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May 03, 2016; 86 (18) Article

The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies

Agnes van Sonderen, Marco W.J. Schreurs, Marienke A.A.M. de Bruijn, Sanae Boukhrissi, Mariska M.P. Nagtzaam, Esther S.P. Hulsenboom, Roelien H. Enting, Roland D. Thijs, Paul W. Wirtz, Peter A.E. Sillevis Smitt, Maarten J. Titulaer
First published April 1, 2016, DOI: https://doi.org/10.1212/WNL.0000000000002637
Agnes van Sonderen
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Marco W.J. Schreurs
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Marienke A.A.M. de Bruijn
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Sanae Boukhrissi
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Mariska M.P. Nagtzaam
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Esther S.P. Hulsenboom
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Roelien H. Enting
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Roland D. Thijs
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Paul W. Wirtz
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Peter A.E. Sillevis Smitt
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Maarten J. Titulaer
From the Departments of Neurology (A.v.S., M.A.A.M.d.B., M.M.P.N., E.S.P.H., P.A.E.S.S., M.J.T.) and Immunology (M.W.J.S., S.B.), Erasmus Medical Center, Rotterdam; Department of Neurology (A.v.S., P.W.W.), Haga Teaching Hospital, The Hague; Department of Neurology (R.H.E.), University Medical Center Groningen/Rijksuniversiteit Groningen; and Stichting Epilepsie Instellingen Nederland (SEIN) (R.D.T.), Heemstede, the Netherlands.
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Citation
The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies
Agnes van Sonderen, Marco W.J. Schreurs, Marienke A.A.M. de Bruijn, Sanae Boukhrissi, Mariska M.P. Nagtzaam, Esther S.P. Hulsenboom, Roelien H. Enting, Roland D. Thijs, Paul W. Wirtz, Peter A.E. Sillevis Smitt, Maarten J. Titulaer
Neurology May 2016, 86 (18) 1692-1699; DOI: 10.1212/WNL.0000000000002637

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Abstract

Objective: To assess the clinical relevance of a positive voltage-gated potassium channel (VGKC) test in patients lacking antibodies to LGI1 and Caspr2.

Methods: VGKC-positive patients were tested for LGI1 and Caspr2 antibodies. Patients lacking both antibodies were matched (1:2) to VGKC-negative patients. Clinical and paraclinical criteria were used to blindly determine evidence for autoimmune inflammation in both groups. Patients with an inconclusive VGKC titer were analyzed in the same way.

Results: A total of 1,455 patients were tested by VGKC radioimmunoassay. Fifty-six patients tested positive, 50 of whom were available to be included. Twenty-five patients had antibodies to LGI1 (n = 19) or Caspr2 (n = 6) and 25 patients lacked both antibodies. Evidence for autoimmune inflammation was present in 7 (28%) of the VGKC-positive patients lacking LGI1 and Caspr2, compared to 9 (18%) of the VGKC-negative controls (p = 0.38). Evidence for autoimmune inflammation was mainly found in patients with limbic encephalitis/encephalomyelitis (57%), but not in other clinical phenotypes (5%, p < 0.01). VGKC titers were significantly higher in patients with antibodies to LGI1 or Caspr2 (p < 0.001). However, antibodies to Caspr2 could also be detected in patients with inconclusive low VGKC titer, while many VGKC-positive patients had no evidence for autoimmune inflammation.

Conclusions: VGKC positivity in the absence of antibodies to LGI1 and Caspr2 is not a clear marker for autoimmune inflammation and seems not to contribute in clinical practice. No cutoff value for the VGKC titer was appropriate to discriminate between patients with and without autoimmune inflammation.

GLOSSARY

AMPAR=
AMPA receptor;
Caspr2=
contactin-associated protein-like 2;
CBA=
cell-based immunofluorescence assay;
CJD=
Creutzfeldt-Jakob disease;
EM=
encephalomyelitis;
GABABR=
GABAB receptor;
LE=
limbic encephalitis;
LGI1=
leucine-rich glioma-inactivated protein 1;
mRS=
modified Rankin Scale;
NMDAR=
NMDA receptor;
RIA=
radioimmunoassay;
VGKC=
voltage-gated potassium channel

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.

  • Editorial, page 1657

  • Supplemental data at Neurology.org

  • Received September 18, 2015.
  • Accepted in final form December 22, 2015.
  • © 2016 American Academy of Neurology
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Disputes & Debates: Rapid online correspondence

  • Clinical syndrome matters in all patients, with or without a positive VGKC-test (without LGI1/Caspr2-antibodies) (authors' reply)
    • Agnes van Sonderen, Neurologist, Erasmus University Medical Center, Rotterdam, the Netherlandsm.titulaer@erasmusmc.nl
    • Marco WJ Schreurs, Marienke AAM de Bruijn, Peter AE Sillevis Smitt, Maarten J Titulaer
    Submitted July 07, 2016
  • Voltage-gated potassium channel antibodies: Clinical syndrome matters
    • Tianrong Yeo, Associate Consultant Neurologist, National Neuroscience Institute, Singaporeyeo.tianrong@singhealth.com.sg
    • Josiah Chai Yui Huei, Singapore, Singapore; Kevin Tan, Singapore, Singapore
    Submitted July 05, 2016
  • Thorough study, in line with raw data within the literature (authors' reply)
    • Agnes van Sonderen, Neurologist, Erasmus University Medical Centerm.titulaer@erasmusmc.nl
    • Marco WJ Schreurs, Marienke AAM de Bruijn, Peter AE Sillevis Smitt, Maarten J Titulaer
    Submitted June 24, 2016
  • Small sample, overstated conclusions
    • James B Lilleker, Clinical Research Fellow, University of Manchester, Manchester, UKjames.lilleker@manchester.ac.uk
    • Matthew S Jones, University of Manchester, Manchester, UK. Rajiv Mohanraj, University of Manchester, Manchester, UK
    Submitted June 21, 2016
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