Determines number of lower motor neurons supplying a muscle (i.e. motor unit)
Can be done before clinical weakness becomes apparent by manual muscle testing
MUNIX is more sensitive to change compared to CMAP and ALSFRS-R
This makes MUNIX a biomarker candidate for disease progression
Motor Unit Number Index (MUNIX) :
Neurofilament:
Among the altered biomarkers, CSF Nf-L level remains the best marker of ALS severity, while serum levels correlate strongly with disease progression. The combination of Nf-L and ICAM-1 concentrations in the CSF and IFN-γ concentration in the serum differentiate ALS patients.
Neurofilament:
Tau Protein:
Tau is a protein that stabilizes the cells’ skeleton via an intermediate phosphorylated Tau (pTau) form
ALS patients have significantly greater levels of total tau than controls
Ratio between pTau and total tau is significantly lower in ALS patients, indicating hyperphosphorylation- causes protien clumping
Tau Protein:
Blood biomarkers:
Conclusions
This study suggests that
Serum Creatinine
Albumin
CRP, and
Glucose
Measured at the time of ALS diagnosis as well as their changes after ALS diagnosis could serve as additional prognostic biomarkers for ALS.