Electrodiagnosis in Persons With Amyotrophic Lateral Sclerosis

Abstract

Electrophysiology remains an important tool in the evaluation of patients presenting with signs and symptoms of motor neuron disease (MND). The electrodiagnostic study should include peripheral nerve conduction studies (NCS) and needle electromyography (EMG) to both exclude treatable disease and gather evidence toward a diagnosis of amyotrophic lateral sclerosis (ALS). The recent changes in the revised El Escorial criteria, recommended by the Awaji-shima consensus group, have increased the diagnostic significance of fasciculation potentials to equal that of fibrillation and positive sharp wave potentials in the needle EMG examination of the patient suspected of having ALS. In addition, electrophysiologic evidence is now considered equivalent to clinical signs and symptoms in reaching diagnostic certainty of ALS. These changes, strategies for the design and implementation of an effective electrodiagnostic evaluation, and additional electrophysiologic techniques and their relationship to the evaluation of a patient with ALS are reviewed and discussed.

Role of diffusion tensor imaging or magnetic resonance spectroscopy in the diagnosis and disability assessment of amyotrophic lateral sclerosis

“…Electromyography and motor unit number estimation play an important role in detecting LMNs and are regarded to be the most accurate confirmatory tests for ALS, but these techniques cannot evaluate disease development or the effects of treatment [6,7]. For the UMNs, neuroimaging techniques are a promising way to diagnose ALS or evaluate disease progression, and these techniques include conventional MRI, DTI, MRS and functional MRI [8].…”

LRP 4 antibodies in serum and CSF from amyotrophic lateral sclerosis patients

“…Electromyographic (EMG) changes are strongly supportive for ALS diagnosis. 7 Although suggested long ago, 8 the extent of neuromuscular junction (NMJ) dysfunction in ALS is not known.…”

Endomicroscopy and electromyography of neuromuscular junctions in situ

“…38,53,55 Translational potential of EMG/CEM Electromyography (EMG) is widely used as a diagnostic tool for neuromuscular disorders, including motor neuron diseases such as ALS. 2,[73][74][75] Clinical applications of CEM have, thus far, included investigation of the GI tract, 76 urinary tract, 77 respiratory tract 78 , and cardiovascular system. 79 The present findings lay a foundation for extending CEM to neurological investigation and using it, in association with conventional EMG recording, for diagnosis and monitoring the efficacy of potential treatments of neuromuscular disease.…”