ArticleNeurology2023
Clinical Features and Biomarkers to Differentiate Primary and Amyotrophic Lateral Sclerosis in Patients With an Upper Motor Neuron Syndrome.
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Article in Neurology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed.
- Tolerability, Safety and Effectiveness of Sigh Introduction During Non-Invasive Mechanical Ventilation Cycles in Patients With Amyotrophic Lateral Sclerosis.European journal of neurology · 2026Trial
- Diagnostic Revision From Primary Lateral Sclerosis to Amyotrophic Lateral Sclerosis: A Cohort Study.Neurology · 2026Article
- Association between creatinine-to-cystatin C ratio and ALSFRS-R across clinical phenotypes.BMC neurology · 2026Article
- Longitudinal CSF and Serum Biomarker Dynamics in Tofersen-TreatedInternational journal of molecular sciences · 2026Article
- Primary Lateral Sclerosis Natural History Study: Primary Lateral Sclerosis Functional Rating Scale and Other Outcomes Assessment.Annals of neurology · 2026Article
- Perspective and quality of life in amyotrophic lateral sclerosis patients undergoing percutaneous endoscopic gastrostomy.Frontiers in nutrition · 2026Article
- The genetic architecture of primary lateral sclerosis in a cohort of Italian patients.Journal of neurology · 2025Article
- Role of 2-[Journal of neurology · 2025Article
- Serum neurofilament light chain in distinct phenotypes of amyotrophic lateral sclerosis: A longitudinal, multicenter study.European journal of neurology · 2024Article
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Authors and funding
12 authors.
Paride SchitoFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0002-7152-4472
Tommaso RussoFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0002-3526-5186
Teuta DomiFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Alessandra MandelliFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Laura PozziFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Ubaldo Del CarroFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Paola CarreraFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Federica AgostaFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0003-3121-4979
Angelo QuattriniFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0002-8226-8320
Roberto FurlanFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0001-7376-9425
Massimo FilippiFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.ORCID 0000-0002-5485-0479
Nilo RivaFrom the Experimental Neuropathology Unit (P.S., T.R., T.D., L.P., A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience, San Raffaele Scientific Institute; Neurology Unit (P.S., T.R., M.F.), Neurophysiology Unit (P.S., U.D.C., M.F.), and Neurorehabilitation Unit (P.S., M.F.), IRCCS San Raffaele Scientific Institute; Clinical Neuroimmunology Unit (A.M., R.F.), Division of Neuroscience, Institute of Experimental Neurology, San Raffaele Scientific Institute; Division of Genetics and Cell Biology (P.C.), Unit of Genomics for Human Disease Diagnosis, Laboratory of Clinical Molecular Biology, IRCCS Ospedale San Raffaele; Neuroimaging Research Unit (F.A., M.F.), Institute of Experimental Neurology, Division of Neuroscience, IRCCS San Raffaele Scientific Institute; Vita-Salute San Raffaele University (F.A., M.F.); and 3rd Neurology Unit and Motor Neuron Disease Centre (N.R.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. riva.nilo@hsr.it nilo.riva@istituto-besta.it.ORCID 0000-0002-0513-9517
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesDifferentiation between primary (PLS) and amyotrophic lateral sclerosis (ALS) entails relevant consequences for prognosis and management but is mostly unreliable at early stages. The objectives of the study are (1) to determine the features at onset that could help to differentiate between PLS and ALS, (2) to evaluate the diagnostic performance of an integrated serum biomarker panel, and (3) to identify the prognostic factors for patients presenting with upper motor neuron (UMN) syndrome.
methodsWe selected and retrospectively analyzed the clinical data of patients presenting with UMN syndrome. At the first evaluation, when available, serum biomarkers were measured using ultrasensitive single molecule array.
resultsThe study population included 55 patients with PLS and 50 patients with ALS. Patients with PLS presented a longer time to first neurologic evaluation (PLS: 35.0 months, interquartile range [IQR] 17.0-38.0 months; ALS: 12.5 months, IQR 7.0-21.3 months; DISCUSSION: NfL might help to differentiate patients with PLS from patients with ALS and to predict prognosis in patients with UMN syndrome.
Indexed as
Amyotrophic Lateral SclerosisMotor Neuron DiseaseBiomarkersHumansMotor NeuronsPrognosisRetrospective StudiesBiomarkers
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