Table 1.

Clinical summaries of 22 investigated FAME families

Family	Nationality	Total affected	Mean onset [range]	Myoclonus/CT	TCS	Focal Sz	References
1	Australian/New Zealand of European ancestry	55	18.6 y [4–59,60 y]	55/55 (100%)	8/55 (15%)	2/55 (4%)	2
2	Italian	2	15–25 y	2/2 (100%)	2/2 (100%)	0/2 (0%)
3	Italian	4	2–18y	4/4 (100%)	4/4 (100%)	2/4 (50%)	12
4	Italian	11	22.3 y [12–49,50 y]	11/11 (100%)	11/11 (100%)	3/11 (27%)	4, 17
5	Italian	25	26.6 y [5–39,40 y]	25/25 (100%)	10/25 (40%)	0/25 (0%)	38
6	Italian	12 (3 studied)	12 y [8–17,18 y]	11/12 (91.6%)	6/12 (50%)	1/12 (8.3%)	9, 39
7	Italian	4	22.75 y [10–35,36 y]	4/4 (100%)	3/4 (75%)	0/4 (0%)
8	Italian	10 (6 studied)	18.5 y [17–19,20 y]	6/6 (100%)	4/6 (66.6%)	0/6 (0%)	40
9	Italian	13 (11 studied)	17 y [12–21,22 y]	11/11 (100%)	9/11 (81.1%)	0/14 (0%)	41
10	Italian	16 (14 studied)	15.8 y [13–19,20 y]	14/14 (100%)	10/14 (71%)	0/14 (0%)	41
11	Italian	10 (5 studied)	15.5 y [13–17,18 y]	5/5 (100%)	4/5 (80%)	0/5 (0%)	42
12	Italian	21 (17 studied)	39.2 y [24–55,56 y]	17/17 (100%)	13/17 (76.4%)	0/17(0%)	43
13	Italian	3	17.7 y [12–22,23 y]	3/3 (100%)	1/3 (33%)	0/3 (0%)	42, 44
14	Italian	3	16.3 y [15–18 y]	3/3 (100%)	3/3 (100%)	0/3 (0%)	44, 45
15	Italian	4	30.3 y [18–48,49 y]	4/4 (100%)	3/4 (75%)	2/4 (50%)	17
16	Iraqi of Sephardic Jewish ancestry	15 (10 studied)	21 y [12–31,32 y]	10/10 (100%)	4/10 (40%)	2/10 (20%)
17	Israeli of Sephardic Jewish ancestry	2	21 y [21 y]	2/2 (100%)	2/2 (100%)	0/2 (0%)
18	South African of European ancestry	24 (15 studied)	15.8 y [11–19,20 y]	15/15 (100%)	7/15 (47%)	1/15 (7%)	46
19	French/ Spanish	13	41 y [30–59,60 y]	13/13 (100%)	8/13 (62%)	0/13 (0%)	13, 47
20	French	7 (2 studied)	20 y (n = 1) Childhood (n = 1)	2/2 (100%)	1/2 (50%)	0/2 (0%)	9
21	Syrian	1	20 y	1/1 (100%)	1/1 (100%)	0/1 (0%)
22	Italian	11 (10 studied)	25.1 y [14–39,40 y]	9/10 (90%)a	4/10 (40%)	1/10 (10%)

CT cortical tremor, Focal Sz focal seizures, TCS tonic-clonic seizures, y years, n number of individuals

^aOne family member last evaluated at 9 years of age