Down Syndrome Disintegrative Disorder: New-Onset Autistic Regression, Dementia, and Insomnia in Older Children and Adolescents With Down Syndrome.

Journal Article (Journal Article)

Over a 10-year period in a Down syndrome Clinic, 11 children and adolescents were encountered with a history of new-onset (8) or worsening (3) autistic characteristics. Ten of the 11 (91%) had cognitive decline to a dementia-like state and 9 of the 11 (82%) new-onset insomnia. The mean age at which symptoms developed was 11.4 years (standard deviation = 3.6 years; range 5-14 years), an older age than usual for autistic regression in Down syndrome. Ten of 11 cases (91%) had elevated ("positive") thyroperoxidase antibody titers compared to only 5 of 21 (23%) age-matched control subjects with Down syndrome (P < .001). At follow-up at a mean age of 20.7 years (standard deviation = 3.9 years), 8 of the 11 (73%) were at least somewhat better. Down syndrome disintegrative disorder seems an appropriate name for this newly recognized clinical association, which may be due to autoimmunity.

Full Text

Duke Authors

Cited Authors

  • Worley, G; Crissman, BG; Cadogan, E; Milleson, C; Adkins, DW; Kishnani, PS

Published Date

  • August 2015

Published In

Volume / Issue

  • 30 / 9

Start / End Page

  • 1147 - 1152

PubMed ID

  • 25367918

Electronic International Standard Serial Number (EISSN)

  • 1708-8283

Digital Object Identifier (DOI)

  • 10.1177/0883073814554654


  • eng

Conference Location

  • United States