Assessing the upper motor neurone: novel UMN biomarkers are needed for clinical trials

The paper by Nitert provides a valuable contribution regarding the importance of the upper motor neuron (UMN) as a potential disease biomarker of amyotrophic lateral sclerosis (ALS). There are well-described clinical findings of UMN dysfunction such as increased tone and hyperreflexia, but these can be difficult to grade; others have highlighted the importance of slowing of movements, particularly hand function. Hence, there is a disconnect between simplified clinical scoring systems and the disease itself, and we are left with no standard measurement of UMN involvement in the clinical or research setting. The short bedside scoring system described by Devine, modified from Ravits, has been used, where the decline was most notable for the LMN Score. Compensatory effects potentially limit the utility of clinical scoring; however, for the assessment of the UMN, they are likely to be superior to the ALSFRS-R.

An ideal UMN biomarker should correlate with the temporal course of…

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