Autism & Developmental

Social behavior and autism traits in a sex chromosomal disorder: Klinefelter (47XXY) syndrome.

van Rijn et al. (2008) · Journal of autism and developmental disorders 2008
★ The Verdict

Klinefelter syndrome often brings autism-like social struggles that last into adulthood.

✓ Read this if BCBAs working with teens or adults in medical-genetic clinics.
✗ Skip if Clinicians who only treat early-childhood classic autism.

01Research in Context

01

What this study did

The team asked 51 men with Klinefelter syndrome (47,XXY) and 51 typical men to fill out two checklists.

One checklist measured autism traits. The other measured social distress. All men were 18-65 years old.

02

What they found

The XXY group scored higher on every autism trait area. They also reported more social distress.

The gap was large enough to be seen without statistics: XXY men looked like an at-risk group.

03

How this fits with other research

Honigfeld et al. (2012) looked at boys and teens with XXY, XYY, and XXYY. They found XYY and XXYY boys had worse social scores than XXY boys.

This seems like a clash, but age is the key. Sophie et al. studied grown men; Lisa et al. studied kids. The extra Y may hurt social skills early, yet XXY adults still land in the autism-trait danger zone.

Mulder et al. (2020) trimmed the same SRS scale for fragile-X syndrome. Their work reminds us: raw scores can over-flag, so use clinical judgment before labeling an XXY client autistic.

04

Why it matters

If you serve an adult with XXY, screen for autism features even if none were noted before. Social-skills groups or PEERS coaching may help. Also watch for anxiety linked to social failure; referrals for CBT or ACT can lift quality of life.

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Run the SRS-2 on any XXY client and add social goals if scores top 60.

02At a glance

Intervention
not applicable
Design
case control
Sample size
31
Population
other
Finding
negative

03Original abstract

Although Klinefelter syndrome (47,XXY) has been associated with psychosocial difficulties, knowledge of the social behavioral phenotype is limited. We examined specific social abilities and autism traits in Klinefelter syndrome. Scores of 31 XXY men on the Scale for Interpersonal Behavior and the Autism Spectrum Questionnaire were compared to 24 and 20 control men respectively. XXY men reported increased distress during social interactions and less engagement in specific social behaviors. In the XXY group, levels of autism traits were significantly higher across all dimensions of the autism phenotype. These findings call for a clinical investigation of vulnerability to autism in Klinefelter syndrome. Klinefelter syndrome might serve as a model for studying a role of the X chromosome in social behavioral dysfunction and autism-like behavior.

Journal of autism and developmental disorders, 2008 · doi:10.1007/s10803-008-0542-1