Optimizing electrical impedance myography of the tongue in amyotrophic lateral sclerosis

McIlduff, Courtney; Yim, Sunmin; Pacheck, Adam; Rutkove, Seward B.

doi:10.1002/mus.25375

Cited by 19 publications

(19 citation statements)

References 27 publications

(51 reference statements)

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“…Muscle ultrasound may capture tongue fasciculations in the absence of fasciculation potentials on EMG and the combination of ultrasound and EMG may help the detection of early denervation (116). Likewise, EIM shows promise in detecting changes in the structural composition of the tongue in ALS and may evolve into an important tool to detect early bulbar involvement (117, 118). High-resolution manometry and videomanofluoroscopy may provide unique insights into the dynamics of bolus movement and swallowing pressures enabling early detection of bulbar dysfunction and thus, timely interventions (119, 120).…”

Section: Discussionmentioning

confidence: 99%

Clinical Measures of Bulbar Dysfunction in ALS

et al. 2019

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Bulbar impairment represents a hallmark feature of Amyotrophic Lateral Sclerosis (ALS) that significantly impacts survival and quality of life. Speech and swallowing dysfunction are key contributors to the clinical heterogeneity of ALS and require well-timed and carefully coordinated interventions. The accurate clinical, radiological and electrophysiological assessment of bulbar dysfunction in ALS is one of the most multidisciplinary aspects of ALS care, requiring expert input from speech-language pathologists (SLPs), neurologists, otolaryngologists, augmentative alternative communication (AAC) specialists, dieticians, and electrophysiologists—each with their own evaluation strategies and assessment tools. The need to systematically evaluate the comparative advantages and drawbacks of various bulbar assessment instruments and to develop integrated assessment protocols is increasingly recognized. In this review, we provide a comprehensive appraisal of the most commonly utilized clinical tools for assessing and monitoring bulbar dysfunction in ALS based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) evaluation framework. Despite a plethora of assessment tools, considerable geographical differences exist in bulbar assessment practices and individual instruments exhibit considerable limitations. The gaps identified in the literature offer unique opportunities for the optimization of existing and development of new tools both for clinical and research applications. The multicenter validation and standardization of these instruments will be essential for guideline development and best practice recommendations.

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Section: Discussionmentioning

confidence: 99%

Clinical Measures of Bulbar Dysfunction in ALS

et al. 2019

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“…More recently, EIM has been applied to the evaluation of bulbar dysfunction in ALS, an area of particular importance given the prognostic implications and lack of objective, quantifiable bedside measures of bulbar status (355, 356). Initial investigation has indicated tongue EIM to be a reliable technique, significantly correlating with tongue endurance and the ALSFRS-R bulbar subscore, and distinguishing healthy and diseased muscle (357, 358). Despite the encouraging results emerging principally from a single laboratory, EIM remains in need of development and optimization (355).…”

Section: Electrophysiology Biomarkersmentioning

confidence: 99%

Biomarkers in Motor Neuron Disease: A State of the Art Review

et al. 2019

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Motor neuron disease can be viewed as an umbrella term describing a heterogeneous group of conditions, all of which are relentlessly progressive and ultimately fatal. The average life expectancy is 2 years, but with a broad range of months to decades. Biomarker research deepens disease understanding through exploration of pathophysiological mechanisms which, in turn, highlights targets for novel therapies. It also allows differentiation of the disease population into sub-groups, which serves two general purposes: (a) provides clinicians with information to better guide their patients in terms of disease progression, and (b) guides clinical trial design so that an intervention may be shown to be effective if population variation is controlled for. Biomarkers also have the potential to provide monitoring during clinical trials to ensure target engagement. This review highlights biomarkers that have emerged from the fields of systemic measurements including biochemistry (blood, cerebrospinal fluid, and urine analysis); imaging and electrophysiology, and gives examples of how a combinatorial approach may yield the best results. We emphasize the importance of systematic sample collection and analysis, and the need to correlate biomarker findings with detailed phenotype and genotype data.

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“…The EIM data allow investigators to quantitatively analyze muscle composition and structure. EIM has been extensively used as a painless and reliable outcome measurement to study various neuromuscular diseases including amyotrophic lateral sclerosis (ALS) [20–27], spinal muscular atrophy [28], facioscapulohumeral muscular dystrophy [29], congenital muscular dystrophy [30], inflammatory myopathy [31], inclusion-body myositis [19, 32], radiculopathy [33], and disuse atrophy [34]. Of relevance to our study, EIM has been successfully used in DMD patients and mdx mice [35–42].…”

Section: Introductionmentioning

confidence: 99%

Non-invasive evaluation of muscle disease in the canine model of Duchenne muscular dystrophy by electrical impedance myography

et al. 2017

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Dystrophin-deficient dogs are by far the best available large animal models for Duchenne muscular dystrophy (DMD), the most common lethal childhood muscle degenerative disease. The use of the canine DMD model in basic disease mechanism research and translational studies will be greatly enhanced with the development of reliable outcome measures. Electrical impedance myography (EIM) is a non-invasive painless procedure that provides quantitative data relating to muscle composition and histology. EIM has been extensively used in neuromuscular disease research in both human patients and rodent models. Recent studies suggest that EIM may represent a highly reliable and convenient outcome measure in DMD patients and the mdx mouse model of DMD. To determine whether EIM can be used as a biomarker of disease severity in the canine model, we performed the assay in fourteen young (~6.6-m-old; 6 normal and 8 affected) and ten mature (~16.9-m-old; 4 normal and 6 affected) dogs of mixed background breeds. EIM was well tolerated with good inter-rater reliability. Affected dogs showed higher resistance, lower reactance and phase. The difference became more straightforward in mature dogs. Importantly, we observed a statistically significant correlation between the EIM data and muscle fibrosis. Our results suggest that EIM is a valuable objective measurement in the canine DMD model.

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Optimizing electrical impedance myography of the tongue in amyotrophic lateral sclerosis

Abstract: One hundred kilohertz could serve as an optimal frequency at which to measure EIM phase values of the tongue in ALS. Muscle Nerve 55: 539-543, 2017.

Cited by 19 publications

References 27 publications

Clinical Measures of Bulbar Dysfunction in ALS

Clinical Measures of Bulbar Dysfunction in ALS

Biomarkers in Motor Neuron Disease: A State of the Art Review

Non-invasive evaluation of muscle disease in the canine model of Duchenne muscular dystrophy by electrical impedance myography

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