Assessment of the upper motor neuron in amyotrophic lateral sclerosis.
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.
Brain and Mind Centre, University of Sydney, NSW, Australia; Prince of Wales Clinical School, University of New South Wales, NSW, Australia. Electronic address: w.huynh@neura.edu.au.
William
W
Huynh
Department of Neurology, St Vincent's Hospital, Darlinghurst, Australia.
Neil G
NG
Simon
Hans-Berger Department of Neurology, University Hospital Jena, Jena, Germany.
Julian
J
Grosskreutz
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Martin R
MR
Turner
Western Clinical School, University of Sydney, Sydney, Australia.
Steve
S
Vucic
Brain and Mind Centre, University of Sydney, NSW, Australia.
Matthew C
MC
Kiernan
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D056324:Diffusion Tensor Imaging; D006181:H-Reflex; D006801:Humans; D008279:Magnetic Resonance Imaging; D009046:Motor Neurons / Q000502:physiology*; D049268:Positron-Emission Tomography; D050781:Transcranial Magnetic Stimulation
Amyotrophic lateral sclerosis; Imaging; Motor neuron disease; Transcranial magnetic stimulation; Upper motor neuron