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Open Access 01-07-2020 | Epilepsy | Original Communication

Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome

Authors: Christian G. Bien, Corinna I. Bien, Müjgan Dogan Onugoren, Desiree De Simoni, Verena Eigler, Carl-Albrecht Haensch, Martin Holtkamp, Fatme S. Ismail, Martin Kurthen, Nico Melzer, Kristina Mayer, Felix von Podewils, Helmut Rauschka, Andrea O. Rossetti, Wolf-Rüdiger Schäbitz, Olga Simova, Karsten Witt, Romana Höftberger, Theodor W. May

Published in: Journal of Neurology | Issue 7/2020

Abstract

Objective

To determine frequencies, interlaboratory reproducibility, clinical ratings, and prognostic implications of neural antibodies in a routine laboratory setting in patients with suspected neuropsychiatric autoimmune conditions.

Methods

Earliest available samples from 10,919 patients were tested for a broad panel of neural antibodies. Sera that reacted with leucine-rich glioma-inactivated protein 1 (LGI1), contactin-associated protein-2 (CASPR2), or the voltage-gated potassium channel (VGKC) complex were retested for LGI1 and CASPR2 antibodies by another laboratory. Physicians in charge of patients with positive antibody results retrospectively reported on clinical, treatment, and outcome parameters.

Results

Positive results were obtained for 576 patients (5.3%). Median disease duration was 6 months (interquartile range 0.6–46 months). In most patients, antibodies were detected both in CSF and serum. However, in 16 (28%) patients with N-methyl-d-aspartate receptor (NMDAR) antibodies, this diagnosis could be made only in cerebrospinal fluid (CSF). The two laboratories agreed largely on LGI1 and CASPR2 antibody diagnoses (κ = 0.95). The clinicians (413 responses, 71.7%) rated two-thirds of the antibody-positive patients as autoimmune. Antibodies against the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), NMDAR (CSF or high serum titer), γ-aminobutyric acid-B receptor (GABABR), and LGI1 had ≥ 90% positive ratings, whereas antibodies against the glycine receptor, VGKC complex, or otherwise unspecified neuropil had ≤ 40% positive ratings. Of the patients with surface antibodies, 64% improved after ≥ 3 months, mostly with ≥ 1 immunotherapy intervention.

Conclusions

This novel approach starting from routine diagnostics in a dedicated laboratory provides reliable and useful results with therapeutic implications. Counseling should consider clinical presentation, demographic features, and antibody titers of the individual patient.

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Title: Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome
Authors: Christian G. Bien
Corinna I. Bien
Müjgan Dogan Onugoren
Desiree De Simoni
Verena Eigler
Carl-Albrecht Haensch
Martin Holtkamp
Fatme S. Ismail
Martin Kurthen
Nico Melzer
Kristina Mayer
Felix von Podewils
Helmut Rauschka
Andrea O. Rossetti
Wolf-Rüdiger Schäbitz
Olga Simova
Karsten Witt
Romana Höftberger
Theodor W. May
Publication date: 01-07-2020
Publisher: Springer Berlin Heidelberg
Keywords: Epilepsy
Autoimmune Encephalitis
Encephalitis
Published in: Journal of Neurology / Issue 7/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-09814-3

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome

Abstract

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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