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Abstract
Objectives Differentiation 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.
Methods We 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.
Results The 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; p < 0.01) and lower levels of neurofilament light chain (NfL) (PLS: 81.8 pg/mL, IQR 38.4–111.1 pg/mL; ALS: 155.9 pg/mL, IQR 85.1–366.4 pg/mL; p = 0.01). Two patients with PLS and 3 patients with ALS carried the C9orf72 expansion. NfL resulted an independent predictor of final diagnosis (odds ratio 1.01, 95% CI 1.00–1.02; p = 0.04) and an independent prognostic factor (hazard ratio 1.01, 95% CI 1.00–1.01; p < 0.01).
Discussion NfL might help to differentiate patients with PLS from patients with ALS and to predict prognosis in patients with UMN syndrome.
Footnotes
Submitted and externally peer reviewed. The handling editor was Associate Editor Anthony Amato, MD, FAAN.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received November 4, 2022.
- Accepted in final form February 9, 2023.
- © 2023 American Academy of Neurology
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