Objective: To validate whether serum Neurofilament Light-chain (NfL) levels correlate with disease severity in CADASIL, and to determine whether serum NfL predicts disease progression and survival.
Methods: Fourty-one (pre-) manifest individuals with CADASIL causing mutations and 22 healthy controls were recruited from CADASIL families. At baseline, MRI-lesion load and clinical severity was determined and serum was stored. Disease progression was measured in 30/41 patients at 7-year follow-up, and survival of all individuals was determined at 17-year follow-up. Serum NfL levels were quantified using an ultra-sensitive molecule array. Generalized estimated equation regression (GEE) was used to analyze association between serum NfL, MRI-lesion load, disease severity, and disease progression. With GEE-based Cox regression, survival was analyzed.
Results: At baseline, serum NfL levels correlated with MRI-lesion load [lacune count (=0.64, = 0.002), brain atrophy (=-0.50, = 0.001), and microbleed count (=0.48, = 0.044)], cognition [CAMCOG (=-0.45, = 0.010), MMSE (=-0.61, = 0.003), GIT (=-0.61, < 0.001), TMT-A (=0.70, < 0.001)) and disability (mRS (=0.70, = 0.002)]. Baseline serum NfL predicted 7-year changes in disability ( = 0.34, < 0.001) and cognition (CAMCOG = -4.94, = 0.032), as well as 17-year survival. Higher NfL levels were associated with increased mortality (HR=1.8 per twofold increase in NfL levels, = 0.006).
Interpretation: Serum NfL levels correlate with disease severity, disease progression and 17-year survival in CADASIL patients. Serum NfL is a promising biomarker to monitor and predict disease course in CADASIL, as well as potentially assessing therapeutic response in future clinical trials.
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| http://dx.doi.org/10.1002/acn3.678 | DOI Listing |
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