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September 2017; 4 (5) ArticleOpen Access

IgLON5 antibody

Neurological accompaniments and outcomes in 20 patients

Josephe A. Honorat, Lars Komorowski, Keith A. Josephs, Kai Fechner, Erik K. St Louis, Shannon R. Hinson, Sabine Lederer, Neeraj Kumar, Avi Gadoth, Vanda A. Lennon, Sean J. Pittock, Andrew McKeon
First published July 18, 2017, DOI: https://doi.org/10.1212/NXI.0000000000000385
Josephe A. Honorat
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Lars Komorowski
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Keith A. Josephs
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Kai Fechner
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Erik K. St Louis
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Shannon R. Hinson
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Sabine Lederer
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Neeraj Kumar
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Avi Gadoth
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Vanda A. Lennon
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Sean J. Pittock
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Andrew McKeon
From the Department of Laboratory Medicine and Pathology (J.A.H., S.R.H., V.A.L., S.J.P., A.M.), Department of Neurology (K.A.J., E.K.S.L., N.K., A.G., V.A.L., S.J.P., A.M.), Department of Medicine (E.K.S.L.), Department of Immunology (V.A.L.), and Center for Sleep Medicine (E.K.S.L.), College of Medicine, Mayo Clinic, Rochester, MN; and Institute of Experimental Immunology (L.K., K.F., S.L.), Euroimmun AG, Lubeck, Germany.
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Citation
IgLON5 antibody
Neurological accompaniments and outcomes in 20 patients
Josephe A. Honorat, Lars Komorowski, Keith A. Josephs, Kai Fechner, Erik K. St Louis, Shannon R. Hinson, Sabine Lederer, Neeraj Kumar, Avi Gadoth, Vanda A. Lennon, Sean J. Pittock, Andrew McKeon
Neurol Neuroimmunol Neuroinflamm Sep 2017, 4 (5) e385; DOI: 10.1212/NXI.0000000000000385

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Abstract

Objective: To describe the phenotypes, treatment response, and outcome of IgLON5 autoimmunity.

Methods: Archived serum and CSF specimens from 367 patients known to harbor unclassified antibodies which stained neural synapses diffusely (mimicking amphiphysin-IgG) were reevaluated by indirect immunofluorescence assay (IFA) using a composite of mouse tissues and recombinant IgLON5-transfected cell-based assay (CBA, Euroimmun).

Results: Available specimens (serum, 25; CSF, 9) from 26/367 patients (7%) had identical IFA appearance and robust IgLON5 CBA positivity. Clinical information was available for 20/26 patients; 13 were women. Median disease-onset age was 62 years (range, 46–75 years). Most patients had insidious onset and progression of neurological symptoms affecting movement and sleep predominantly. Sleep disorders were sleep-disordered breathing (11) and parasomnias (3). Brainstem disorders were gait instability (14), dysphagia (10), abnormal eye movements (7), respiratory dysfunction (6), ataxia (5), craniocervical dystonia (3), and dysarthria (3). Findings compatible with hyperexcitability included myoclonus (3), cramps (3), fasciculations (2), and exaggerated startle (2). Neuropsychiatric disorders included cognitive dysfunction (6), psychiatric symptoms (5), and seizures (1). Dysautonomia, in 9, affected bladder function (7), gastrointestinal motility (3), thermoregulation (3), and orthostatic tolerance (1). Just 2 patients had coexisting autoimmune disease. Brain MRI findings were nonspecific and CSF was noninflammatory in all tested. Seven of 9 immunotherapy-treated patients improved: 6 of those 7 were stable at last follow-up. Three untreated patients died. Each IgLON5-IgG subclass (1–4) was readily detectable in ≥80% of specimens using CBA.

Conclusions: IgLON5-IgG is diagnostic of a potentially treatable neurological disorder, where autoimmune clues are otherwise lacking.

GLOSSARY

AHI=
Apnea-Hypopnea Index;
ANNA=
antineuronal nuclear antibody;
CBA=
cell-based assay;
GAD65=
glutamic acid decarboxylase 65;
MSA=
multiple system atrophy;
OSA=
obstructive sleep apnea;
PSP=
progressive supranuclear palsy

Footnotes

  • Funding information and disclosures are provided at the end of the article. Go to Neurology.org/nn for full disclosure forms. The Article Processing Charge was funded by the authors.

  • Supplemental data at Neurology.org/nn

  • Received March 7, 2017.
  • Accepted in final form June 8, 2017.
  • Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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