IgLON5 antibody
Open Access
- 1 September 2017
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology Neuroimmunology & Neuroinflammation
- Vol. 4 (5), e385
- https://doi.org/10.1212/nxi.0000000000000385
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.Keywords
This publication has 26 references indexed in Scilit:
- Neph3 associates with regulation of glomerular and neural development in zebrafishDifferentiation, 2012
- CSF complements serum for evaluating paraneoplastic antibodies and NMO-IgGNeurology, 2011
- Paraneoplastic Jaw Dystonia and Laryngospasm With Antineuronal Nuclear Autoantibody Type 2 (Anti-Ri)Archives of Neurology, 2010
- Neuropathology of variants of progressive supranuclear palsyCurrent Opinion in Neurology, 2010
- UCH‐L1 expression of podocytes in diseased glomeruli and in vitroThe Journal of Pathology, 2008
- PROGRESSIVE ENCEPHALOMYELITIS, RIGIDITY, AND MYOCLONUS: A NOVEL GLYCINE RECEPTOR ANTIBODYNeurology, 2008
- Nestin expression in repopulating mesangial cells promotes their proliferationLaboratory Investigation, 2008
- Paraneoplastic anti–N‐methyl‐D‐aspartate receptor encephalitis associated with ovarian teratomaAnnals of Neurology, 2007
- IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channelThe Journal of Experimental Medicine, 2005
- DIFFERENTIAL NEURAL CONTROL OF GLOMERULAR ULTRAFILTRATIONClinical and Experimental Pharmacology and Physiology, 2004