Assessment of the upper motor neuron in amyotrophic lateral sclerosis. Issue 7 (July 2016)

• Record Type: Journal Article
• Authors: Huynh, William; Simon, Neil G.; Grosskreutz, Julian; Turner, Martin R.; Vucic, Steve; Kiernan, Matthew C.
• Is Part Of: Clinical neurophysiology. Volume 127:Issue 7(2016:Jul.)
• Page Start: 2643
• Page End: 2660
• Publication Date: 2016-07
• Subjects: Amyotrophic lateral sclerosis -- Motor neuron disease -- Upper motor neuron -- Imaging -- Transcranial magnetic stimulation; Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2016.04.025
• Languages: English
• ISSNs: 1388-2457

Abstract

Highlights: Clinical signs of UMN involvement are an important component in diagnosis of ALS. Novel neuroimaging and electrophysiology may facilitate demonstration of UMN degeneration in ALS. Improving early ALS diagnosis can facilitate the development of effective therapies. Abstract: 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.