IgLON5 antibody

Josephe A. Honorat, Lars Komorowski, Keith Anthony Josephs, Kai Fechner, Erik K St Louis, Shannon R. Hinson, Sabine Lederer, Neeraj Kumar, Avi Gadoth, Vanda A Lennon, Sean J Pittock, Andrew B McKeon

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Article number385
JournalNeurology: Neuroimmunology and NeuroInflammation
Volume4
Issue number5
DOIs
StatePublished - 2017
Externally publishedYes

Fingerprint

Antibodies
Immunoglobulin G
Parasomnias
Primary Dysautonomias
Fasciculation
Muscle Cramp
Dysarthria
Myoclonus
Gastrointestinal Motility
Body Temperature Regulation
Dyskinesias
Sleep Apnea Syndromes
Ataxia
Indirect Fluorescent Antibody Technique
Deglutition Disorders
Eye Movements
Nervous System Diseases
Serum
Autoimmunity
Gait

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

IgLON5 antibody. / Honorat, Josephe A.; Komorowski, Lars; Josephs, Keith Anthony; Fechner, Kai; St Louis, Erik K; Hinson, Shannon R.; Lederer, Sabine; Kumar, Neeraj; Gadoth, Avi; Lennon, Vanda A; Pittock, Sean J; McKeon, Andrew B.

In: Neurology: Neuroimmunology and NeuroInflammation, Vol. 4, No. 5, 385, 2017.

Research output: Contribution to journalArticle

Honorat, JA, Komorowski, L, Josephs, KA, Fechner, K, St Louis, EK, Hinson, SR, Lederer, S, Kumar, N, Gadoth, A, Lennon, VA, Pittock, SJ & McKeon, AB 2017, 'IgLON5 antibody', Neurology: Neuroimmunology and NeuroInflammation, vol. 4, no. 5, 385. https://doi.org/10.1212/NXI.0000000000000385
Honorat, Josephe A. ; Komorowski, Lars ; Josephs, Keith Anthony ; Fechner, Kai ; St Louis, Erik K ; Hinson, Shannon R. ; Lederer, Sabine ; Kumar, Neeraj ; Gadoth, Avi ; Lennon, Vanda A ; Pittock, Sean J ; McKeon, Andrew B. / IgLON5 antibody. In: Neurology: Neuroimmunology and NeuroInflammation. 2017 ; Vol. 4, No. 5.
@article{3fa711e6218c4c3398aedfa7e986eaed,
title = "IgLON5 antibody",
abstract = "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.",
author = "Honorat, {Josephe A.} and Lars Komorowski and Josephs, {Keith Anthony} and Kai Fechner and {St Louis}, {Erik K} and Hinson, {Shannon R.} and Sabine Lederer and Neeraj Kumar and Avi Gadoth and Lennon, {Vanda A} and Pittock, {Sean J} and McKeon, {Andrew B}",
year = "2017",
doi = "10.1212/NXI.0000000000000385",
language = "English (US)",
volume = "4",
journal = "Neurology: Neuroimmunology and NeuroInflammation",
issn = "2332-7812",
publisher = "Lippincott Williams and Wilkins Ltd.",
number = "5",

}

TY - JOUR

T1 - IgLON5 antibody

AU - Honorat, Josephe A.

AU - Komorowski, Lars

AU - Josephs, Keith Anthony

AU - Fechner, Kai

AU - St Louis, Erik K

AU - Hinson, Shannon R.

AU - Lederer, Sabine

AU - Kumar, Neeraj

AU - Gadoth, Avi

AU - Lennon, Vanda A

AU - Pittock, Sean J

AU - McKeon, Andrew B

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85029101866&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85029101866&partnerID=8YFLogxK

U2 - 10.1212/NXI.0000000000000385

DO - 10.1212/NXI.0000000000000385

M3 - Article

AN - SCOPUS:85029101866

VL - 4

JO - Neurology: Neuroimmunology and NeuroInflammation

JF - Neurology: Neuroimmunology and NeuroInflammation

SN - 2332-7812

IS - 5

M1 - 385

ER -