Table 1.
Studies used for analysis, including CAE diagnostic criteria, seizure-free outcomes, and dropout rates.
| Publ Yeara |
First Author |
N | Age at Onset, years |
EEG 3 Hz SWDe |
Nl EEG Backgrounde |
≥1 Sz per daye |
Sz Duration 5–30 se |
Abrupt Sz On/ Offsete |
No GTC or Other Szse |
No Abnl Motor Activitye |
No Other Disorders,f |
Seizure Free Allg |
Seizure Free On Medsg |
Seizure Free Off Medsg |
Dropout Rateh |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1945 | Lennox | 40 | <20 | + | + | + | + | + | + | + | + | 28% | 28% | ||
| 1955 | Janz | 88 | 4 – 14 | + | − | − | − | − | − | − | − | 16% | |||
| 1957 | Paal | 38 | 3 – 9 | + | − | − | − | − | − | − | − | 36% | 36% | 12% | |
| 1962 | Holowach | 144 | 2 – 9 | + | − | + | + | + | + | + | − | 43% | 63% | 22% | |
| 1963 | Currier | 32 | 3 −17 (7.5)b | + | − | + | − | − | + | + | + | 38% | 38% | 0% | |
| 1963 | Hertoft | 50 | 2 – 12 (5.9)c | + | + | − | − | − | + | − | + | 38% | 38% | 12% | |
| 1965 | Gordon | 45 | 1 – 14 | + | − | − | − | − | + | − | − | 33% | 33% | 4% | |
| 1965 | Livingston | 117 | 2 – 15 | + | − | + | + | + | − | − | − | 78% | 78% | ||
| 1966 | Gibberd | 139 | 5 – 10 | + | − | − | + | + | − | + | − | 41% | 41% | ||
| 1976 | Sato | 48 | 2 – 13 (6.8)c | + | − | − | − | − | − | + | − | 35% | 35% | 8% | |
| 1977 | Ohtahara | 9 | 1 – 15 | + | − | − | − | − | − | − | − | 100% | 100% | ||
| 1978 | Okuma | 22 | childhood | + | − | − | − | − | − | + | − | 73% | 50% | ||
| 1979 | Annegers | 33 | + | − | − | − | − | − | − | − | 60% | 60% | 34% | ||
| 1980 | Kawai | 43 | childhood | + | − | − | − | − | + | + | − | 77% | 77% | 12% | |
| 1983 | Sato | 39 | 2 – 13 (6.8)c | + | − | − | − | − | + | + | − | 64% | |||
| 1983 | Losieau | 69 | 3 – 14 | + | − | − | − | − | + | + | + | 44% | 44% | ||
| 1984 | Wolf | 294 | 4 – 12 | + | − | − | − | − | − | − | − | 50–65% | 50–65% | ||
| 1985 | Dieterich | 184 | 8 – 15 (6.5)d | + | − | − | − | − | − | − | − | 44–96% | 44–96% | ||
| 1991 | Olsson | 56 | 3 – 11 | + | − | − | − | − | + | + | − | 82% | 82% | ||
| 1995 | Loiseau | 39 | 3 – 10 | + | − | + | − | − | + | − | − | 95% | 95% | 41% | |
| 1996 | Wirrell | 65 | 1 – 14 | + | + | + | − | − | + | − | + | 74% | 74% | 11% | |
| 2004 | Trinka | 64 | <10 (7)c | + | + | + | − | + | − | + | + | 60% | 20% | ||
| 2004 | Coppola | 29 | 3 – 13 (7.5)c | + | + | − | − | − | + | + | + | 53–68% | 53–68% | 32% | |
| 2005 | Grosso | 84 | pre-puberty | + | + | + | − | + | − | − | + | 90% | 90% | 15% | |
| 2008 | Verrotti | 21 | 5 – 13 (8.9)c | + | + | + | + | + | + | + | + | 67% | 67% | 52% | |
| 2008 | Holmes | 46 | 3 – 13 (7.3)c | + | − | − | − | − | + | − | + | 46% | 46% | 43% | |
| 2009 | Callenbach | 43 | 1 – 16 (5.4)c | + | + | + | + | + | + | + | + | 70–95% | 70–95% | 9% | |
| 2012 | Hwang | 89 | 4 – 9.8(6.4)c | + | + | + | + | + | + | + | + | 64–87% | 64–87% | 25% | |
| 2013 | Glauser | 446 | 2.5–13(7.4)b | + | + | + | + | + | + | + | + | 50–86% | 50–86% |
Full references included in Supplementary Table S1.
Median.
Mean.
Mode.
These criteria were used (+) or not used (−) for the definition of CAE in each study.
Disorders considered in different studies included psychiatric disease, autism, pervasive development disorders and structural brain lesions, as well as any cardiovascular, hepatic, renal, gynecologic, musculoskeletal, or endocrine disorders.
Seizure freedom was defined as being completely without seizures over the length of follow-up. Seizure Free All included outcome for all patients regardless of treatment; Seizure Free On Meds, outcome for patients while still on medications; Seizure Free Off meds, outcome for patients after medications were discontinued.
Dropout rate was defined as percentage of subjects initially in the study who were lost to follow-up.
Abbreviations: Publ, Publication. N, number of patients included. EEG, electroencephalogram. SWD, spike-wave discharge. Nl, normal. Sz, seizure. GTC, generalized tonic-clonic seizure. Abnl, abnormal.