Table 1 The clinical and demographical characteristics of the patients under study.
From: A population based study to analyse amyotrophic lateral sclerosis as a multi-step process
ALS patients (tot.216) | ALS patients (%) | ||
|---|---|---|---|
Family History | |||
sALS | 208 | 96,3% | |
fALS | 8 | 3,7% | |
Site of onset | |||
Spinal-onset | 160 | 74,10% | |
Bulbar-onset | 50 | 23,10% | |
Respiratory-onset | 6 | 2,80% | |
M/W ratio | 1,18 | ||
Mean age at onset | 65 (59–70) | ||
Cognitive Features | |||
ALS-FTD | 6 | 2,8% | |
Frontal-lobe dysfunction (no dementia) | 41 | 19% | |
Genetics | |||
Causative mutation | 8 | 3,7% | TARDBP : 3 (1 = G294V; 2 = A382T); FUS:1 (R521G); C9orf72:4 (GGGGCC expansions) |
VUS | 22 | 10,2% |
