Assessment & Research

Case Report: Autism Risk Within the Context of Two Chromosome 15 Syndromes.

Kellerman et al. (2023) · Journal of autism and developmental disorders 2023
★ The Verdict

In Angelman syndrome, aggression often means "I’m anxious because my caregiver left"—so probe separation context first.

✓ Read this if BCBAs serving teens or adults with Angelman syndrome in day or residential programs.
✗ Skip if Clinicians whose caseloads hold only idiopathic autism with no genetic diagnoses.

01Research in Context

01

What this study did

The team looked at 50 people with Angelman syndrome. They wanted to know how anxiety shows up in this group.

Parents and caregivers told stories about each person. The team wrote down any signs that looked like worry or fear.

02

What they found

Aggression was the top anxiety sign. It showed up in most of the 50 cases.

The second big trigger was being away from the main caregiver. When mom, dad, or the daily aide left, problem behavior spiked.

03

How this fits with other research

English et al. (1995) first listed behavior issues in Angelman. They noted hyperactivity and laughing fits. Vassos et al. (2023) zooms in on one slice of that list—anxiety-driven aggression—showing the field is moving from wide checklists to sharper targets.

Leader et al. (2022) asked the families the same kind of questions. They found that tummy pain and poor sleep travel together with aggression. The new case series adds caregiver separation as a fresh, specific trigger.

SLibero et al. (2016) reviewed how to treat aggression in autism. Their top picks—FBA, reinforcement, and FCT—still apply. Vassos et al. (2023) says the aggression you see in Angelman may really be anxiety, so start your FBA by testing caregiver presence.

04

Why it matters

If you work with Angelman syndrome, treat hitting or kicking as a possible anxiety signal, not just "problem behavior." Ask, "Who just left the room?" before you pick an intervention. Quick caregiver return, paired with FCT for "I want you," may cut episodes faster than a plain behavior plan.

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Add one question to your FBA interview: "What happens right before the aggression—does a favorite staff walk away?"

02At a glance

Intervention
not applicable
Design
case series
Sample size
50
Population
other
Finding
not reported

03Original abstract

Anxiety is being increasingly identified in Angelman syndrome (AS). Qualitative questions and quantitative assessments were used to evaluate for anxiety in 50 subjects with AS. In-person evaluations assessed behaviors concerning for anxiety and circumstances wherein they occurred. Caregivers completed anxiety and other behavioral rating scales. Caregiver responses were categorized and compared to items from anxiety rating scales. The most common behavioral manifestation of anxiety was "aggression." The most common circumstance was "separation from caregiver/parent." Subjects had elevated scores on anxiety, irritability and hyperactivity scales with lower mean scores among subjects with a maternal deletion. The Pediatric Anxiety Rating Scale best captured behaviors described by caregivers. Existing anxiety scales should be adapted for use in AS.

Journal of autism and developmental disorders, 2023 · doi:10.1097/GIM.0b013e3181def138