SLPCohortAmerican journal on intellectual and developmental disabilities2022

NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome.

Emma E Condy, Lindsey Becker, Cristan Farmer and 4 others

PMID 36306408

WHAT IT FOUND

Most people with Williams syndrome completed the NIH Toolbox iPad tests, but age-corrected scores hit a statistical floor for adults.

Use uncorrected scores to track progress, as age-corrected ones may falsely suggest decline in older patients.

Key findings

0179% of participants completed all three cognitive tests, with 89% completion rates for each individual task.

02Age-corrected scores showed a substantial floor effect, particularly in participants aged 20 to 50 years.

03Performance on the memory task was significantly better than on the executive function tasks.

STILL TO COME

How it was doneWhat they foundWhat it means for SLPs

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What it does not show

The study used a wide age range, but had few participants in the 3-7 year age group, limiting conclusions about younger children. Age-corrected scores have a statistical floor of 54, which compresses performance data for lower-functioning individuals. The study did not account for co-occurring medical or psychiatric conditions or medication use, which could affect attention and test completion. Prompting was allowed during testing, which deviates from standard administration and could potentially influence reaction-time-based scores.

Declared interests

The study was supported by the NIH Intramural Research Program.

The easy way to misread this

Do not interpret low age-corrected scores in adults with Williams syndrome as evidence of cognitive decline. The study found a statistical floor effect where age-corrected scores dropped as age increased, even if ability remained stable. Use uncorrected scores for more accurate longitudinal tracking.

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