Intellectual disability as relevant to self-injurious behaviour
Intellectual disability.
One of the main psychological factors that is relevant to self-injurious behaviour in Cornelia de Lange Syndrome is the associated degree of intellectual disability. Research has indicated that people with Cornelia de Lange Syndrome have an intellectual disability ranging from mild to profound degree of disability, with the majority of people having a severe disability 5, 7, 13, 14, 15, 16, 17. In our research project we found that 45.6 % of people with Cornelia de Lange Syndrome had a profound degree of intellectual disability and 30.43% had a severe degree of intellectual disability when we used a standardised measure of adaptive behaviour (see Box 2.3). Presence of self-injury is related to intellectual disability and research has suggested that individuals with a greater degree of disability are more likely to display self-injury 18, 19 (see Chapter 3).
The table below shows the percentage of individuals in each category of degree of intellectual disability in our study of 54 people with Cornelia de Lange Syndrome. Note that the many of individuals with Cornelia de Lange Syndrome have either profound or severe intellectual disabilities.
Percentage of individuals in each category of degree of intellectual disability
| Profound | Severe | Moderate | Mild | Borderline | |
|---|---|---|---|---|---|
| CdLS | 45.65 | 40.43 | 15.22 | 8.70 | 0.0 |
Since discovery of genes, in combination with technology we see a increase of diagnosis for CdLS. These increased cases are typical less severe (mild). This means we advise you to also read Genotype versus Phenotype correlation
on behalf of the Cornelia de Lange Syndrome Foundation, published on 20 juin 2003
