Highlights
- •In this study, a commercial assay was used to detect LGI1/CASPR2 antibodies.
- •Seven of 8 patients with very high VGKC-complex antibody levels (median 2663.5 pM) had LGI1/CASPR2 antibodies.
- •The patient without LGI1/CASPR2 antibodies remained negative on re-testing with a live cell assay.
- •There was high inter-rater reliability between the 2 test readers.
- •We discuss the performance characteristics of the commercial assay and the patients' clinico-serological associations.
Abstract
Background
The presence of VGKC-complex antibodies, without LGI1/CASPR2 antibodies, as a standalone
marker for neurological autoimmunity remains controversial. Additionally, the lack
of an unequivocal VGKC-complex antibody cut-off level defining neurological autoimmunity
makes it important to test for monospecific antibodies. We aim to determine the performance
characteristics of a commercial assay (Euroimmun, Lübeck, Germany) for LGI1/CASPR2
antibody detection in patients with very high VGKC-complex antibody levels and report
their clinico-serological associations.
Methods
We identified 8 patients in our cohort with the highest VGKC-complex antibody levels
(median 2663.5 pM, range 933–6730 pM) with VGKC-complex antibody related syndromes (Group A). Two other groups were
identified; 1 group with suspected neuronal surface antibody syndromes and negative
for VGKC-complex antibodies (Group B, n = 8), and another group with cerebellar ataxia and negative for onconeuronal antibodies
(Group C, n = 8).
Results
Seven out of 8 patients (87.5%) in Group A had LGI1 and/or CASPR2 antibodies. One
Group B patient had LGI1 antibodies but was negative on re-testing with a live cell
assay. No Group C patients had monospecific antibodies. Inter-rater reliability was
high; combining Groups A and B patients, the kappa statistic was 0.87 and 1.0 for
LGI1 and CASPR2 antibodies respectively.
Conclusion
We demonstrated that a high proportion of patients with very high VGKC-complex antibody
levels and relevant clinical syndromes have LGI1 and/or CASPR2 antibodies detected
by the commercial assay. Our findings lend support to the use of the assay for rapid
and reliable detection of LGI1 and CASPR2 antibodies.
Keywords
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Article info
Publication history
Published online: April 27, 2017
Accepted:
April 25,
2017
Received in revised form:
March 26,
2017
Received:
November 21,
2016
Identification
Copyright
© 2017 Elsevier B.V. All rights reserved.
