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. Author manuscript; available in PMC: 2017 Jul 30.
Published in final edited form as: Am J Med Genet A. 2014 Nov 26;167A(2):331–344. doi: 10.1002/ajmg.a.36864

TABLE II.

Epilepsy parameters for individuals with Angelman syndrome, late-adolescence through adulthood

Full Cohort
% (n)
Female
odds ratio
21–25y
odds ratio
26–30y
odds ratio
31–50y
odds ratio
UBE3A
odds ratio
UPD
odds ratio

History of seizures 94% (109) 0.437 IS (100%, 29) 0.335 0.775 IS (100%, 9) 0.111*
Current seizures
 Seizure free/off AEDs a 12% (103) 0.937 0.786 1.197 2.470 1.584 0.792
 Seizure free/AED Tx a 48% (103) 0.844 0.525 0.238* 0.202* 0.945 1.970
 Active seizures b 41% (103) 1.154 2.345 4.102* 6.701* 1.853 0.494
 Current AED ≥2 45% (103) 0.676 2.241 1.445 1.556 0.625 0.750
 ≥ 2 semiologies c 12% (103) 0.497 1.418 2.966 1.832 IS (0%, 9) 0.886
Frequency
 Monthly d 29% (103) 1.021 3.051 4.558* 7.802* 1.214 0.810
 Daily 16% (103) 2.266 2.11 10.328* 6.883* 0.567 IS (0%, 8)
Seizure severity
 Modified E-chess score δ 4.65 (SD: 2.754, n=103) 0.055 0.807 1.706** 1.688** -0.139 -0.569
 Score > 6 26% (103) 0.817 1.464 3.032 2.28 0.857 1.000
Current medications
 Valproate 39% (103) 0.673 1.614 2.234 1.493 0.424 0.890
 Clonazepam 17% (103) 1.033 1.232 0.431 0.277 IS (0%, 9) 0.500
 Lamotrigine 17% (103) 1.809 2.080 0.622 0.855 0.567 0.648
 Levetiracetam 13% (103) 0.588 3.948 0.955 9.329* 0.775 IS (0%, 8)
 Ethosuximide 9% (103) 0.49 0.198 0.279 0.383 1.675 1.914
 Topiramate 9% (103) 0.264 0.258 0.653 0.477 3.943 1.533
Lifetime seizure severity
 AEDs >2 lifetime 63% (103) 1.782 2.020 1.188 1.577 0.283 0.942
 AEDs >3 lifetime 45% (103) 1.107 2.455 2.008 1.754 0.140 0.671
 Seizure onset <3y 68% (103) 0.847 1.555 2.076 2.777 0.206* 1.235
Most severe age δ 7.53 (SD: 7.035, n=100) 1.192 1.772 −0.111 4.041 −2.536 1.339
 0–5 y 52% (100) 0.472 0.863 0.667 1.827 2.000 1.000
 6–10 y 25% (100) 0.875 0.568 1.071 0.154 0.964 0.482
 11–15 y 12% (100) 4.519* 0.922 2.956 0.602 IS (0%, 9) 0.766
 16–20 y 6% (100) 6.19 2.827 IS (0%, 18) IS (0%, 15) 2.062 2.357
 21–25 y 5% (62) 2.370 -- -- -- IS (0%, 5) 7.167
 26–30 y 0% (33) -- -- -- -- -- --
 >31 y 20% (15) 0.500 -- -- -- IS (0%, 2) IS (0%, 1)

Age sub-groups compared with the 16–20y cohort. Genotype sub-groups compared with the Del cohort.

a

cohort of individuals with history of seizures who have not had an event in 1 year or more.

b

includes 3 individuals who were not being treated with an AED.

c

reported to have 2 or more types of seizure, i.e. events that look different to caregiver.

d

events occurring at least monthly. δ. regression coefficient listed for each covariate.

*

statistically significant odds ratio (α = 0.05).

**

statistically significant regression coefficient (α=0.05). IS: infinite solution (%, n), AED: anti-epileptic drugs, Tx: treatment, y: years.