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September 29, 2020; 95 (13) Article

Comparison of ice pack test and single-fiber EMG diagnostic accuracy in patients referred for myasthenic ptosis

View ORCID ProfileMaria Pia Giannoccaro, View ORCID ProfileMatteo Paolucci, Corrado Zenesini, Vitantonio Di Stasi, Vincenzo Donadio, Patrizia Avoni, Rocco Liguori
First published August 11, 2020, DOI: https://doi.org/10.1212/WNL.0000000000010619
Maria Pia Giannoccaro
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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  • ORCID record for Maria Pia Giannoccaro
Matteo Paolucci
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Corrado Zenesini
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Vitantonio Di Stasi
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Vincenzo Donadio
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Patrizia Avoni
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Rocco Liguori
From the Dipartimento di Scienze Biomediche e Neuromotorie (M.P.G., P.A., R.L.), Università di Bologna; IRCCS Istituto delle Scienze Neurologiche di Bologna (M.P.G., C.Z., V.D.S., V.D., P.A., R.L.); UO Neurosonologia e Cefalee (M.P.), Università Campus Bio-Medico, Rome; and UO Neurologia (M.P.), Ospedale M. Bufalini, Cesena, AUSL Romagna, Italy.
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Comparison of ice pack test and single-fiber EMG diagnostic accuracy in patients referred for myasthenic ptosis
Maria Pia Giannoccaro, Matteo Paolucci, Corrado Zenesini, Vitantonio Di Stasi, Vincenzo Donadio, Patrizia Avoni, Rocco Liguori
Neurology Sep 2020, 95 (13) e1800-e1806; DOI: 10.1212/WNL.0000000000010619

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Abstract

Objective To compare the diagnostic accuracy of ice pack test (IPT) and single-fiber EMG (SF-EMG) in patients with suspected ocular myasthenia (OM) presenting with ptosis.

Methods We studied consecutive patients referred for the clinical suspicion of OM. Patients underwent IPT and stimulated SF-EMG on the orbicularis oculi muscle. Receiver operating characteristic curve analysis was performed to determine the accuracy of IPT, SF-EMG, and their combination.

Results We included 155 patients, 102 with OM and 53 with other diagnosis (OD). The IPT had a sensitivity of 86% (95% confidence interval [CI] 79–93) and a specificity of 79% (95% CI 68–90). SF-EMG showed a sensitivity of 94% (95% CI 89–98) and a specificity of 79% (95% CI 68–90). Overall, IPT and SF-EMG showed discordant results in 30 cases, 16 OM and 14 OD. The combination of IPT and SF-EMG, using the positivity of at least one test for OM diagnosis, increased the sensitivity to 98% (95% CI 95–100), reducing the specificity to 66% (95% CI 53–78), whereas using the positivity of both tests, we obtained a sensitivity of 82% (95% CI 75–90) and a specificity of 92% (95% CI 85–99). The negativity of both tests had a 94% (95% CI 87–100) negative predictive value. Comparison of the areas under the curve showed no differences in the diagnostic accuracy of IPT, SF-EMG, and their combinations.

Conclusions IPT and SF-EMG have similar diagnostic accuracy in patients with OM presenting with ptosis. The negativity of both tests strongly suggests another diagnosis.

Classification of evidence This study provides Class I evidence that both the IPT and SF-EMG accurately identify patients with OM.

Glossary

AChEI=
acetylcholinesterase inhibitor;
AChR-ab=
acetylcholine receptor antibody;
AUC=
area under the curve;
CI=
confidence interval;
IPT=
ice pack test;
MRD=
margin reflex distance;
NPV=
negative predictive value;
OD=
other diagnosis;
OM=
ocular myasthenia;
PPV=
positive predictive value;
RNS=
repetitive nerve stimulation;
SF-EMG=
single-fiber EMG

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Editorial, page 563

  • Class of Evidence: NPub.org/coe

  • Received December 19, 2019.
  • Accepted in final form April 14, 2020.
  • © 2020 American Academy of Neurology
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Disputes & Debates: Rapid online correspondence

  • Author response: Comparison of ice-pack test and single fiber EMG diagnostic accuracy in patients referred for myasthenic ptosis
    • Maria Pia Giannoccaro, Neurologist, IRCCS Istituto delle Scienze Neurologiche di Bologna and Department of Biomedical and Neuromotor Sciences, University of Bologna
    • Rocco Liguori, Neurologist, IRCCS Istituto delle Scienze Neurologiche di Bologna and Department of Biomedical and Neuromotor Sciences, University of Bologna
    Submitted September 05, 2020
  • Reader response: Comparison of ice-pack test and single fiber EMG diagnostic accuracy in patients referred for myasthenic ptosis
    • Nicholas Silvestri, Assoc. Prof. of Neurology, Asst. Dean for Student & Academic Affairs, Dept. of Neurology, University at Buffalo Jacobs School of Medicine & Biomedical Sciences (Buffalo, NY)
    Submitted September 01, 2020
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