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Integrated evaluation of a panel of neurochemical biomarkers to optimize diagnosis and prognosis in amyotrophic lateral sclerosis.
Falzone, Yuri Matteo; Domi, Teuta; Mandelli, Alessandra; Pozzi, Laura; Schito, Paride; Russo, Tommaso; Barbieri, Alessandra; Fazio, Raffaella; Volontè, Maria Antonietta; Magnani, Giuseppe; Del Carro, Ubaldo; Carrera, Paola; Malaspina, Andrea; Agosta, Federica; Quattrini, Angelo; Furlan, Roberto; Filippi, Massimo; Riva, Nilo.
Afiliação
  • Falzone YM; Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Domi T; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Mandelli A; Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Pozzi L; Clinical Neuroimmunology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Schito P; Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Russo T; Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Barbieri A; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Fazio R; Experimental Neuropathology Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Milan, Italy.
  • Volontè MA; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Magnani G; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Del Carro U; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Carrera P; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Malaspina A; Neurology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Agosta F; Neurophysiology Unit, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Quattrini A; Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, Division of Genetics and Cell Biology, San Raffaele Hospital, Scientific Institute for Research and Health Care, Milan, Italy.
  • Furlan R; Centre for Neuroscience and Trauma, Blizard Institute, Queen Mary University of London, London, UK.
  • Filippi M; Neuroimaging Research Unit, Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
  • Riva N; Vita-Salute San Raffaele University, Milan, Italy.
Eur J Neurol ; 29(7): 1930-1939, 2022 07.
Article em En | MEDLINE | ID: mdl-35263489
ABSTRACT
BACKGROUND AND

PURPOSE:

This study was undertaken to determine the diagnostic and prognostic value of a panel of serum biomarkers and to correlate their concentrations with several clinical parameters in a large cohort of patients with amyotrophic lateral sclerosis (ALS).

METHODS:

One hundred forty-three consecutive patients with ALS and a control cohort consisting of 70 patients with other neurodegenerative disorders (DEG), 70 patients with ALS mimic disorders (ALSmd), and 45 healthy controls (HC) were included. Serum neurofilament light chain (NfL), ubiquitin carboxyl-terminal hydrolase isozyme L1 (UCHL1), glial fibrillary acidic protein (GFAP), and total tau protein levels were measured using ultrasensitive single molecule array.

RESULTS:

NfL correlated with disease progression rate (p < 0.001) and with the measures of upper motor neuron burden (p < 0.001). NfL was higher in the ALS patients with classic and pyramidal phenotype. GFAP was raised in ALS with cognitive-behavioral impairment compared with ALS with normal cognition. NfL displayed the best diagnostic performance in discriminating ALS from HC (area under the curve [AUC] = 0.990), DEG (AUC = 0.946), and ALSmd (AUC = 0.850). UCHL1 performed well in distinguishing ALS from HC (AUC = 0.761), whereas it was not helpful in differentiating ALS from DEG and ALSmd. In multivariate analysis, NfL (p < 0.001) and UCHL1 (p = 0.038) were independent prognostic factors. Survival analysis combining NfL and UCHL1 effectively stratified patients with lower NfL levels (p < 0.001).

CONCLUSIONS:

NfL is a useful biomarker for the diagnosis of ALS and the strongest predictor of survival. UCHL1 is an independent prognostic factor helpful in stratifying survival in patients with low NfL levels, likely to have slowly progressive disease. GFAP reflects extramotor involvement, namely cognitive impairment or frontotemporal dementia.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Demência Frontotemporal / Esclerose Lateral Amiotrófica Tipo de estudo: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Eur J Neurol Assunto da revista: NEUROLOGIA Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Itália

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Demência Frontotemporal / Esclerose Lateral Amiotrófica Tipo de estudo: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Eur J Neurol Assunto da revista: NEUROLOGIA Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Itália