No Evidence of Early Developmental Delay in Juvenile-Onset Huntington's Disease Patients

Lucy Olson; Sarah Dickens; Jordan L Schultz; Mohit Neema; Peggy C Nopoulos

doi:10.1002/mdc3.14287

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No Evidence of Early Developmental Delay in Juvenile-Onset Huntington's Disease Patients

Journal article

Open access

Peer reviewed

No Evidence of Early Developmental Delay in Juvenile-Onset Huntington's Disease Patients

Lucy Olson, Sarah Dickens, Jordan L Schultz, Mohit Neema and Peggy C Nopoulos

Movement disorders clinical practice (Hoboken, N.J.), Vol.12(3), pp.346-352

03/2025

DOI: 10.1002/mdc3.14287

PMCID: PMC11952948

PMID: 39644245

Appears in UI Libraries Support Open Access

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Abstract

Previous studies suggest that early developmental delay is a common feature of Juvenile-Onset Huntington's disease (JOHD), with highest incidence in those with very high CAG repeats (> 80). However, all reports of developmental delay in JOHD are exclusively based on retrospective review of medical charts. Comprehensive assessment of birth history metrics may provide better insight into the question of early life development in JOHD. To explore the prevalence of prematurity, birth complications, low birth weight and developmental delay in patients with JOHD in comparison to control participants. Parents of patients with JOHD and gene-non-expanded (GNE) control participants from Kids-HD (n = 104) and Kids-JOHD (n = 34, 24% with CAG > 80) studies completed a comprehensive birth history questionnaire. Answers focused on prematurity, birth complications, and birth weight, and along with reports of early developmental milestones, were compared between groups. There were no statistically significant differences in prematurity, birth weights, birth complications, or motor and verbal developmental milestones between JOHD patients and GNE controls (all P values > 0.1). Furthermore, stratifying JOHD patients by CAG expansion (low vs. high) also showed no significant differences (GNE vs. low or GNE vs. high). These findings support the notion that JOHD does not manifest as developmental delay before motor symptom onset and highlight a new framework to understand the course and nature of the disease.

JOHD

developmental delay

Huntington's disease

birth metrics

high CAG

UIOWA OA Agreement

Details

Title: Subtitle: No Evidence of Early Developmental Delay in Juvenile-Onset Huntington's Disease Patients
Creators: Lucy Olson - University of Iowa
Sarah Dickens - University of Iowa, Psychiatry
Jordan L Schultz - University of Iowa
Mohit Neema - University of Iowa, Psychiatry
Peggy C Nopoulos - University of Iowa
Resource Type: Journal article
Publication Details: Movement disorders clinical practice (Hoboken, N.J.), Vol.12(3), pp.346-352
DOI: 10.1002/mdc3.14287
PMID: 39644245
PMCID: PMC11952948
NLM abbreviation: Mov Disord Clin Pract
ISSN: 2330-1619
eISSN: 2330-1619
Publisher: Wiley; HOBOKEN
Grant note: R01 NS055903 / NINDS NIH HHS 071108 / CHDI Foundation
Language: English
Electronic publication date: 12/07/2024
Date published: 03/2025
Academic Unit: Neurology; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Pharmacy Practice and Science
Record Identifier: 9984756230902771

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