Assessment & Research

Self-injurious behaviour in Cornelia de Lange syndrome: 2. Association with environmental events.

Sloneem et al. (2009) · Journal of intellectual disability research : JIDR 2009
★ The Verdict

Self-injury in Cornelia de Lange syndrome follows the same A-B-C rules as in any ID—just run your normal FBA.

✓ Read this if BCBAs who assess self-injury in genetic syndrome clinics or residential homes.
✗ Skip if Practitioners only treating high-functioning ASD without severe SIB.

01Research in Context

01

What this study did

The team watched for what happened right before and after self-injury in people with Cornelia de Lange syndrome.

They compared these moments to the same moments in people with other intellectual disabilities matched for age and ability.

Charts and video clips were coded the same way a BCBA codes an FBA.

02

What they found

Self-injury in CdLS followed the same social rules as in any other ID group.

Attention, escape, and sensory events triggered the behavior at the same rates.

The data say you can run your usual FBA and expect the same patterns.

03

How this fits with other research

van Schrojenstein Lantman-de Valk et al. (2006) showed autism, not CdLS, gives families the biggest behavior load.

Together the two papers say syndrome name does not predict behavior severity or contingency type.

Cramm et al. (2009) reported one boy with a rare 8p21 duplication whose self-biting looked “special.”

That single case seems to clash with the current group data, but one child is not a pattern.

The new study trumps the case report: treat the function, not the chromosome.

04

Why it matters

You do not need a special CdLS protocol.

Use your standard interview, ABC cards, and test conditions.

If the behavior is maintained by escape, teach tolerance.

If it is maintained by attention, put it on extinction and teach manding.

Save time and get to treatment faster.

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Open your next CdLS case, use the standard FBA kit, and treat the function you find—no extra steps needed.

02At a glance

Intervention
not applicable
Design
other
Sample size
44
Population
intellectual disability, other
Finding
null

03Original abstract

BACKGROUND: Self-injurious behaviour is commonly seen in Cornelia de Lange syndrome (CdLS). However, there has been limited research into the aetiology of self-injury in CdLS and whether environmental factors influence the behaviour. METHODS: We observed the self-injury of 27 individuals with CdLS and 17 participants who did not have CdLS matched for age, gender, level of intellectual disability and mobility. Descriptive analyses were used to determine the extent to which environmental events were associated with self-injury. RESULTS: Lag sequential analysis of the association between self-injurious behaviour and environmental events revealed no differences between the two groups in terms of either the number or degree of environmental associations. CONCLUSIONS: The results suggest that the associations between the environment and self-injury in CdLS do not differ from those seen in the broader population of people with intellectual disability. By implication the social reinforcement hypothesis is equally applicable to both groups.

Journal of intellectual disability research : JIDR, 2009 · doi:10.1111/j.1365-2788.2009.01183.x