Evaluating motor progression of juvenile-onset Huntington's Disease: An Enroll-HD analysis.

ABSTRACT:

Introduction

Juvenile-onset Huntington's disease (JOHD) is characterized by a unique motor phenotype relative to patients with adult-onset Huntington's Disease (AOHD). This study characterized motor progression of JOHD to propose improved outcome measures for this group.

Methods

We used linear mixed effect regression models to compare progression of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score (TMS) and the chorea score between patients with JOHD and AOHD. We then evaluated all 31 subscales that make up the UHDRS over time within patients with JOHD to identify measures that may be used to track motor progression most reliably.

Results

The JOHD cohort had faster TMS progression compared to AOHD (p = 0.006) but no group difference in the rate

SUBMITTER: Nopoulos S 

PROVIDER: S-EPMC10903276 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature