Table 2.
Defining features of ESES and CSWS
| Feature | Options | Number (percentage) CNS | Number (percentage) AES | Number (percentage) total | Potential answers based on available literature |
|---|---|---|---|---|---|
|
| |||||
| Are “ESES” and “CSWS” synonyms? | Yes | 75 (61) | 42 (55.3) | 117 (58.8) | “ESES” and “CSWS” are considered essentially synonyms and interchangeable terms by some authors (Liukkonen, et al. 2010, Peltola, et al. 2011, Tassinari, et al. 2000). |
| No | 32 (26) | 29 (38.2) | 61 (30.7) | ||
| Do not know | 16 (13) | 5 (6.6) | 21 (10.6) | ||
| Skipped question | 14 | 6 | 20 | ||
| Other authors indicate that “ESES” refers to the electroencephalographic pattern present in several syndromes and “CSWS” is the most severe presentation of the electro-clinical syndromes with ESES (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Scholtes, et al. 2005) | |||||
|
| |||||
| Cut-off value for ESES | Any amount | 1 (0.8) | 1 (1.3) | 2 (1) | Some authors consider at least 85% (Liukkonen, et al. 2010, Peltola, et al. 2011, Saltik, et al. 2005, Tassinari, et al. 2000), others a minimum threshold of 75% (Kevelam, et al. 2012), 60% (Inutsuka, et al. 2006), or 25% (Van Hirtum-Das, et al. 2006). |
| At least 50% | 33 (26.8) | 28 (37.3) | 61 (30.8) | ||
| At least 85% | 76 (61.8) | 38 (50.7) | 114 (57.6) | ||
| Not sure | 13 (10.6) | 8 (10.7) | 21 (10.6) | ||
| Skipped question | 13 | 6 | 19 | ||
| Additionally, it has been noted that the cut-off value for ESES changes over time (Sánchez Fernández, et al. 2012c) | |||||
|
| |||||
| Cut-off value for CSWS | Any amount | 1 (0.8) | 1 (1.3) | 2 (1) | If ESES and CSWS are considered synonyms (Liukkonen, et al. 2010, Peltola, et al. 2011, Tassinari, et al. 2000), then the threshold for CSWS would be the same as in ESES. |
| At least 50% | 27 (22.3) | 28 (36.8) | 55 (27.9) | ||
| At least 85% | 69 (57) | 36 (47.4) | 105 (53.3) | ||
| Not sure | 24 (19.8) | 11 (14.5) | 35 (17.8) | ||
| Skipped question | 13 | 6 | 19 | If CSWS is considered an electro-clinical syndrome, not an EEG pattern (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Scholtes, et al. 2005), then the thresholds should be applied to the definition of ESES, not to the definition of CSWS. | |
|
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| Cut-off values in ESES are | Mandatory | 55 (44.7) | 35 (46.1) | 90 (45.2) | The cut-off value could be considered mandatory (Tassinari, et al. 2000) or typical, but not required (Nieuwenhuis&Nicolai 2006). |
| Typical, not required | 50 (40.7) | 32 (42.1) | 82 (41.2) | ||
| Not sure | 18 (14.6) | 9 (11.8) | 27 (13.6) | ||
| Skipped question | 13 | 6 | 19 | ||
| The cut-off value for ESES may also change over time(Sánchez Fernández, et al. 2012c). | |||||
|
| |||||
| Cut-off values in CSWS are | Mandatory | 47 (38.5) | 31 (40.8) | 78 (39.4) | If ESES and CSWS are considered synonyms (Liukkonen, et al. 2010, Peltola, et al. 2011, Tassinari, et al. 2000), then the threshold would be the same as in ESES. |
| Typical, not required | 49 (40.2) | 35 (46.1) | 84 (42.4) | ||
| Not sure | 26 (21.3) | 10 (13.2) | 36 (18.2) | ||
| Skipped question | 13 | 6 | 19 | ||
| If CSWS is considered an electro-clinical syndrome, not an EEG pattern (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Scholtes, et al. 2005), then the thresholds should be applied to the definition of ESES, not to the definition of CSWS. | |||||
|
| |||||
| For the diagnosis of ESES cognitive and/or language regression is | Mandatory | 24 (19.8) | 14 (18.9) | 38 (19.5) | If ESES is considered an electro-clinical syndrome (Liukkonen, et al. 2010, Peltola, et al. 2011, Tassinari, et al. 2000), then the regression would be required. |
| Typical, not required | 74 (61.2) | 46 (62.2) | 120 (61.5) | ||
| Not relevant for diagnosis | 14 (11.6) | 11 (14.9) | 25 (12.8) | ||
| Not sure | 9 (7.4) | 3 (4.1) | 12 (6.2) | ||
| Skipped question | 14 | 8 | 22 | If ESES is considered an electrical pattern (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Scholtes, et al. 2005), then the regression should be applied to the definition of CSWS, not to the definition of ESES | |
|
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| For the diagnosis of CSWS cognitive and/or language regression is | Mandatory | 32 (26.7) | 21 (28.4) | 53 (27.3) | The literature suggests that cognitive and/or language regression is required for the diagnosis of the clinical encephalopathy (Loddenkemper, et al. 2011, Sánchez Fernández, et al. in press, Tassinari, et al. 2000) |
