Journal article
Repetitive ocular vestibular evoked myogenic potentials in myasthenia gravis.
- de Meel RHP From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Keene KR From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Wirth MA From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Weber KP From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Badrising UA From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Verschuuren JJ From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland.
- Tannemaat MR From the Department of Neurology (R.H.P.d.M., K.R.K., U.A.B, J.J.V., M.R.T.), Leiden University Medical Center, the Netherlands; and Departments of Ophthalmology (M.A.W., K.P.W.) and Neurology (K.P.W.), University Hospital Zurich, University of Zurich, Switzerland. m.r.tannemaat@lumc.nl.
- 2020-03-29
Published in:
- Neurology. - 2020
Adult
Aged
Case-Control Studies
Electromyography
Female
Graves Ophthalmopathy
Humans
Lambert-Eaton Myasthenic Syndrome
Male
Middle Aged
Myasthenia Gravis
Myasthenic Syndromes, Congenital
Neuromuscular Diseases
Prospective Studies
Receptor Protein-Tyrosine Kinases
Receptors, Cholinergic
Sensitivity and Specificity
Vestibular Evoked Myogenic Potentials
English
OBJECTIVE
To validate the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test for diagnostic use in myasthenia gravis (MG) and to investigate its value in diagnostically challenging subgroups.
METHODS
The RoVEMP test was performed in 92 patients with MG, 22 healthy controls, 33 patients with a neuromuscular disease other than MG (neuromuscular controls), 4 patients with Lambert-Eaton myasthenic syndrome, and 2 patients with congenital myasthenic syndrome.
RESULTS
Mean decrement was significantly higher in patients with MG (28.4% ± 32.2) than in healthy controls (3.2% ± 13.9; p < 0.001) or neuromuscular controls (3.8% ± 26.9; p < 0.001). With neuromuscular controls as reference, a cutoff of ≥14.3% resulted in a sensitivity of 67% and a specificity of 82%. The sensitivity of the RoVEMP test was 80% in ocular MG and 63% in generalized MG. The RoVEMP test was positive in 6 of 7 patients with seronegative MG (SNMG) with isolated ocular weakness. Of 10 patients with SNMG with negative repetitive nerve stimulation (RNS) results, 73% had an abnormal RoVEMP test. The magnitude of decrement was correlated with the time since the last intake of pyridostigmine (B = 5.40; p = 0.019).
CONCLUSIONS
The RoVEMP test is a new neurophysiologic test that, in contrast to RNS and single-fiber EMG, is able to measure neuromuscular transmission of extraocular muscles, which are the most affected muscles in MG. Especially in diagnostically challenging patients with negative antibody tests, negative RNS results, and isolated ocular muscle weakness, the RoVEMP test has a clear added value in supporting the diagnosis of MG.
CLASSIFICATION OF EVIDENCE
This study provides Class III evidence that RoVEMP distinguishes MG from other neuromuscular diseases.
To validate the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test for diagnostic use in myasthenia gravis (MG) and to investigate its value in diagnostically challenging subgroups.
METHODS
The RoVEMP test was performed in 92 patients with MG, 22 healthy controls, 33 patients with a neuromuscular disease other than MG (neuromuscular controls), 4 patients with Lambert-Eaton myasthenic syndrome, and 2 patients with congenital myasthenic syndrome.
RESULTS
Mean decrement was significantly higher in patients with MG (28.4% ± 32.2) than in healthy controls (3.2% ± 13.9; p < 0.001) or neuromuscular controls (3.8% ± 26.9; p < 0.001). With neuromuscular controls as reference, a cutoff of ≥14.3% resulted in a sensitivity of 67% and a specificity of 82%. The sensitivity of the RoVEMP test was 80% in ocular MG and 63% in generalized MG. The RoVEMP test was positive in 6 of 7 patients with seronegative MG (SNMG) with isolated ocular weakness. Of 10 patients with SNMG with negative repetitive nerve stimulation (RNS) results, 73% had an abnormal RoVEMP test. The magnitude of decrement was correlated with the time since the last intake of pyridostigmine (B = 5.40; p = 0.019).
CONCLUSIONS
The RoVEMP test is a new neurophysiologic test that, in contrast to RNS and single-fiber EMG, is able to measure neuromuscular transmission of extraocular muscles, which are the most affected muscles in MG. Especially in diagnostically challenging patients with negative antibody tests, negative RNS results, and isolated ocular muscle weakness, the RoVEMP test has a clear added value in supporting the diagnosis of MG.
CLASSIFICATION OF EVIDENCE
This study provides Class III evidence that RoVEMP distinguishes MG from other neuromuscular diseases.
- Language
-
- English
- Open access status
- closed
- Identifiers
-
- DOI 10.1212/WNL.0000000000009306
- PMID 32217778
- Persistent URL
- https://sonar.ch/global/documents/94673
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