Table 3.

Prevalence of variants in ALS genes by family categorization

	Sporadic	All FALS	Definite	Probable A	Probable B	Possible
Total Subjects	349	42	16	3	18	5
ANG	2	1	1	-	-	-
ATXN2	11	1	-	-	1	-
C9ORF72	21	14	7	1	6	-
DAO	2	1	1	-	-	-
DCTN1	10	3	2	-	-	1
EWSR1	2	-	-	-	-	-
FIG4	5	-	-	-	-	-
FUS	3	2	1	-	-	1
OPTN	2	-	-	-	-	-
SETX	29	3	1	-	2	-
SOD1	4	6	4	-	2	-
SQSTM1	9	1	-	-	1	-
TAF15	2	-	-	-	-	-
TARDBP	2	1	1	-	-	-
UBQLN2	-	-	-	-	-	-
VAPB	6	-	-	-	-	-
VCP	-	1	-	1	-	-
Total Variants	110	34	18	2	12	2
Subjects with any variant	97	27	13	2	10	2
% Subjects with variants	27.8%	64.3%	81.3%	66.7%	55.6%	40.0%

Familial ALS categories were assigned according to recently proposed criteria.^13,38

Definite FALS (38% of families): at least two first- or second-degree relatives with ALS;

Probable A FALS (7% of families): one first-degree relative with ALS;

Probable B FALS (43% of families): one second-degree relative with ALS;

Possible FALS (12% of families): one distant relative with ALS;

Sporadic ALS (89% of entire cohort): all subjects not meeting criteria for any FALS category.