Diaphragmatic CMAP amplitude from phrenic nerve stimulation predicts functional decline in ALS

Objective To evaluate phrenic nerve motor amplitude (PhrenicAmp) as an independent predictor of functional decline in amyotrophic lateral sclerosis (ALS). We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction. Methods We...

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Veröffentlicht in:Journal of neurology 2020-07, Vol.267 (7), p.2123-2129
Hauptverfasser: Miranda, Bruno, Gromicho, Marta, Pereira, Mariana, Pinto, Susana, Swash, Michael, de Carvalho, Mamede
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container_end_page 2129
container_issue 7
container_start_page 2123
container_title Journal of neurology
container_volume 267
creator Miranda, Bruno
Gromicho, Marta
Pereira, Mariana
Pinto, Susana
Swash, Michael
de Carvalho, Mamede
description Objective To evaluate phrenic nerve motor amplitude (PhrenicAmp) as an independent predictor of functional decline in amyotrophic lateral sclerosis (ALS). We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction. Methods We included consecutive ALS patients with PhrenicAmp and FVC at baseline. Participants were evaluated with the revised ALS Functional Rating Scale (ALSFRS-R) at inclusion and at, at least, one subsequent follow-up visit. The outcome measure of functional decline was the percentage reduction in ALSFRS-R from baseline. Bulbar dysfunction was defined by the presence of any relevant symptom on the ALSFRS-R bulbar sub-score. Correlations and mixed-effects regressions were used to study the relationship between functional decline and both PhrenicAmp and FVC baseline evaluations. Results A total of 249 ALS patients were included; 64.2% of these had bulbar dysfunction. At inclusion, significant correlations were found between PhrenicAmp and FVC ( p  
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We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction. Methods We included consecutive ALS patients with PhrenicAmp and FVC at baseline. Participants were evaluated with the revised ALS Functional Rating Scale (ALSFRS-R) at inclusion and at, at least, one subsequent follow-up visit. The outcome measure of functional decline was the percentage reduction in ALSFRS-R from baseline. Bulbar dysfunction was defined by the presence of any relevant symptom on the ALSFRS-R bulbar sub-score. Correlations and mixed-effects regressions were used to study the relationship between functional decline and both PhrenicAmp and FVC baseline evaluations. Results A total of 249 ALS patients were included; 64.2% of these had bulbar dysfunction. At inclusion, significant correlations were found between PhrenicAmp and FVC ( p  &lt; 0.001), as well as between each respiratory measure and ALSFRS-R (all p  &lt; 0.001). The functional decline at first (median 3 months) and second (median 6 months) follow-up visits was significantly correlated with baseline values of both respiratory evaluations (all p  &lt; 0.01) in the entire ALS population, but only with baseline PhrenicAmp (all p  &lt; 0.05) in bulbar dysfunction cases. Regression analysis revealed that PhrenicAmp (all p  &lt; 0.05), but not FVC, was a significant independent predictor of functional decline in ALS patients and in those with bulbar dysfunction. Conclusion Baseline PhrenicAmp is an independent predictor of functional decline in ALS, whether or not bulbar dysfunction is present.</description><identifier>ISSN: 0340-5354</identifier><identifier>EISSN: 1432-1459</identifier><identifier>DOI: 10.1007/s00415-020-09818-z</identifier><identifier>PMID: 32253508</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Action Potentials - physiology ; Aged ; Amyotrophic lateral sclerosis ; Amyotrophic Lateral Sclerosis - complications ; Amyotrophic Lateral Sclerosis - diagnosis ; Amyotrophic Lateral Sclerosis - physiopathology ; Clinical Neurology ; Deglutition Disorders - etiology ; Deglutition Disorders - physiopathology ; Diaphragm - physiopathology ; Disease Progression ; Electric Stimulation ; Female ; Follow-Up Studies ; Humans ; Life Sciences &amp; Biomedicine ; Male ; Medicine ; Medicine &amp; Public Health ; Middle Aged ; Neurology ; Neuroradiology ; Neurosciences ; Neurosciences &amp; Neurology ; Original Communication ; Phrenic nerve ; Phrenic Nerve - physiology ; Salivary Gland Diseases - etiology ; Salivary Gland Diseases - physiopathology ; Science &amp; Technology ; Speech Disorders - etiology ; Speech Disorders - physiopathology ; Vital Capacity - physiology</subject><ispartof>Journal of neurology, 2020-07, Vol.267 (7), p.2123-2129</ispartof><rights>Springer-Verlag GmbH Germany, part of Springer Nature 2020</rights><rights>Springer-Verlag GmbH Germany, part of Springer Nature 2020.