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J Child Neurol. 2015 Aug;30(9):1147-52. doi: 10.1177/0883073814554654. Epub 2014 Nov 3.

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

Author information

1
Division of Pediatric Neurology (Developmental Medicine), Durham, NC, USA gordon.worley@duke.edu.
2
Division of Genetics and Metabolism, Durham, NC, USA.
3
Division of Laboratory Medicine, Department of Pathology, Duke University Medical Center, Durham, NC, USA.
4
Division of Endocrinology, Department of Pediatrics, Durham, NC, USA.

Abstract

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.

KEYWORDS:

Down syndrome; Hashimoto; autism; catatonia; disintegrative disorder; insomnia; thyroperoxidase antibody

PMID:
25367918
DOI:
10.1177/0883073814554654
[Indexed for MEDLINE]

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