CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement

<i>Objective:</i> To assess whether phosphorylated neurofilament heavy chain (pNfH) can discriminate different upper motor neuron (UMN) syndromes, namely, ALS, UMN-predominant ALS, primary lateral sclerosis (PLS) and hereditary spastic paraparesis (hSP) and to test the prognostic value o...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Cecilia Simonini, Elisabetta Zucchi, Roberta Bedin, Ilaria Martinelli, Giulia Gianferrari, Nicola Fini, Gianni Sorarù, Rocco Liguori, Veria Vacchiano, Jessica Mandrioli
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	motor neuron disease neurofilaments upper motor neuron degeneration Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/5652ddbb3ec04dbdbfdfac055c02f307
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:5652ddbb3ec04dbdbfdfac055c02f307
record_format	dspace
spelling	oai:doaj.org-article:5652ddbb3ec04dbdbfdfac055c02f3072021-11-25T16:49:43ZCSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement10.3390/biomedicines91116232227-9059https://doaj.org/article/5652ddbb3ec04dbdbfdfac055c02f3072021-11-01T00:00:00Zhttps://www.mdpi.com/2227-9059/9/11/1623https://doaj.org/toc/2227-9059<i>Objective:</i> To assess whether phosphorylated neurofilament heavy chain (pNfH) can discriminate different upper motor neuron (UMN) syndromes, namely, ALS, UMN-predominant ALS, primary lateral sclerosis (PLS) and hereditary spastic paraparesis (hSP) and to test the prognostic value of pNfH in UMN diseases. <i>Methods:</i> CSF and serum pNfH were measured in 143 patients presenting with signs of UMN and later diagnosed with classic/bulbar ALS, UMNp-ALS, hSP, and PLS. Between-group comparisons were drawn by ANOVA and receiver operating characteristic (ROC) analysis was performed. The prognostic value of pNfH was tested by the Cox regression model. <i>Results:</i> ALS and UMNp-ALS patients had higher CSF pNfH compared to PLS and hSP (<i>p</i> < 0.001). ROC analysis showed that CSF pNfH could differentiate ALS, UMNp-ALS included, from PLS and hSP (AUC = 0.75 and 0.95, respectively), while serum did not perform as well. In multivariable survival analysis among the totality of UMN patients and classic/bulbar ALS, CSF pNfH independently predicted survival. Among UMNp-ALS patients, only the progression rate (HR4.71, <i>p</i> = 0.01) and presence of multifocal fasciculations (HR 15.69, <i>p</i> = 0.02) were independent prognostic factors. <i>Conclusions:</i> CSF pNfH is significantly higher in classic and UMNp-ALS compared to UMN diseases with a better prognosis such as PLS and hSP. Its prognostic role is confirmed in classic and bulbar ALS, but not among UMNp, where clinical signs remained the only independent prognostic factors.Cecilia SimoniniElisabetta ZucchiRoberta BedinIlaria MartinelliGiulia GianferrariNicola FiniGianni SorarùRocco LiguoriVeria VacchianoJessica MandrioliMDPI AGarticlemotor neuron diseaseneurofilamentsupper motor neurondegenerationBiology (General)QH301-705.5ENBiomedicines, Vol 9, Iss 1623, p 1623 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	motor neuron disease neurofilaments upper motor neuron degeneration Biology (General) QH301-705.5
spellingShingle	motor neuron disease neurofilaments upper motor neuron degeneration Biology (General) QH301-705.5 Cecilia Simonini Elisabetta Zucchi Roberta Bedin Ilaria Martinelli Giulia Gianferrari Nicola Fini Gianni Sorarù Rocco Liguori Veria Vacchiano Jessica Mandrioli CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
description	<i>Objective:</i> To assess whether phosphorylated neurofilament heavy chain (pNfH) can discriminate different upper motor neuron (UMN) syndromes, namely, ALS, UMN-predominant ALS, primary lateral sclerosis (PLS) and hereditary spastic paraparesis (hSP) and to test the prognostic value of pNfH in UMN diseases. <i>Methods:</i> CSF and serum pNfH were measured in 143 patients presenting with signs of UMN and later diagnosed with classic/bulbar ALS, UMNp-ALS, hSP, and PLS. Between-group comparisons were drawn by ANOVA and receiver operating characteristic (ROC) analysis was performed. The prognostic value of pNfH was tested by the Cox regression model. <i>Results:</i> ALS and UMNp-ALS patients had higher CSF pNfH compared to PLS and hSP (<i>p</i> < 0.001). ROC analysis showed that CSF pNfH could differentiate ALS, UMNp-ALS included, from PLS and hSP (AUC = 0.75 and 0.95, respectively), while serum did not perform as well. In multivariable survival analysis among the totality of UMN patients and classic/bulbar ALS, CSF pNfH independently predicted survival. Among UMNp-ALS patients, only the progression rate (HR4.71, <i>p</i> = 0.01) and presence of multifocal fasciculations (HR 15.69, <i>p</i> = 0.02) were independent prognostic factors. <i>Conclusions:</i> CSF pNfH is significantly higher in classic and UMNp-ALS compared to UMN diseases with a better prognosis such as PLS and hSP. Its prognostic role is confirmed in classic and bulbar ALS, but not among UMNp, where clinical signs remained the only independent prognostic factors.
format	article
author	Cecilia Simonini Elisabetta Zucchi Roberta Bedin Ilaria Martinelli Giulia Gianferrari Nicola Fini Gianni Sorarù Rocco Liguori Veria Vacchiano Jessica Mandrioli
author_facet	Cecilia Simonini Elisabetta Zucchi Roberta Bedin Ilaria Martinelli Giulia Gianferrari Nicola Fini Gianni Sorarù Rocco Liguori Veria Vacchiano Jessica Mandrioli
author_sort	Cecilia Simonini
title	CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
title_short	CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
title_full	CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
title_fullStr	CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
title_full_unstemmed	CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement
title_sort	csf heavy neurofilament may discriminate and predict motor neuron diseases with upper motor neuron involvement
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/5652ddbb3ec04dbdbfdfac055c02f307
work_keys_str_mv	AT ceciliasimonini csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT elisabettazucchi csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT robertabedin csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT ilariamartinelli csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT giuliagianferrari csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT nicolafini csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT giannisoraru csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT roccoliguori csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT veriavacchiano csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement AT jessicamandrioli csfheavyneurofilamentmaydiscriminateandpredictmotorneurondiseaseswithuppermotorneuroninvolvement
_version_	1718412896199770112

CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement

Ejemplares similares