Autism & Developmental

Autistic-Like Traits in Pena-Shokeir Syndrome.

Kalınlı et al. (2019) · Journal of autism and developmental disorders 2019
★ The Verdict

Even when severe birth defects dominate the chart, still screen for autism.

✓ Read this if BCBAs who treat children with multiple congenital anomalies.
✗ Skip if Clinicians who only serve kids with idiopathic ASD and no medical complexity.

01Research in Context

01

What this study did

Doctors wrote up one child with Pena-Shokeir syndrome.

The child also showed signs of autism.

No treatment was tested; the team just described what they saw.

02

What they found

The child had stiff joints, small chin, and feeding tubes.

The same child lined up toys, avoided eye contact, and repeated words.

These behaviors met autism criteria even though the main diagnosis was Pena-Shokeir.

03

How this fits with other research

Mammarella et al. (2022) and Artemios et al. (2019) each tell a similar story.

They each report one patient with a rare genetic syndrome who also meets ASD criteria.

Together the papers build a rule: look for autism any time a child has a complex genetic condition.

Moss et al. (2009) review seven such syndromes and say the same thing in larger form.

The case reports extend the review by adding Pena-Shokeir to the list.

04

Why it matters

When a child has many medical tubes and braces, it is easy to miss social quirks.

This paper reminds you to run an autism screen anyway.

If the score is high, start ABA while the medical team handles the physical issues.

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02At a glance

Intervention
not applicable
Design
case study
Sample size
1
Population
autism spectrum disorder, other
Finding
not reported

03Original abstract

Pena-Shokeir syndrome (PSS) is a rare, early lethal disease. PSS is characterized by fetal growth restriction, craniofacial deformities, multiple ankyloses and pulmonary hypoplasia. Because of the primary concern of physical health problems, psychiatric evaluation is frequently underestimated in PSS patients. Our case report describes a child with PSS who presented with autistic spectrum disorder symptoms.

Journal of autism and developmental disorders, 2019 · doi:10.1007/s10803-018-3824-2