Assessment of the upper motor neuron in amyotrophic lateral sclerosis

William Huynh*, Neil G. Simon, Julian Grosskreutz, Martin R. Turner, Steve Vucic, Matthew C. Kiernan

*Corresponding author for this work
70 Citations (Scopus)


Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is concurrently affected by muscle wasting and lower motor neuron degeneration, particularly in the early symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improved detection of lower motor neuron impairment through electrophysiological features that have improved diagnostic sensitivity, assessment of upper motor neuron involvement remains essentially clinical. As a result, there is often a significant diagnostic delay that in turn may impact institution of disease-modifying therapy and access to other optimal patient management. Biomarkers of pathological UMN involvement are also required to ensure patients with suspected ALS have timely access to appropriate therapeutic trials. The present review provides an analysis of current and recently developed assessment techniques, including novel imaging and electrophysiological approaches used to study corticomotoneuronal pathology in ALS.

Original languageEnglish
JournalClinical Neurophysiology
Issue number7
Pages (from-to)2643-2660
Number of pages18
Publication statusPublished - 01.07.2016
Externally publishedYes

Research Areas and Centers

  • Centers: Center for Neuromuscular Diseases


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