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. 2023 Nov 30;9(6):e200109. doi: 10.1212/NXG.0000000000200109

Table 1.

Pooled Proportion of ALS Cases That Are Familial (fALS), According to Geographic Region and Study Type

graphic file with name NXG-2023-000153t1.jpg

Region Studies fALS cases Total ALS cases Estimate (lower limit, upper limit)
Asia 24 332 7,748 0.04 (0.03, 0.06)a
 Case series 23 331 7,664 0.05 (0.03, 0.06)a
 Population-based 1 1 84 0.01 (NA, NA)
Australasia 4 30 432 0.08 (0.04, 0.16)a
 Case series 3 20 188 0.12 (0.08, 0.17)a
 Population-based 1 10 244 0.04 (NA, NA)
Europe 77 2,783 30,553 0.09 (0.00, 0.71)
 Case series 40 1,848 16,730 0.11 (0.00, 0.71)
 Population-based 37 935 13,823 0.06 (0.05, 0.07)a
Latin America 17 275 2,518 0.06 (0.04, 0.09)a
 Case series 12 237 1,708 0.07 (0.05, 0.12)a
 Population-based 5 38 810 0.05 (0.03, 0.07)a
Middle East and North Africa 10 159 1,832 0.09 (0.01, 0.40)
 Case series 9 154 1,750 0.09 (0.01, 0.40)
 Population-based 1 5 82 0.06 (NA, NA)
North America 28 975 16,989 0.06 (0.05, 0.08)a
 Case series 16 479 6,705 0.08 (0.06, 0.10)a
 Population-based 12 496 10,284 0.05 (0.04, 0.06)a
Sub-Saharan Africa 5 23 514 0.05 (0.03, 0.07)a
 Case series 4 15 329 0.05 (0.03, 0.09)a
 Population-based 1 8 185 0.04 (NA, NA)
Overall 165 4,577 60,586 0.08 (0.00, 0.71)
 Case series 107 3,084 35,074 0.09 (0.00, 0.71)
 Population-based 58 1,493 25,512 0.05 (0.04, 0.06)a

Abbreviation: ALS = amyotrophic lateral sclerosis; NA = not applicable.

a

Random-effects meta-analytic estimates and corresponding 95% confidence intervals are presented (>2 and I2 < 90%). Otherwise, weighted averages and ranges are presented.