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PLoS One. 2016 Sep 22;11(9):e0163479. doi: 10.1371/journal.pone.0163479. eCollection 2016.

Cerebrospinal Fluid Inflammatory Biomarkers Reflect Clinical Severity in Huntington's Disease.

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Huntington's Disease Centre, Institute of Neurology, University College London, London, United Kingdom.
Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden.
Department of Molecular Neuroscience, Institute of Neurology, University College London, London, United Kingdom.



Immune system activation is involved in Huntington's disease (HD) pathogenesis and biomarkers for this process could be relevant to study the disease and characterise the therapeutic response to specific interventions. We aimed to study inflammatory cytokines and microglial markers in the CSF of HD patients.


CSF TNF-α, IL-1β, IL-6, IL-8, YKL-40, chitotriosidase, total tau and neurofilament light chain (NFL) from 23 mutation carriers and 14 healthy controls were assayed.


CSF TNF-α and IL-1β were below the limit of detection. Mutation carriers had higher YKL-40 (p = 0.003), chitotriosidase (p = 0.015) and IL-6 (p = 0.041) than controls. YKL-40 significantly correlated with disease stage (p = 0.007), UHDRS total functional capacity score (r = -0.46, p = 0.016), and UHDRS total motor score (r = 0.59, p = 4.5*10-4) after adjustment for age.


YKL-40 levels in CSF may, after further study, come to have a role as biomarkers for some aspects of HD. Further investigation is needed to support our exploratory findings.

Conflict of interest statement

SJT receives Grant funding for her research from the Medical Research Council UK, the Wellcome Trust, CHDI Foundation, the BBSRC, Rosetrees Trust, and the UCL/UCLH Biomedical Research Centre. In the past year SJT has been on advisory boards or had consultancies with Ixico Technologies, Shire Human Genetic Therapies, and TEVA Pharmaceuticals. All honoraria paid for these consultancies and advisory boards go to UCL. EJW has undertaken consultancy work for Ionis, Roche and Shire Pharmaceuticals, from which all fees were paid to his employer UCL to support his research. The other authors have no conflict of interest to declare. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

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