ArticleJournal of neurology2025
Simple diameters, accurate stages:a practical approach to HD-ISS staging.
Article in Journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Corrections and comments
- Erratum issued
Authors and funding
14 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHuntington's disease (HD) is a neurodegenerative disorder caused by CAG repeat expansions. The Huntington's Disease Integrated Staging System (HD-ISS) classifies disease stages (0-3) using clinical, biological, and imaging assessments, with caudate and putamen volumes serving as key early biomarkers. Conventional automated volumetric methods typically require specialized software and experienced operators, limiting broader accessibility. Simple linear measurements, such as the bicaudate ratio (BCR) and bifrontal-to-bicaudate ratio (FCR), may provide a practical alternative for assessing striatal atrophy.
methodsReference caudate and putamen volumes were measured using automated segmentation (FreeSurfer). Linear measurements (frontal horn width, intercaudate distance, inner table width) were used to calculate BCR and FCR in a training cohort of 44 HD mutation carriers. Regression models predicting caudate and putamen volumes based on FCR were developed and validated in an independent test cohort of 11 HD mutation carriers. Interrater reliability was assessed via intraclass correlation coefficients (ICC).
resultsInterrater reliability was excellent. Among linear measurements, FCR had the strongest correlation with caudate (r = 0.866; R
conclusionFCR-based linear estimation of caudate and putamen volumes provides accurate and reliable HD-ISS staging. This simplified method offers a practical and time-efficient alternative to conventional volumetry, potentially facilitating wider application of HD-ISS staging in clinical practice and non-specialist research settings.
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