Photo Credit: Dr Microbe

The following is a summary of “Rate of change in upper and lower motor neuron burden is associated with survival in amyotrophic lateral sclerosis,” published in the April 2025 issue of Journal of Neurology by Maranzano et al. 

Researchers conducted a retrospective study to assess whether the rate of change in upper (ΔUMN) and lower (ΔLMN) motor neuron signs from symptom onset to first clinical assessment predicts survival and disease progression in amyotrophic lateral sclerosis (ALS). 

They evaluated a retrospective inpatient cohort of 1,000 patients with ALS. They assessed UMN and LMN burden using the Penn Upper Motor Neuron Score and Lower Motor Neuron Score. For 421 patients, the ENCALS survival model was computed. Univariate and regularized Cox regressions estimated effects on survival. ROC curve analysis on a training sub-cohort identified a ΔLMN cut-off for prolonged vs short survival, which was cross-validated on a test sub-cohort. A multinomial regression model compared ΔUMN and ΔLMN scores across ENCALS groups. 

The results showed ΔUMN (HR = 1.30) and ΔLMN (HR = 4.22) were negatively associated with survival. A ΔLMN cut-off of 0.22 predicted short vs prolonged survival. ENCALS groups with shorter survival had higher ΔUMN and ΔLMN scores. PUMNS and LMNS gross scores showed no significant association. 

Investigators suggested that ΔUMN and ΔLMN reflected the progressing degeneration of motor neuron subpopulations. They represented reliable and easily measurable clinical indexes to estimate survival in ALS. 

Source: link.springer.com/article/10.1007/s00415-025-13052-w