Refining the Language of Huntington's Disease Progression with the Huntington's Disease Integrated Staging System (HD-ISS)

Cristina Sampaio^{1

2}, Sandra K Kostyk^{3

4}, Sarah J Tabrizi^{5

6}, Anne E Rosser^{7

8}

Affiliations

¹ CHDI Management, Inc. Advisors to CHDI Foundation, Princeton, NJ, USA.
² Faculdade Medicina da Universidade de Lisboa (FMUL), Portugal.
³ Department of Neurology, The Ohio State University, College of Medicine, Columbus, OH, USA.
⁴ Department of Neuroscience, The Ohio State University, College of Medicine, Columbus, OH, USA.
⁵ Department of Neurodegenerative Disease, Huntington's Disease Centre, UCL Queen Square Institute of Neurology, University College London, London, UK.
⁶ Dementia Research Institute at UCL, London, UK.
⁷ Division of Psychological Medicine and Clinical Neurosciences, Brain Repair and Intracranial Neurotherapeutics (B.R.A.I.N.) Biomedical Research Unit, Cardiff University, Cardiff, UK.
⁸ Brain Repair Group, School of Biosciences, Cardiff University, Cardiff, UK.

PMID: 38968053
PMCID: PMC11307057
DOI: 10.3233/JHD-240043

Editorial

Refining the Language of Huntington's Disease Progression with the Huntington's Disease Integrated Staging System (HD-ISS)

Cristina Sampaio et al. J Huntingtons Dis. 2024.

. 2024;13(2):115-118.

doi: 10.3233/JHD-240043.

Authors

Cristina Sampaio^{1

2}, Sandra K Kostyk^{3

4}, Sarah J Tabrizi^{5

6}, Anne E Rosser^{7

8}

Affiliations

¹ CHDI Management, Inc. Advisors to CHDI Foundation, Princeton, NJ, USA.
² Faculdade Medicina da Universidade de Lisboa (FMUL), Portugal.
³ Department of Neurology, The Ohio State University, College of Medicine, Columbus, OH, USA.
⁴ Department of Neuroscience, The Ohio State University, College of Medicine, Columbus, OH, USA.
⁵ Department of Neurodegenerative Disease, Huntington's Disease Centre, UCL Queen Square Institute of Neurology, University College London, London, UK.
⁶ Dementia Research Institute at UCL, London, UK.
⁷ Division of Psychological Medicine and Clinical Neurosciences, Brain Repair and Intracranial Neurotherapeutics (B.R.A.I.N.) Biomedical Research Unit, Cardiff University, Cardiff, UK.
⁸ Brain Repair Group, School of Biosciences, Cardiff University, Cardiff, UK.

PMID: 38968053
PMCID: PMC11307057
DOI: 10.3233/JHD-240043

No abstract available

PubMed Disclaimer

Conflict of interest statement

Sarah Tabrizi is an Associate Editor and Anne Rosser is an Editorial Board Member of this journal but neither were involved in the peer-review process of the original article or of this editorial nor had access to any information regarding its peer-review.

Figures

**Fig. 1**
Figure 1 provides a schematic representation of how the Huntington’s Disease Integrated Staging System (HD-ISS) aligns with the terminology traditionally used in HD research. In this diagram: •Clinical motor diagnosis, or a Diagnostic Confidence Level (DCL) of 4, corresponds to the latter part of HD-ISS stage 2. •The Shoulson and Fahn stages, which are applicable after clinical motor diagnosis and are based on specific Total Functional Capacity (TFC) scores, overlap with the end of HD-ISS stage 2 and encompass all of stage 3. The figure also illustrates various terms used to denote periods before clinical motor diagnosis. While their usage has not been strictly defined, we propose that HD-ISS stages 0 and 1 can be referred to as pre-symptomatic, whereas stages 2 and 3 of the HD-ISS can be considered symptomatic stages.

See this image and copyright information in PMC

References

1. DiFiglia M, Leavitt BR, Macdonald D, Thompson LM, Huntington’s Disease Nomenclature Working Group. Towards standardizing nomenclature in Huntington’s disease research. J Huntingtons Dis. 2024. 10.3233/JHD-240044. - DOI - PMC - PubMed
1. Gregory S, Long JD, Klöppel S, Razi A, Scheller E, Minkova L, et al.. Operationalizing compensation over time in neurodegenerative disease. Brain. 2017;140(4):1158–65. - PMC - PubMed
1. The Huntington’s Disease Collaborative Research Grou. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell. 1993;72(6):971–83. - PubMed
1. Unified Huntington’s Disease Rating Scale: Reliability and consistency. Mov Disord. 1996;11(2):136–4. - PubMed
1. Tabrizi SJ, Langbehn DR, Leavitt BR, Roos RA, Durr A, Craufurd D, et al., TRACK-HD investigators. Biological and clinical manifestations of Huntington’s disease in the longitudinal TRACK-HD study: Cross-sectional analysis of baseline data. Lancet Neurol. 2009;8(9):791–801. - PMC - PubMed

Publication types

Actions

MeSH terms

Actions
Actions
Actions
Actions

LinkOut - more resources

Full Text Sources
Medical
- MedlinePlus Health Information

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Refining the Language of Huntington's Disease Progression with the Huntington's Disease Integrated Staging System (HD-ISS)

Affiliations

Refining the Language of Huntington's Disease Progression with the Huntington's Disease Integrated Staging System (HD-ISS)

Authors

Affiliations

Conflict of interest statement

Figures

Similar articles

References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Medical