Neurophysiological and imaging biomarkers of lower motor neuron dysfunction in motor neuron diseases/amyotrophic lateral sclerosis: IFCN handbook chapter

Lin, Cindy Shin-Yi and Howells, James and Rutkove, Seward and Nandedkar, Sanjeev and Neuwirth, Christoph and Noto, Yu-ichi and Shahrizaila, Nortina and Whittaker, Roger G. and Bostock, Hugh and Burke, David and Tankisi, Hatice (2024) Neurophysiological and imaging biomarkers of lower motor neuron dysfunction in motor neuron diseases/amyotrophic lateral sclerosis: IFCN handbook chapter. Clinical Neurophysiology, 162. pp. 91-120. ISSN 1388-2457, DOI https://doi.org/10.1016/j.clinph.2024.03.015.

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Official URL: https://doi.org/10.1016/j.clinph.2024.03.015

Abstract

This chapter discusses comprehensive neurophysiological biomarkers utilised in motor neuron disease (MND) and, in particular, its commonest form, amyotrophic lateral sclerosis (ALS). These encompass the conventional techniques including nerve conduction studies (NCS), needle and high-density surface electromyography (EMG) and H-reflex studies as well as novel techniques. In the last two decades, new methods of assessing the loss of motor units in a muscle have been developed, that are more convenient than earlier methods of motor unit number estimation (MUNE), and may use either electrical stimulation (e.g. MScanFit MUNE) or voluntary activation (MUNIX). Electrical impedance myography (EIM) is another novel approach for the evaluation that relies upon the application and measurement of high-frequency, low-intensity electrical current. Nerve excitability techniques (NET) also provide insights into the function of an axon and reflect the changes in resting membrane potential, ion channel dysfunction and the structural integrity of the axon and myelin sheath. Furthermore, imaging ultrasound techniques as well as magnetic resonance imaging are capable of detecting the constituents of morphological changes in the nerve and muscle. The chapter provides a critical description of the ability of each technique to provide neurophysiological insight into the complex pathophysiology of MND/ALS. However, it is important to recognise the strengths and limitations of each approach in order to clarify utility. These neurophysiological biomarkers have demonstrated reliability, specificity and provide additional information to validate and assess lower motor neuron dysfunction. Their use has expanded the knowledge about MND/ALS and enhanced

Item Type: Article
Funders: UNSPECIFIED
Uncontrolled Keywords: Motor neuron disease; Amyotrophic Lateral Sclerosis; Biomarkers; Electromyography; Nerve excitability testing; Motor unit number estimation; MScanFit; MUNIX; Electrical impedance myography; Neuromuscular ultrasonography; neuromuscular MRI
Subjects: R Medicine > R Medicine (General)
Divisions: Faculty of Medicine > Medicine Department
Depositing User: Ms. Juhaida Abd Rahim
Date Deposited: 25 Sep 2024 07:51
Last Modified: 25 Sep 2024 07:51
URI: http://eprints.um.edu.my/id/eprint/45187

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