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>6</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000524368200001</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c441t-274b1cc9624007302c13d58a2a0034818aa215e1765da74b58fe708e7786e0483</citedby><cites>FETCH-LOGICAL-c441t-274b1cc9624007302c13d58a2a0034818aa215e1765da74b58fe708e7786e0483</cites><orcidid>0000-0002-1574-6476 ; 0000-0002-0727-5897 ; 0000-0003-2111-4579 ; 0000-0002-8717-8914 ; 0000-0001-7556-0158 ; 0000-0003-4660-6051</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00415-020-09818-z$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00415-020-09818-z$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>315,781,785,27929,27930,28253,41493,42562,51324</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32253508$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Miranda, Bruno</creatorcontrib><creatorcontrib>Gromicho, Marta</creatorcontrib><creatorcontrib>Pereira, Mariana</creatorcontrib><creatorcontrib>Pinto, Susana</creatorcontrib><creatorcontrib>Swash, Michael</creatorcontrib><creatorcontrib>de Carvalho, Mamede</creatorcontrib><title>Diaphragmatic CMAP amplitude from phrenic nerve stimulation predicts functional decline in ALS</title><title>Journal of neurology</title><addtitle>J Neurol</addtitle><addtitle>J NEUROL</addtitle><addtitle>J Neurol</addtitle><description>Objective To evaluate phrenic nerve motor amplitude (PhrenicAmp) as an independent predictor of functional decline in amyotrophic lateral sclerosis (ALS). We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction. Methods We included consecutive ALS patients with PhrenicAmp and FVC at baseline. Participants were evaluated with the revised ALS Functional Rating Scale (ALSFRS-R) at inclusion and at, at least, one subsequent follow-up visit. The outcome measure of functional decline was the percentage reduction in ALSFRS-R from baseline. Bulbar dysfunction was defined by the presence of any relevant symptom on the ALSFRS-R bulbar sub-score. Correlations and mixed-effects regressions were used to study the relationship between functional decline and both PhrenicAmp and FVC baseline evaluations. Results A total of 249 ALS patients were included; 64.2% of these had bulbar dysfunction. At inclusion, significant correlations were found between PhrenicAmp and FVC ( p  &lt; 0.001), as well as between each respiratory measure and ALSFRS-R (all p  &lt; 0.001). The functional decline at first (median 3 months) and second (median 6 months) follow-up visits was significantly correlated with baseline values of both respiratory evaluations (all p  &lt; 0.01) in the entire ALS population, but only with baseline PhrenicAmp (all p  &lt; 0.05) in bulbar dysfunction cases. Regression analysis revealed that PhrenicAmp (all p  &lt; 0.05), but not FVC, was a significant independent predictor of functional decline in ALS patients and in those with bulbar dysfunction. 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Gromicho, Marta ; Pereira, Mariana ; Pinto, Susana ; Swash, Michael ; de Carvalho, Mamede</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c441t-274b1cc9624007302c13d58a2a0034818aa215e1765da74b58fe708e7786e0483</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Action Potentials - physiology</topic><topic>Aged</topic><topic>Amyotrophic lateral sclerosis</topic><topic>Amyotrophic Lateral Sclerosis - complications</topic><topic>Amyotrophic Lateral Sclerosis - diagnosis</topic><topic>Amyotrophic Lateral Sclerosis - physiopathology</topic><topic>Clinical Neurology</topic><topic>Deglutition Disorders - etiology</topic><topic>Deglutition Disorders - physiopathology</topic><topic>Diaphragm - physiopathology</topic><topic>Disease Progression</topic><topic>Electric Stimulation</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Life