Table 3.
Demographic and clinical details of ALS incident cases for the two year period, 1998 - 1999, classified by region.
| Country (Region) | ||||||||
|---|---|---|---|---|---|---|---|---|
| Piemonte (N=265) | Lombardy (N=194) | Puglia (N=130) | Ireland (N=154) | Scotland (N=231) | Lancashire (N=54) | Total (N=1028) | ||
| Age (Mean ± SD) | 65.9±10.8 | 61.9±12.6 | 62.5±11.5 | 63.2±15.3 | 66.5±11.7 | 65.4±10.6 | 64.4±12.3 | |
| Median Time, days, to Diagnosis (IQR) | 240 (150-390) | 255 (159-372) | 271 (180-540) | 280 (180-495) | 270 (180-390) | 389 (267-584) | 270 (175-421) | |
| Sex (Males) | 144 (54.3%) | 104 (53.6%) | 81 (62.3%) | 74 (48.1%) | 125 (54.1%) | 26 (48.2%) | 554 (53.9%) | |
| Familial ALS (%) | 8(3.2%) | Na | 4 (2%) | 7 (4.6%) | Na | Na | Na | |
| EEC 98 | ||||||||
| Definite | 119 (44.9%) | 92 (47.4%) | 29 (22.3%) | 90 (58.4%) | 116 (50.2%) | 3 (5.6%) | 449 (43.7%) | |
| Probable | 79 (29.8%) | 54 (27.8%) | 44 (33.9%) | 37 (24.0%) | 0 (0%) | 17 (31.5%) | 231 (22.5%) | |
| Possible | 67 (25.3%) | 33 (17.0%) | 45 (34.6%) | 10 (6.5%) | 43 (18.6%) | 10 (18.5%) | 208 (20.2%) | |
| Suspected | 0 (0%) | 15 (7.7%) | 12 (9.2%) | 7 (4.6%) | 37 (16.0%) | 24 (44.4%) | 95 (9.2%) | |
| Other | 0 (0%) | 0 (0%) | 0 (0%) | 10 (6.5%) | 35 (15.2%) | 0 (0%) | 45 (4.4%) | |
| Site of onset | ||||||||
| Spinal | 182 (68.7%) | 132 (68.0%) | 96 (73.9%) | 76 (49.4%) | 116 (50.2%) | 32 (59.3%) | 634 (61.7%) | |
| Bulbar | 83 (31.3%) | 54 (27.8%) | 25 (19.2%) | 53 (34.4%) | 41 (17.8%) | 22 (40.7%) | 278 (27.0%) | |
| Generalized† | 0 (0%) | 4 (2.1%) | 9 (6.9%) | 19 (12.3%) | 0 (0%) | 0 (0%) | 32 (3.1%) | |
| Unspecified | 0 (0%) | 4 (2.1%) | 0 (0%) | 6 (3.9%) | 74 (32.0%) | 0 (0%) | 84 (8.2%) | |
EEC 98 = El Escorial Classification
†
Generalized-onset ALS is classified as bulbar-onset ALS by convention
Na: prevalence of familial cases was not available