Case no |
WS |
Age at onset (months) |
Age at onset of ACTH (months) |
Treatment delay |
cHPI (/min) or |
SS on previous EEG if present |
SS on our initial EEG at onset of ACTH |
Relapse of spasms |
Post-treatment HPI |
Post-treatment SS |
Cognitive outcome at final visit |
Age at final visit (months) |
type |
(months) |
dHPI (%) at onset of ACTH |
C1: Cryptogenic cases with age at onset >3 months |
1 |
C |
5.5 |
7.5 |
2 |
10/min (30%) |
- |
Yes |
No |
<3/min |
Yes |
None |
144 |
2 |
C |
6 |
8 |
2 |
8/min (24%) |
- |
Yes |
No |
<3/min |
Yes |
None |
54 |
3 |
C |
6 |
8 |
2 |
9/min (27%) |
- |
Yes |
No |
<3/min |
Yes |
None |
54 |
4 |
C |
7 |
12 |
5 |
21/min (65%) |
- |
Yes |
Yes |
4-10.4/min |
Yes |
Mild |
60 |
C2: Cryptogenic cases with age at onset <3 months |
5 |
C |
1.5 |
4 |
2.5 |
100% |
Yes |
No |
No |
<3/min |
No |
Marked |
8 |
6 |
C |
2 |
16 |
14 |
100% |
Yes |
No |
No |
<3/min |
No |
Severe |
30 |
7 |
C |
2 |
50 |
48 |
100% |
Yes |
No |
No |
<3/min |
No |
Severe |
60 |
S1: Symptomatic severe perinatal hypoxic ischemic encephalopathy |
8 |
S |
9 |
10 |
1 |
100% |
Not reported |
No |
No |
<2/min |
No |
Severe |
36 |
9 |
S |
10 |
11 |
1 |
100% |
Not reported |
No |
No |
<2/min |
No |
Severe |
39 |
S2: Agenesis of corpus callosum |
10 |
S |
2 |
54 |
52 |
100% |
Not reported |
No |
No |
<3/min |
No |
Severe |
66 |
11 |
S |
3 |
72 |
69 |
100% |
Not reported |
No |
No |
<3/min |
No |
Severe |
84 |
S3a: Hypogenesis of corpus callosum with treatment delay > 1year |
12 |
S |
2.5 |
33.5 |
31 |
100% |
Not reported |
No |
No |
<3/min |
No |
Severe |
57.5 |
13 |
S |
5 |
38.5 |
33.5 |
100% |
Not reported |
No |
No |
<3/min |
No |
Severe |
62.5 |
S3b: Hypogenesis of corpus callosum with treatment delay < 1 year |
14 |
S |
2 |
9 |
7 |
60% |
Yes |
No |
Yes |
20-Apr |
No |
Marked |
45 |
15 |
S |
8 |
16 |
8 |
100% |
Yes |
No |
Yes |
26-Apr |
No |
Marked |
64 |
16 |
S |
7 |
18 |
11 |
100% |
Yes |
No |
No |
<3/min |
No |
Severe |
66 |