Autistic disorder symptoms in Rett syndrome.
Girls with classic Rett syndrome show autism-like behaviors at the same rate as any child with severe-profound ID, so screen with standard tools and compare to ID norms.
01Research in Context
What this study did
The team looked at 29 girls with classic Rett syndrome. They gave two checklists: the DBC and the DISCO. Both ask about autism-like behaviors.
They wanted to know if these girls showed more autistic features than other kids with severe-profound ID.
What they found
Autistic signs were common, but the rate matched what you see in any child with severe-profound ID. In other words, Rett syndrome itself did not add extra autism risk.
The data say: screen for autism, but expect scores typical for the cognitive level.
How this fits with other research
Wilkinson et al. (1998) looked at a different group — girls with the preserved-speech variant of Rett. Most of them already carried an autism diagnosis. That paper seems to clash with Josette et al., but the key is the subtype: preserved-speech variant vs classic Rett.
Repp et al. (1992) and Raslear et al. (1992) used the same small case-series method to map communication loss in classic Rett. Their work supports the idea that social-communication scores will be low, yet not necessarily above the ID baseline.
Bauman (1991) set the stage by describing the full clinical picture. Josette et al. simply added a finer lens on one piece — autistic features — within that already-described frame.
Why it matters
If you assess a girl with Rett, run the DBC or DISCO to check for comorbid autism, but interpret scores against severe-profound ID norms. Don’t over-label. Use the same cut-offs you use for other kids at that cognitive level, then plan interventions that fit both the Rett motor profile and any autism-specific needs you find.
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02At a glance
03Original abstract
According to the major classification systems it is not possible to diagnose a comorbid autistic disorder in persons with Rett syndrome. However, this is a controversial issue, and given the level of functioning of persons with Rett syndrome, the autistic disorder is expected to be present in a comparable proportion as in people with the same level of functioning. To investigate, parents of 52 females with classical and atypical Rett syndrome (2.4-49.3 years) completed the Developmental Behavior Checklist (DBC), the Diagnostic Interview for Social and Communication Disorders (DISCO) and the Dutch Vineland Screener 0-6 (VS 0-6). All participants had a severe to profound intellectual disability (ID) according to the VS 0-6. Behavior indicated an autistic disorder in 42 (DBC) to 58 percent (DISCO) of the Rett cases. Autistic behavior had decreased in 19 percent such that they no longer met the criteria for autistic disorder. Some participants were suspected of having a comorbid autistic disorder, though not more often than can be expected at their level of functioning. Clinicians should be aware of the possibility of a comorbid autistic disorder as much as they should be in other people with this level of functioning.
Autism : the international journal of research and practice, 2009 · doi:10.1177/1362361309338184