Sciences &amp; Biomedicine</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine &amp; Public Health</topic><topic>Middle Aged</topic><topic>Neurology</topic><topic>Neuroradiology</topic><topic>Neurosciences</topic><topic>Neurosciences &amp; Neurology</topic><topic>Original Communication</topic><topic>Phrenic nerve</topic><topic>Phrenic Nerve - physiology</topic><topic>Salivary Gland Diseases - etiology</topic><topic>Salivary Gland Diseases - physiopathology</topic><topic>Science &amp; Technology</topic><topic>Speech Disorders - etiology</topic><topic>Speech Disorders - physiopathology</topic><topic>Vital Capacity - physiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Miranda, Bruno</creatorcontrib><creatorcontrib>Gromicho, Marta</creatorcontrib><creatorcontrib>Pereira, Mariana</creatorcontrib><creatorcontrib>Pinto, Susana</creatorcontrib><creatorcontrib>Swash, Michael</creatorcontrib><creatorcontrib>de Carvalho, Mamede</creatorcontrib><collection>Web of Science - Science Citation Index Expanded - 2020</collection><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Health &amp; 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We also assessed both PhrenicAmp and forced vital capacity (FVC) as predictors of functional loss in patients with bulbar dysfunction. Methods We included consecutive ALS patients with PhrenicAmp and FVC at baseline. Participants were evaluated with the revised ALS Functional Rating Scale (ALSFRS-R) at inclusion and at, at least, one subsequent follow-up visit. The outcome measure of functional decline was the percentage reduction in ALSFRS-R from baseline. Bulbar dysfunction was defined by the presence of any relevant symptom on the ALSFRS-R bulbar sub-score. Correlations and mixed-effects regressions were used to study the relationship between functional decline and both PhrenicAmp and FVC baseline evaluations. Results A total of 249 ALS patients were included; 64.2% of these had bulbar dysfunction. At inclusion, significant correlations were found between PhrenicAmp and FVC ( p  &lt; 0.001), as well as between each respiratory measure and ALSFRS-R (all p  &lt; 0.001). The functional decline at first (median 3 months) and second (median 6 months) follow-up visits was significantly correlated with baseline values of both respiratory evaluations (all p  &lt; 0.01) in the entire ALS population, but only with baseline PhrenicAmp (all p  &lt; 0.05) in bulbar dysfunction cases. Regression analysis revealed that PhrenicAmp (all p  &lt; 0.05), but not FVC, was a significant independent predictor of functional decline in ALS patients and in those with bulbar dysfunction. Conclusion Baseline PhrenicAmp is an independent predictor of functional decline in ALS, whether or not bulbar dysfunction is present.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>32253508</pmid><doi>10.1007/s00415-020-09818-z</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0002-1574-6476</orcidid><orcidid>https://orcid.org/0000-0002-0727-5897</orcidid><orcidid>https://orcid.org/0000-0003-2111-4579</orcidid><orcidid>https://orcid.org/0000-0002-8717-8914</orcidid><orcidid>https://orcid.org/0000-0001-7556-0158</orcidid><orcidid>https://orcid.org/0000-0003-4660-6051</orcidid></addata></record>
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subjects Action Potentials - physiology
Aged
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - complications
Amyotrophic Lateral Sclerosis - diagnosis
Amyotrophic Lateral Sclerosis - physiopathology
Clinical Neurology
Deglutition Disorders - etiology
Deglutition Disorders - physiopathology
Diaphragm - physiopathology
Disease Progression
Electric Stimulation
Female
Follow-Up Studies
Humans
Life Sciences & Biomedicine
Male
Medicine
Medicine & Public Health
Middle Aged
Neurology
Neuroradiology
Neurosciences
Neurosciences & Neurology
Original Communication
Phrenic nerve
Phrenic Nerve - physiology
Salivary Gland Diseases - etiology
Salivary Gland Diseases - physiopathology
Science & Technology
Speech Disorders - etiology
Speech Disorders - physiopathology
Vital Capacity - physiology
title Diaphragmatic CMAP amplitude from phrenic nerve stimulation predicts functional decline in ALS
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