Table 1.

Clinical features of Innsbruck CACNA1A patients who underwent EEG examination

Family	Genotype	Phenotype	Pt. ID	Age at onset	Age at first EEG	Chronic cerebellar signs	Cerebellar atrophy at MRI	Pathologic findings at first EEG	IEDsa
1	R1667W	FHM1	1-I*	12	56	x	x	x
			1-II	34	42	x	x	x
			1-III	58	58	x	x	x
			1-IV	1	11	x	x	x	x
2	c.3102 + 2 T > C	EA2	2-I	1	28	x	x	x	x
			2-II*	27	37	x	x	x
3	T666M	FHM1	3-I	40	71	x	x
			3-II	5	47	x	x	x
			3-III	13	42	x	x
			3-IV	4	18	x	x
			3-V	7	10	x	x	x
			3-VI	1,5	2		na
			3-VII*	11	11		x	x
			3-VIII*	1,5	3	x	na
4	c.3089 + 2 T > C	EA2	4-I	10	54	x	x
			4-II	45	54	x	x	x
			4-III	2	15	x		x	x
			4-IV	10	20	x	X		x
			4-V	1	26	x		x	x
			4-VI*	1	3		na	x	x
5	R198Q	EA2	5-I	1	22	x	x	x	x
6	S218L	FHM1	6-I	1	6	x	x	x
7	c.959G > A	EA2	7-I	8	48	x	x
8	c.3603dup	EA2	8-I	7	45	x	x
9	G540R	EA2	9-I	1,5	18		x	x
10	C1869R	EA2	10-I*	36	41	x	x	x
11	I239T**	EA2	11-I*	61	68	x	x
			11-II*	55	73	x	x
12	R2248H**	EA2	12-I*	45	41	x	x	x
13	D2173Y**	EA2	13-I*	50	52	x	x	x
14	T666M	FHM1	14-I*	2	41	x	x	x
15	A754V**	EA2	15-I*	1	1				x
16	Q1154X	EA2	16-I*	3	64	x	x	x
17	12/23	SCA6	17-I	55	67	x	x
	13/23		17-II	40	62	x	x
	14/23		17-III	47	48	x	x
18		SCA6	18-I	63	67	x	x
19	8/23	SCA6	19-I	36	39	x	x

IEDs: interictal epileptic discharges

*These patients have been described herein for the first time

**Newly reported CACNA1A variants

^aBoth first EEG and follow-up EEGs are considered