Comparison of the Clinical Spectrum of Juvenile- and Adult-Onset Huntington Disease
Year: 2025
Type: article
Abstract: BACKGROUND AND OBJECTIVES: Differences in clinical characteristics between juvenile-onset Huntington disease (JHD) and adult-onset HD (AHD) are hypothesized but not directly compared. This study compares clinical characteristics occurrence and severity across age-at-onset (AO) subtypes. METHODS: Using the national juvenile-onset HD patient cohort and the international Enroll-HD registry (NCT01574053), we compared childhood-onset JHD (cJHD; AO 0-10), adolescent-onset JHD (aJHD; AO 11-20), and adult-onset HD (AHD; AO 21-65) on proportions of clinical characteristics at onset and psychiatric characteristics in pooled datasets. Kruskal-Wallis test was applied to UHDRS-Total Motor Score (UHDRS-TMS) items of the Enroll-HD dataset to compare the severity of motor disease characteristics 6-10 years after onset. RESULTS: < 0.001). DISCUSSION: This study highlights distinct clinical patterns in JHD subtypes compared with AHD. Stratification by age at onset-defined HD subtypes is needed in future studies.
Source: Neurology
Institution: Leiden University Medical Center
Language: English
Subfield: Cellular and Molecular Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: hybrid
OpenAlex ID: W4409676682