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Accepted version

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2019-02-01

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cc-by-nc-nd (c) Asian Australasian Society of Neurological Surgeons; Churchill Livingstone , 2019
Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/141942

Cerebrospinal fluid neurofilament light in suspected sporadic Creutzfeldt-Jakob disease

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https://doi.org/10.1016/j.jocn.2018.09.031

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of human prion disease. It is invariably fatal and displays a short clinical disease stage. The key event in sCJD is the propagation of a beta-sheet rich conformer of the physiological PrPC protein, known as PrPSc. Neuropathological disease characteristics include gliosis, neuronal loss and spongiform degeneration; disease clinical manifestations refer to mental and visual disabilities, cognitive impairment, gait or limb ataxia, myoclonus and mutism. Definite sCJD diagnosis requires post-mortem brain material histopathological examination. However, highly certain pre-mortem differential diagnosis is desired to exclude other treatable disorders and to reduce disease transmission risks. Detection and/or quantification of cerebrospinal fluid (CSF) biomarkers reflecting neuronal damage and PrPC misfolding in the diseased brain significantly enhance pre-mortem diagnosis. Previously established and newly identified biomarkers are used towards this direction. Increased CSF Neurofilament light chain (NFL) concentrations have been reported in several neurological disorders, including prion diseases. In the present study, we analyzed CSF NFL levels in two independent patient cohorts, consisting of highly suspected sCJD cases that were further classified as sCJD or non-CJD according to established diagnostic criteria. CSF NFL concentrations were increased in sCJD compared to non-CJD cases in both cohorts (area under the curve (with 95% confidence interval) equal to 0.89 (0.82 to 0.97) and 0.86 (0.77 to 0.96), respectively. CSF NFL was associated neither to age nor to sex but correlated with total-tau concentrations in both cohorts. Overall, our data provide independent validation of CSF NFL utility in sCJD differential diagnosis.

Subject

Líquid cefalorraquidi, Malaltia de Creutzfeldt-Jakob, Malalties per prions, Malalties neurodegeneratives

Subject (English)

Cerebrospinal fluid, Creutzfeldt-Jakob disease, Prion diseases, Neurodegenerative Diseases

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Articles publicats en revistes (Patologia i Terapèutica Experimental)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Citation

KANATA, Eirini, et al. Cerebrospinal fluid neurofilament light in suspected sporadic Creutzfeldt-Jakob disease. Journal of Clinical Neuroscience. 2019. Vol. 60, num. 124-127. ISSN 0967-5868. [consulted: 19 of August of 2026]. Available at: https://hdl.handle.net/2445/141942

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