Table 3

Demographic and clinical details of amyotrophic lateral sclerosis incident cases for the 2 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 (years) (mean±SD)65.9±10.861.9±12.662.5±11.563.2±15.366.5±11.765.4±10.664.4±12.3
Time to diagnosis (days) (median (IQR))240 (150–390)255 (159–372)271 (180–540)280 (180–495)270 (180–390)389 (267–584)270 (175–421)
Sex (males) (n (%))144 (54.3)104 (53.6)81 (62.3)74 (48.1)125 (54.1)26 (48.2)554 (53.9)
Familial ALS (n (%))8 (3.2)NA4 (2)7 (4.6)NANANA
El Escorial Classification 98 (n (%))
 Definite119 (44.9)92 (47.4)29 (22.3)90 (58.4)116 (50.2)3 (5.6)449 (43.7)
 Probable79 (29.8)54 (27.8)44 (33.9)37 (24.0)0 (0)17 (31.5)231 (22.5)
 Possible67 (25.3)33 (17.0)45 (34.6)10 (6.5)43 (18.6)10 (18.5)208 (20.2)
 Suspected0 (0)15 (7.7)12 (9.2)7 (4.6)37 (16.0)24 (44.4)95 (9.2)
 Other0 (0)0 (0)0 (0)10 (6.5)35 (15.2)0 (0)45 (4.4)
Site of onset (n (%))
 Spinal182 (68.7)132 (68.0)96 (73.9)76 (49.4)116 (50.2)32 (59.3)634 (61.7)
 Bulbar83 (31.3)54 (27.8)25 (19.2)53 (34.4)41 (17.8)22 (40.7)278 (27.0)
 Generalised*0 (0)4 (2.1)9 (6.9)19 (12.3)0 (0)0 (0)32 (3.1)
 Unspecified0 (0)4 (2.1)0 (0)6 (3.9)74 (32.0)0 (0)84 (8.2)
  • * Generalised onset ALS is classified as bulbar onset ALS by convention.

  • NA, prevalence of familial cases was not available.

  • ALS, amyotrophic lateral sclerosis.