AI Article Synopsis

  • The study investigates the potential of phosphorylated neurofilament heavy chain (pNfH) in distinguishing various upper motor neuron (UMN) syndromes, including ALS and related conditions like PLS and hSP.
  • Results showed significantly higher levels of CSF pNfH in patients with ALS and UMN-predominant ALS compared to PLS and hSP, indicating its utility in differentiating these conditions.
  • Additionally, CSF pNfH was found to be a prognostic marker for survival in classic/bulbar ALS, but clinical signs were more predictive in UMN-predominant ALS patients.

Article Abstract

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. 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. ALS and UMNp-ALS patients had higher CSF pNfH compared to PLS and hSP ( < 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, = 0.01) and presence of multifocal fasciculations (HR 15.69, = 0.02) were independent prognostic factors. 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615649PMC
http://dx.doi.org/10.3390/biomedicines9111623DOI Listing

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