Table 4:
Development Score for subjects >=1 years of age at last visit
| Development Score Mean (95% CI) | Adjusted Association* | ||||
|---|---|---|---|---|---|
| Variables | Yes | No | Adjusted Pvalue | R2 | Cohen’s f2 |
| Hypsarrhythmia | 2.2 (1.5,2.8) | 3.2 (2.6,3.8) | 0.0251 | 0.23 | 0.04 |
| Epileptic Spasms | 2.5 (2.1,3.0) | 3.6 (2.5,4.7) | 0.0926 | 0.23 | 0.04 |
| Tonic | 2.6 (2.1,3.2) | 2.9 (2.2,3.7) | 0.7612 | 0.20 | 0.00 |
| HTSS | 2.7 (2.0,3.4) | 2.7 (2.2,3.3) | 0.4590 | 0.21 | 0.01 |
| HTSS like | 2.8 (1.9,3.7) | 2.7 (2.2,3.3) | 0.9062 | 0.20 | 0.00 |
| Any HTSS | 2.7 (2.2,3.3) | 2.8 (2.0,3.5) | 0.0575 | 0.24 | 0.05 |
| GTC | 3.1 (2.2,3.9) | 2.6 (2.0,3.1) | 0.6566 | 0.20 | 0.00 |
| Myoclonic | 2.6 (1.8,3.3) | 2.8 (2.3,3.4) | 0.3130 | 0.21 | 0.01 |
| Clonic | 2.9 (2.0,3.8) | 2.7 (2.2,3.2) | 0.8099 | 0.20 | 0.00 |
| Atonic | 4.0 (2.1,5.9) | 2.6 (2.1,3.1) | 0.2165 | 0.22 | 0.03 |
| Absence | 2.5 (−16.6,21.6) | 2.7 (2.3,3.2) | 0.4113 | 0.21 | 0.01 |
| Focality | 3.1 (2.3,3.9) | 2.6 (2.0,3.1) | 0.2960 | 0.21 | 0.01 |
| Any seizure free period | 3.2 (2.5,3.8) | 2.4 (1.8,3.0) | 0.0279 | 0.25 | 0.07 |
| Cortical Visual Impairment | 2.2 (1.8,2.7) | 4.2 (3.3,5.2) | 0.0001 | 0.34 | 0.21 |
| Age at Last Visit | - | - | <0.0001 | 0.20 | |
Analysis of binary variables association with 7-point CDKL5 Developmental Score adjusted for age at last visit. R2 and Cohen’s f2 effect size are provided as a demonstration of the effect size with f2>=0.02,0.15,0.35 representing small, medium, and large effect sizes respectively23. HTSS – Hypermotor Tonic Spasm Sequence (including HTSS-like as referenced in the text); GTC – Generalized Tonic Clonic; focality could represent either a focal seizure or one of the other seizure types with focal features (for example an asymmetric tonic seizure was marked as both focality and tonic seizure).