± 12.5 | 31.5 ± 18.3 |
| n | 9 | 77 | 3 | 26 |
| ALSFRS-R scorea | 36.0 ± 5.1 | 39.5 ± 5.4 | 46.3 ± 1.5 | 34.7 ± 11.2 |
| n | 8 | 51 | 4 | 22 |
AS, apparently sporadic; M/F, male/female; UL, upper limb; LL, lower limb; ALSFRS-R, amyotrophic lateral sclerosis rating scale-revised. aValue are means ± SD. bvs. familial cases: p < 0.0001.