Phelan McDermid Syndrome (pms) Prevalence Tracker

POPULATION PREVALENCE ESTIMATE

13.7

per

100,000


Approximately 45,000

1 in ~7,300

individuals have PMS in U.S.

95% CONFIDENCE INTERVAL

10.02 - 18.60 per 100,000

(1 in ~10,000 to 1 in ~5,400)

HOW THE ESTIMATE WAS DERIVED

Data sources

The study team reached out to clinical genetic testing laboratories, research centers, and clinical centers.

Data collected

Sites provided the number of PMS diagnoses made out of the total number of participants with autism tested at their site.

Adjustments made

Further extrapolations were made to adjust for the proportion of individuals with PMS who do not have autism, autism diagnosis age limitations, and type of genetic variant.

Population extrapolation

The Centers for Disease Control and Prevention (CDC) estimates of autism prevalence rates were used to extrapolate to the general population.

Final estimate

After applying extrapolations, including adjustments for assay sensitivity and other factors, the final weighted average was 13.7 per 100,000.

STUDY DETAILS

Sources participating

10

Autism cases evaluated

179,837

Frequency of PMS diagnosis among autism cases

1% to ~2.5%

(Raw results varied by testing method and should not be directly compared.)

Study Design

Population-based prevalence estimation study

Population represented

General population (via extrapolation from autism rates)

This project was funded by Neuren Pharmaceuticals, CureSHANK and Seaver Autism Center.

Key Findings

This population-based study found PMS to affect approximately 13.7 per 100,000 individuals (1 in ~7,300), with a 95% CI of 10.02-18.60 per 100,000 (1 in ~10,000 to 1 in ~5,400), substantially higher than previous estimates of 2.5-10 per million births (0.25-1 per 100,000). Phelan-McDermid syndrome is a rare genetic condition caused by deletion or mutation of the SHANK3 gene on chromosome 22.

Read the Landmark Prevalence Study Announcement

Learn more about the landmark study estimating that Phelan-McDermid syndrome may affect approximately 1 in 7,300 people, why the findings matter, and what they mean for families, researchers, and the future of precision medicine.

Read the Full Announcement →