The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

Abstract

eprints@whiterose.ac.uk https://eprints.whiterose.ac.uk/ Reuse Unless indicated otherwise, fulltext items are protected by copyright with all rights reserved. The copyright exception in section 29 of the Copyright, Designs and Patents Act 1988 allows the making of a single copy solely for the purpose of non-commercial research or private study within the limits of fair dealing. The publisher or other rights-holder may allow further reproduction and re-use of this version -refer to the White Rose Research Online record for this item. Where records identify the publisher as the copyright holder, users can verify any specific terms of use on the publisher's website. TakedownIf you consider content in White Rose Research Online to be in breach of UK law, please notify us by emailing eprints@whiterose.ac.uk including the URL of the record and the reason for the withdrawal request. 1The role of cranial and thoracic EMG within diagnostic criteria for ALS

Electrodiagnostic findings in amyotrophic lateral sclerosis: Variation with region of onset and utility of thoracic paraspinal muscle examination

“…However, on average, less than 3 craniobulbar muscles were examined per ALS patient which could partially explain the low yield. Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria 19 . The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non-ALS patients, reporting specificity values of 93% and 95.6%, respectively. 18,19 However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study. In addition, both previous studies employed a control group that included all non-ALS patients who underwent similar EDX studies during the same study periods.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non‐ALS patients, reporting specificity values of 93% and 95.6%, respectively 18,19 . However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study.…”

“…Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria. 19 The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles. For example, in a rat ALS model, persistent tongue motility deficit was apparent in the early phase of the disease, but neuromuscular innervation, citrate synthase protein levels and activity, and uncoupling protein 3 protein levels remained unchanged in the tongue even in the late stage, which was different from those of sternomastoid and limb muscles.…”

Electrodiagnostic findings in amyotrophic lateral sclerosis: Variation with region of onset and utility of thoracic paraspinal muscle examination

“…However, on average, less than 3 craniobulbar muscles were examined per ALS patient which could partially explain the low yield. Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria 19 . The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non-ALS patients, reporting specificity values of 93% and 95.6%, respectively. 18,19 However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study. In addition, both previous studies employed a control group that included all non-ALS patients who underwent similar EDX studies during the same study periods.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non‐ALS patients, reporting specificity values of 93% and 95.6%, respectively 18,19 . However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study.…”

“…Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria. 19 The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles. For example, in a rat ALS model, persistent tongue motility deficit was apparent in the early phase of the disease, but neuromuscular innervation, citrate synthase protein levels and activity, and uncoupling protein 3 protein levels remained unchanged in the tongue even in the late stage, which was different from those of sternomastoid and limb muscles.…”

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

“…A further limitation is the lack of an ALS-mimic group. While we have previously shown that bulbar EMG abnormalities are highly specific to ALS (Jenkins et al , 2016), evaluation of the present device in patients with conditions such as spinobulbar muscular atrophy would provide insight into whether tongue impedance measurements are specific to ALS. In limb muscles early results suggest that impedance have limited efficacy in distinguishing ALS from mimic conditions (Sanchez et al , 2017b).…”

Electrodiagnostic findings in amyotrophic lateral sclerosis: Variation with region of onset and utility of thoracic paraspinal muscle examination

“…However, on average, less than 3 craniobulbar muscles were examined per ALS patient which could partially explain the low yield. Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria 19 . The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non-ALS patients, reporting specificity values of 93% and 95.6%, respectively. 18,19 However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study. In addition, both previous studies employed a control group that included all non-ALS patients who underwent similar EDX studies during the same study periods.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non‐ALS patients, reporting specificity values of 93% and 95.6%, respectively 18,19 . However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study.…”

“…Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria. 19 The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles. For example, in a rat ALS model, persistent tongue motility deficit was apparent in the early phase of the disease, but neuromuscular innervation, citrate synthase protein levels and activity, and uncoupling protein 3 protein levels remained unchanged in the tongue even in the late stage, which was different from those of sternomastoid and limb muscles.…”

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

“…A further limitation is the lack of an ALS-mimic group. While we have previously shown that bulbar EMG abnormalities are highly specific to ALS (Jenkins et al , 2016), evaluation of the present device in patients with conditions such as spinobulbar muscular atrophy would provide insight into whether tongue impedance measurements are specific to ALS. In limb muscles early results suggest that impedance have limited efficacy in distinguishing ALS from mimic conditions (Sanchez et al , 2017b).…”

Electrodiagnostic findings in amyotrophic lateral sclerosis: Variation with region of onset and utility of thoracic paraspinal muscle examination

“…However, on average, less than 3 craniobulbar muscles were examined per ALS patient which could partially explain the low yield. Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria 19 . The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non-ALS patients, reporting specificity values of 93% and 95.6%, respectively. 18,19 However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study. In addition, both previous studies employed a control group that included all non-ALS patients who underwent similar EDX studies during the same study periods.…”

“…Two prior studies focused on the value of TPSP muscles in differentiating ALS from non‐ALS patients, reporting specificity values of 93% and 95.6%, respectively 18,19 . However, both publications examined smaller groups of ALS patients (35 and 170 ALS patients, respectively) than our study.…”

“…Similar observations were made by Jenkins et al who reported that adding cranial muscle examination only moved 1.4% of patients to higher diagnostic categories (probable/definite) using the Awaji criteria. 19 The relatively low yield of EMG study of the craniobulbar muscles could be related to possible intrinsic biochemical and/or physiological differences between craniobulbar and limb muscles. For example, in a rat ALS model, persistent tongue motility deficit was apparent in the early phase of the disease, but neuromuscular innervation, citrate synthase protein levels and activity, and uncoupling protein 3 protein levels remained unchanged in the tongue even in the late stage, which was different from those of sternomastoid and limb muscles.…”

Multi-dimensional electrical impedance myography of the tongue as a potential biomarker for amyotrophic lateral sclerosis

“…A further limitation is the lack of an ALS-mimic group. While we have previously shown that bulbar EMG abnormalities are highly specific to ALS (Jenkins et al , 2016), evaluation of the present device in patients with conditions such as spinobulbar muscular atrophy would provide insight into whether tongue impedance measurements are specific to ALS. In limb muscles early results suggest that impedance have limited efficacy in distinguishing ALS from mimic conditions (Sanchez et al , 2017b).…”