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Table 1 Clinical, cognitive and genetic data

From: Encephalopathy in children with Dravet syndrome is not a pure consequence of epilepsy

Clinical and cognitive characteristics SCN1A+ SCN1A- Total
N 58 9 67
Sex: boys N (%) 33 (56.9) 6 (66.7) 39 (58.2)
Age in years: mean (SD)    
  at last follow-up 9.1 (5.3) 8.7 (4.6) 9.0 (5.2)
  at first examination 4.0 (5.4) 4.0 (2.7) 4.0 (5.2)
Follow-up duration 5.1 (4.2) 4.7 (5.6) 5.0 (4.4)
Family history for seizures: N (%) 21 (36.2) 4 (44.4) 25 (37.3)
  Dravet Syndrome 3 (5.2) 0 3 (4.5)
  Febrile seizures 14 (24.1) 3 (33.3) 17 (25.4)
  Febrile seizures plus 2 (3.4) 0 2 (3.0)
  Epilepsy 8 (13.8) 1 (11.1) 9 (13.4)
Age at first seizure: N (%)    
  0-3 months 6 (10.3) 1 (11.1) 7 (10.4)
  3-6 months 29 (50.0) 5 (55.6) 34 (50.7)
  6-9 months 18 (31.0) 1 (11.1) 19 (28.4)
  > 9 months 5 (8.3) 2 (22.2) 7 (10.5)
Type of seizures: N (%)    
  Tonic-clonic 51 (87.9) 8 (88.9) 59 (88.1)
  Absences 32 (55.2) 5 (55.6) 37 (55.2)
  Myoclonic 29 (50.0) 6 (66.7) 35 (52.2)
  Tonic 7 (12.1) 4 (44.4) 11 (16.4)
  Clonic 38 (65.5) 7 (77.8) 45 (67.2)
  Partial 23 (39.7) 3 (33.3) 26 (38.8)
Sensitivity to fever 51 (87.9) 7 (77.8) 58 (86.6)
Photosensitivity 11 (19.0) 4 (44.4) 15 (22.4)
Status Epilepticus (SE)    
  Present: N (%) 46 (79.3) 6 (66.7) 52 (77.6)
  Age at first SE: N (%)    
  0-3 months 0 1 (16.7) 1 (1.9)
  3-6 months 12 (26.1) 3 (50.0) 15 (28.8)
  6-9 months 14 (30.4) 0 14 (26.9)
  9-12 months 7 (15.2) 1 (16.7) 8 (15.4)
  >12 months 13 (28.3) 1 (16.7) 14 (26.9)
Neurological features: N (%)    
  Ataxia/Gait disorders* 45 (77.5) 4 (44) 49 (73)
  Pyramidal signs 4 (6.9) 1 (11.1) 5 (7.5)
Last DQ/IQ    
  Mean (SD) 53.4 (20.9) 42.9 (11.3) 51.9 (20.2)
  Range 30-105 30-65 30-105
  Age at last DQ/IQ (years)    
  Mean (SD) 6.0 (4.6) 8.7 (3.7) 6.4 (4.5)
  Range 1.1-23.9 4.6-16.1 1.1-23.9
Semi-quantitative psychomotor score    
  (SQPS)    
  Mild 33 (57.9) 5 (55.6) 38 (57.6)
  Moderate 9 (15.8) 4 (44.4) 13 (19.7)
  Severe 15 (26.3) 0 15 (22.7)
  1. N = number, SD = standard deviation, DQ/IQ = developmental/intellectual quotient. *p = 0.017.