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February 16, 2016; 86 (7) Article

Ocular vestibular evoked myogenic potentials as a test for myasthenia gravis

Yulia Valko, Sally M. Rosengren, Hans H. Jung, Dominik Straumann, Klara Landau, Konrad P. Weber
First published January 20, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002383
Yulia Valko
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Sally M. Rosengren
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Hans H. Jung
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Dominik Straumann
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Klara Landau
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Konrad P. Weber
From the Departments of Ophthalmology (Y.V., K.L., K.P.W.) and Neurology (Y.V., H.H.J., D.S., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Department of Neurology (S.M.R.), Royal Prince Alfred Hospital, Sydney; and the Central Clinical School (S.M.R.), University of Sydney, Australia.
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Citation
Ocular vestibular evoked myogenic potentials as a test for myasthenia gravis
Yulia Valko, Sally M. Rosengren, Hans H. Jung, Dominik Straumann, Klara Landau, Konrad P. Weber
Neurology Feb 2016, 86 (7) 660-668; DOI: 10.1212/WNL.0000000000002383

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Abstract

Objective: To explore whether ocular vestibular evoked myogenic potentials (oVEMP) can be used to detect a decrement in the extraocular muscle activity of patients with myasthenia gravis (MG).

Methods: Twenty-seven patients with MG, including 13 with isolated ocular and 14 with generalized MG, and 28 healthy controls participated. We applied repetitive vibration stimuli to the forehead and recorded the activity of the inferior oblique muscle with 2 surface electrodes placed beneath the eyes. To identify the oVEMP parameters with the highest sensitivity and specificity, we evaluated the decrement over 10 stimulus repetitions at 3 different repetition rates (3 Hz, 10 Hz, and 20 Hz).

Results: Repetitive stimulation at 20 Hz yielded the best differentiation between patients with MG and controls with a sensitivity of 89% and a specificity of 64% when using a unilateral decrement of ≥15.2% as cutoff. When using a bilateral decrement of ≥20.4% instead, oVEMP allowed differentiation of MG from healthy controls with 100% specificity, but slightly reduced sensitivity of 63%. For both cutoffs, sensitivity was similar in isolated ocular and generalized MG.

Conclusion: Our study demonstrates that the presence of an oVEMP decrement is a sensitive and specific marker for MG. This test allows direct and noninvasive examination of extraocular muscle activity, with similarly good diagnostic accuracy in ocular and generalized MG. Thus, oVEMP represents a promising diagnostic tool for MG.

Classification of evidence: This study provides Class III evidence that oVEMP testing accurately identifies patients with MG with ocular symptoms (sensitivity 89%, specificity 64%).

GLOSSARY

AChR=
acetylcholine receptor;
IO=
inferior oblique muscle;
MG=
myasthenia gravis;
oVEMP=
ocular vestibular evoked myogenic potentials;
RNS=
repetitive nerve stimulation;
ROC=
receiver operating characteristics;
SFEMG=
single-fiber EMG

Footnotes

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

  • Supplemental data at Neurology.org

  • Editorial, page 590

  • Received July 2, 2015.
  • Accepted in final form September 29, 2015.
  • © 2016 American Academy of Neurology
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