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Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.
Valko, Yulia; Wirth, Magdalena A; Fierz, Fabienne C; Schesny, Marianne K; Rosengren, Sally; Schmückle-Meier, Tanja; Bockisch, Christopher J; Straumann, Dominik; Schreiner, Bettina; Weber, Konrad P.
Afiliação
  • Valko Y; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Wirth MA; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Fierz FC; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Schesny MK; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Rosengren S; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Schmückle-Meier T; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Bockisch CJ; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Straumann D; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Schreiner B; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
  • Weber KP; From the Neurology Department (Y.V., M.K.S., C.J.B., D.S., B.S., K.P.W.), Clinical Neuroscience Center, and Ophthalmology Department (M.A.W., F.C.F., T.S.-M., C.J.B., K.P.W.), University Hospital Zurich, University of Zurich, Switzerland; Neurology Department and Institute of Clinical Neurosciences
Neurology ; 102(10): e209395, 2024 May.
Article em En | MEDLINE | ID: mdl-38669629
ABSTRACT
BACKGROUND AND

OBJECTIVES:

We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia.

METHODS:

In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm.

RESULTS:

Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG.

DISCUSSION:

The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION ClinicalTrials.gov NCT03049956.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Blefaroptose / Diplopia / Potenciais Evocados Miogênicos Vestibulares / Miastenia Gravis Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Neurology Ano de publicação: 2024 Tipo de documento: Article
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Blefaroptose / Diplopia / Potenciais Evocados Miogênicos Vestibulares / Miastenia Gravis Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Neurology Ano de publicação: 2024 Tipo de documento: Article