Table 2

Distribution of FALS and clinical characteristics considering the number of generations with ALS

MonoG (49)BiG (152)MultiG (34)P value
No of cases
 N=242 (85.7%)105 (69.1%)
 N≥3 (%)7 (14.3%)47 (30.9%)34 (100.0%)
Age of onset in the last generation <0.0001
 Mean (years)63.6±10.257.3±11.750.2±11.5
 n4814734
Site of onset0.59
 Bulbar (%)24.528.818.2
 UL (%)34.729.527.3
 LL (%)40.841.854.5
Duration of the disease0.84
 Median (months)30.3±34.430.1±33.830.0±62.1
 Range7.0–179.24.0–209.25.9–221.2
 n279923
  • 2: with two cases, ≥3: FALS with at least three ALS cases.

  • ALS, amyotrophic lateral sclerosis; BiG, Two generations with ALS cases; FALS, familial amyotrophic lateral sclerosis; MonoG, One generation with ALS cases; MultiG, at least 3three generations with ALS cases.