| Typical, not required | 64 (53.3) | 49 (66.2) | 113 (58.3) | ||
| Not relevant for diagnosis | 11 (9.2) | 2 (2.7) | 13 (6.7) | ||
| Not sure | 13 (10.8) | 2 (2.7) | 15 (7.7) | ||
| Skipped question | 14 | 8 | 22 | ||
|
| |||||
| How do you diagnose CSWS? | Clinical assessment only | 0 (0) | 0 (0) | 0 (0) | If CSWS is considered an electroencephalographic pattern (Liukkonen, et al. 2010, Peltola, et al. 2011, Tassinari, et al. 2000), then the diagnosis of CSWS would be based on EEG assessment only. |
| EEG assessment only | 41 (33.1) | 21 (27.6) | 62 (31) | ||
| MRI assessment only | 0 (0) | 0 (0) | 0 | ||
| Clinical and EEG assessment | 77 (62.1) | 50 (65.8) | 127 (63.5) | ||
| All of the above | 6 (4.8) | 5 (6.6) | 11 (5.5) | ||
| Skipped question | 13 | 6 | 19 | ||
| If CSWS is considered an electro-clinical syndrome (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Scholtes, et al. 2005), then it should be diagnosed with clinical and EEG assessments. | |||||
|
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| Segment of EEG used for calculation of the epileptiform activity | Whole night (all phases) | 35 (27.6) | 17 (21.8) | 52 (25.4) | Some authors calculate epileptiform activity during the complete nocturnal sleep duration (Tassinari, et al. 2000), others during the first 30 minutes of non-REM sleep stages of the first and last sleep cycles (Aeby, et al. 2005), at least one sleep-wake cycle (Saltik, et al. 2005), the whole-night, first non-REM sleep cycle or nap EEG (Inutsuka, et al. 2006) or the first five minutes of non-REM sleep (Sánchez Fernández, et al. 2012c). |
| Whole night (non-REM) | 51 (40.2) | 34 (43.6) | 85 (41.5) | ||
| Comparison wake/sleep | 15 (11.8) | 10 (12.8) | 25 (12.2) | ||
| Variable | 4 (3.2) | 3 (3.9) | 7 (3.4) | ||
| Unknown | 15 (11.8) | 4 (5.1) | 19 (9.3) | ||
| Other selected samples | 7 (5.5) | 10 (12.8) | 17 (8.3) | ||
| Skipped question | 17 | 14 | 31 | ||
|
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| Quantification of spike-wave activity | Bilateral and synchronous only | 15 (12.2) | 11 (14.5) | 26 (13.1) | There is currently insufficient evidence to support that unilateral discharges should be quantified differently from bilateral discharges (Bureau 1995, Kramer, et al. 2009, Liukkonen, et al. 2010, Saltik, et al. 2005, Sánchez Fernández, et al. 2012b, Van Hirtum-Das, et al. 2006). |
| Bilateral even if not synchronous | 25 (20.3) | 22 (29) | 47 (23.6) | ||
| Unilateral equivalent to bilateral | 50 (40.7) | 32 (42.1) | 82 (41.2) | ||
| Unknown | 33 (26.8) | 11 (14.5) | 44 (22.1) | ||
| Skipped question | 14 | 6 | 20 | ||
|
| |||||
| Method of quantification of epileptiform activity | Percentage of one-second bins occupied by spike-waves | 57 (48.3) | 43 (58.9) | 100 (52.4) | Some authors do not specify the exact method of calculation (Caraballo, et al. 2008, Inutsuka, et al. 2006, Kramer, et al. 2009, Liukkonen, et al. 2010, Saltik, et al. 2005, Tassinari, et al. 2000). |
| Total number of spike-waves per unit of time | 53 (44.9) | 25 (34.3) | 78 (40.8) | ||
| Other | 8 (6.8) | 5 (6.9) | 13 (6.8) | ||
| Skipped question | 27 | 14 | 41 | ||
| Other authors used the percentage of one-second bins occupied by at least one spike-wave (Aeby, et al. 2005, Sánchez Fernández, et al. 2012c) or the total number of spike-waves per unit of time (Sánchez Fernández, et al. 2012c). | |||||
|
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| Time of occurrence of the neuropsychological regression in CSWS | Infancy (0-2 years) | 7 (5.7) | 3 (4) | 10 (5) | Regression usually occurs in childhood (Loddenkemper, et al. 2011, Nickels&Wirrell 2008, Nieuwenhuis&Nicolai 2006, Sánchez Fernández, et al. in press, Tassinari, et al. 2000) |
| Childhood (3-12 years) | 107 (86.3) | 66 (86.8) | 173 (86.5) | ||
| Adolescence (13-18 years) | 0 (0) | 1 (1.3) | 1 (0.5) | ||
| Adulthood (19-59 years) | 0 (0) | 0 (0) | 0 | ||
| Elderly years (60 or more years) | 0 (0) | 0 (0) | 0 | ||
| Unknown | 10 (8.1) | 6 (7.9) | 16 (8) | ||
| Skipped question | 13 | 6 | 19 | ||
Legend: CSWS: Continuous spikes and waves during sleep. EEG: Electroencephalogram. ESES: Electrical status epilepticus in sleep. MRI: Magnetic resonance imaging. REM: Rapid eye movement.