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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 VIII.

Challenging behavior 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

Challenging behavior
 Aggressive towards others a 72% (109) 1.302 0.377 0.222* 0.284 1.387 1.585
 Self-injurious 52% (109) 0.715 0.601 0.992 0.212* 4.118 1.765
 Obsessive or persistent 48% (109) 0.643 1.313 0.453 1.362 0.758 0.406
 Identified emotional trigger
  Anxiety 20% (85) 0.465 0.985 1.173 0.678 0.762 3.200
  Frustration 47% (85) 1.031 0.524 2.241 0.372 2.464 1.478
 Behavior function
  Social attention 61% (109) 1.123 1.212 0.699 1.406 0.304 1.420
  Tangible demand 61% (109) 1.507 0.587 3.080 0.634 1.364 0.682
  Avoidant escape 57% (109) 1.452 0.992 1.631 0.317 0.952 1.143
  Sensory stimulation 45% (109) 0.327* 1.394 1.306 1.122 0.656 1.969
Behavior modification
 Consistent routine 93% (109) 0.290 0.344 0.159 0.172 IS (100%, 9) 0.794
 Sensitive to change in routine 50% (109) 1.119 0.699 0.556 1.898 2.111 0.452
 Motivation
  Immediate reward 55% (84) 2.185 0.824 0.326 0.169* 3.222 7.519
  Delayed reward 15% (84) 4.060 0.376 0.179 0.435 1.857 13.000*
Psychiatric medications
 Anxyolytic/antidepressant
  Current 12% (109) 0.261 1.204 0.669 1.046 2.063 0.802
  Past 6% (109) 1.356 1.450 IS (0%, 23) 0.841 2.187 4.375
 SSRI
  Current 10% (109) 0.836 0.361 0.221 0.324 11.833* 5.917
  Past 11% (109) 1.032 1.066 1.948 0.616 IS (0%, 9) 1.806
 Antipsychotic
  Current 10% (109) 0.838 0.835 1.078 IS (0%, 16) 1.196 IS (0%, 10)
  Past 19% (109) 0.701 1.592 0.865 0.275 0.587 3.128
 Stimulant/antihypnotic
  Current 5% (109) 4.456 0.666 IS (0%, 23) 2.835 IS (0%, 9) IS (0%, 10)
  Past 8% (109) 1.301 0.680 0.421 1.318 IS (100%, 9) 0.917

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

b

behavior that has the potential to harm others.

*

statistically significant odds ratio (α = 0.05). IS: infinite solution (%, n), SSRI: selective serotonin reuptake inhibitor. Behavior medications: anxyolytic/anti-depressants (current: busparone-3, guanfacine-2, trazedone-2, clonidine-1, duloxetine-1, alprazolam-1, clobazam-1, clomipramine-1, clorazepate-1, diazepam-1, lorazepam-1, past: clonidine-3, guanfacine-2, clonazepam-2, diezepam-2, busarone-1); selective seratonin reuptake inhibitor (current: fluoxetine - 4, sertraline- 4, citalopram-3, escitalopram-1, past: sertraline-4, fluvoxetine-3, fluvoxamine-2, paroxetine-2, citalopram-1, escitalopram-1); antipsychotics (current: risperidone-6, quetiapine-2, aripiprazole-1, olazapine-1, paliperidone-1, past: risperdal-19, aripiprazol-5, olanzapine-4, quetiapine-2, promethazine-1, haloperidol (PRN)-1, ziprasidone-1); anxyolytic/anti-depressants (current: busparone-3, guanfacine-2, trazedone-2, clonidine-1, duloxetine-1, alprazolam-1, clobazam-1, clomipramine-1, clorazepate-1, diazepam-1, lorazepam-1, past: clonidine-3, guanfacine-2, clonazepam-2, diezepam-2, busarone-1); antihypnotic/stimulant (current: atomoxetine-2, buprobion-1, modafinil-1, dexmethamphetamine-1, past: methylphenidate-7, dexmethamphetamine-6); antiepileptics (current: oxcarbazepine-2, lamotrigine-1, topiramate-1, past: valproic acid-1, ethosuximide-1); Other: (current: vitamin B complex - 3, past: vitamin B complex-1, lithium-1).