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Abstract Details

Plasma Neurofilament Light (NfL) Levels Do Not Correlate with Severity of Clinical Symptoms in Manifest Huntington’s Disease (HD)
Movement Disorders
Movement Disorders Posters (7:00 AM-5:00 PM)
040
To determine whether plasma neurofilament-light (NfL) levels correlate with severity of clinical symptoms in individuals with premanifest (Pre-HD) and manifest Huntington’s Disease (HD); in addition, whether an NfL cut-off point could distinguish pre-HD from HD with reasonable sensitivity and specificity.
A strong correlation between NfL protein levels and HD severity would substantiate its usefulness as a marker of disease progression.

98 participants, including 33 control, 26 pre-HD, and 39 manifest HD subjects, underwent blood sample collection and clinical assessment, using both UHDRS and non-UHDRS measures, at one academic HD Center. Years to onset (YTO)(Aylward); in addition to probability of disease onset in 5 years and predicted years to 60% probability of disease onset (Langbehn) were calculated. Plasma NfL levels were measured using a Meso Scale Discovery Assay. ROC analysis was used to determine whether plasma NFL cut-off points could distinguish the various cohorts.
Cohorts differed by age (p<.0001) but not gender. Plasma NfL levels were significantly different across diagnostic groups (Kruskal-Wallis χ2= 58.81, p<.0001) and were significantly correlated with age (r=.50, p<.0001) but not gender (U=949, p=.08) or CAG repeat number (r=.04, p=.72). Age-adjusted NfL levels were not correlated with clinical measures in either the HD or Pre-HD subjects, but were correlated when the cohorts were combined. In Pre-HD, NfL levels were correlated with Aylward score, probability of onset in 5 years, and years until 60% onset probability. An NfL cut-off point of <53.15pg/ml (AUC=0.97, sensitivity=94.9%, specificity=85.9%, p<.0001) distinguished manifest HD from control; a value of <74.84pg/ml (AUC=0.94, sensitivity=87.2%, specificity=88.5%, p<.0001) distinguished manifest from pre-HD.

The lack of correlation between plasma NfL levels and clinical measures in manifest HD limits its usefulness for tracking disease progression; however, plasma NfL may have importance in predicting probability of disease onset and distinguishing pre-HD from HD with reasonable sensitivity and specificity. 
Authors/Disclosures
Jody Corey-Bloom, MD, PhD, FÂ鶹´«Ã½Ó³»­ (UCSD Neurosciences)
PRESENTER 		Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UniQure. Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Pharmaceuticaks. Dr. Corey-Bloom has received personal compensation in the range of $10,000-$49,999 for serving as a Co-Director, HD-Net with Huntington Study Group.
Georgia M. Parkin, PhD (University of California, San Diego) Georgia Parkin has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file