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Biology subjects

Startin, C. M.

Publications and source records attributed to Startin, C. M..

2 recordsLinked to original sources

Neurofilament light as a blood biomarker for neurodegeneration in Down syndrome

INTRODUCTIONDown syndrome (DS) may be considered a genetic form of Alzheimers disease (AD) due to universal development of AD neuropathology, but diagnosis and treatment trials are hampered by a lack of reliable blood biomarkers. A potential biomarker is neurofilament light (NF-L), due to its association with axonal damage in neurodegenerative conditions.\n\nMETHODSWe measured blood NF-L concentration in 100 adults with DS using Simoa NF-light(R) assays, and examined relationships with age, and cross-sectional and longitudinal dementia diagnosis.\n\nRESULTSNF-L levels increased with age (Spearmans rho = 0.789, p<0.001), with a steep increase after age 40, and were predictive of dementia status (p=0.022 adjusting for age, sex, and APOE4) but showed no relationship with longstanding epilepsy or premorbid ability. Baseline NF-L levels were associated with longitudinal dementia status.\n\nDISCUSSIONNF-L is a biomarker for neurodegeneration in DS, with potential for use in future clinical trials to prevent or delay dementia.\n\nResearch in contextO_ST_ABSSystematic reviewC_ST_ABSThe authors reviewed the literature using PubMed searches supplemented with our knowledge of pending papers in this research area. While blood NF-L has been associated with clinical features of progression in a number of neurodegenerative conditions, we have not identified any reports of NF-L associated with cognitive decline in DS, a genetic form of AD.\n\nInterpretationOur findings demonstrate the potential utility of NF-L as a blood biomarker of neurodegeneration in DS, a population that may not be able to tolerate more invasive procedures such as neuroimaging and lumbar punctures to track progression.\n\nFuture directionsThe association between NF-L and other markers of longitudinal AD progression should be explored further in future work.

molecular biology

Sequence of cognitive changes associated with development of Alzheimer’s disease in Down syndrome – data driven analysis

ObjectiveIndividuals with Down syndrome (DS) have an extremely high genetic risk for Alzheimers disease (AD) however the course of cognitive decline associated with progression to dementia is ill-defined. Data-driven methods can estimate long-term trends from cross-sectional data while adjusting for variability in baseline ability, which complicates dementia assessment in those with DS.\n\nMethodsWe applied an event-based model to cognitive test data and informant-rated questionnaire data from 283 adults with DS (the largest study of cognitive functioning in DS to date) to estimate the sequence of cognitive decline and individuals disease stage.\n\nResultsDecline in tests of memory, sustained attention / motor coordination, and verbal fluency occurred early, demonstrating that AD in DS follows a similar pattern of change to other forms of AD. Later decline was found for informant measures. Using the resulting staging model, we showed that adults with a clinical diagnosis of dementia and those with APOE 3:4 or 4:4 genotype were significantly more likely to be staged later, suggesting the model is valid.\n\nInterpretationOur results identify tests of memory and sustained attention may be particularly useful measures to track decline in the preclinical/prodromal stages of AD in DS whereas informant-measures may be useful in later stages (i.e. during conversion to dementia, or post-diagnosis). These results have implications for the selection of outcome measures of treatment trials to delay or prevent cognitive decline due to AD in DS. As clinical diagnoses are generally made late into AD progression, early assessment is essential.

neuroscience