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Title: Cerebrospinal fluid neurofilament light in suspected sporadic Creutzfeldt-Jakob disease
Author: Kanata, Eirini
Golanska, Ewa
Villar Piqué, Anna
Karsanidou, Aikaterini
Dafou, Dimitra
Xanthopoulos, Konstantinos
Schmitz, Matthias
Ferrer, Isidro (Ferrer Abizanda)
Karch, André
Sikorska, Beata
Liberski, Pawel P.
Sklaviadis, Theodoros
Zerr, Inga
Llorens Torres, Franc
Keywords: Líquid cefalorraquidi
Malaltia de Creutzfeldt-Jakob
Malalties per prions
Malalties neurodegeneratives
Cerebrospinal fluid
Creutzfeldt-Jakob disease
Prion diseases
Neurodegenerative Diseases
Issue Date: 1-Feb-2019
Publisher: Elsevier
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.
Note: Versió postprint del document publicat a:
It is part of: Journal of Clinical Neuroscience, 2019, vol. 60, p. 124-127
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ISSN: 0967-5868
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Patologia i Terapèutica Experimental)

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