Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak emerged in December 2019 in SouthEastern China and rapidly spreaded throughout the globe. Lombardy (and the city of Milan) is one of the hardest-hit regions of Italy. Although fever and respiratory symptoms are the core presenting features of coronavirus disease 2019 (COVID-19), there is an increasing awareness of neurological manifestations as an important factor for the prognosis [1]. We report a patient with a post-infectious Guillain-Barré syndrome (GBS) associated with SARS-CoV-2 infection. On 13 April 2020, a man in his sixties, living in the urban area of Milan, referred to our emergency department complaining a three-day history of progressive limb weakness and distal paresthesia at four-limbs. His past medical history was unremarkable. Twenty days before he had developed fever (37.7-38.5 °C), headache and myalgia followed by anosmia and ageusia (Fig. 1). He was, therefore, prescribed a home self-isolation protocol until symptom resolution. He recovered within about ten days, except for the persistence of
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.