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Clinical manifestations of the anti-IgLON5 disease.
Clinical manifestations of the anti-IgLON5 disease.
- Source :
-
Neurology [Neurology] 2017 May 02; Vol. 88 (18), pp. 1736-1743. Date of Electronic Publication: 2017 Apr 05. - Publication Year :
- 2017
-
Abstract
- Objective: To report the presentation, main syndromes, human leukocyte antigen (HLA) association, and immunoglobulin G (IgG) subclass in the anti-IgLON5 disease: a disorder with parasomnias, sleep apnea, and IgLON5 antibodies.<br />Methods: This was a retrospective clinical analysis of 22 patients. The IgG subclass was determined using reported techniques.<br />Results: Patients' median age was 64 years (range 46-83). Symptoms that led to initial consultation included sleep problems (8 patients; 36%), gait abnormalities (8; 36%), bulbar dysfunction (3; 14%), chorea (2; 9%), and cognitive decline (1; 5%). By the time of diagnosis of the disorder, 4 syndromes were identified: (1) a sleep disorder with parasomnia and sleep breathing difficulty in 8 (36%) patients; (2) a bulbar syndrome including dysphagia, sialorrhea, stridor, or acute respiratory insufficiency in 6 (27%); (3) a syndrome resembling progressive supranuclear palsy (PSP-like) in 5 (23%); and (4) cognitive decline with or without chorea in 3 (14%). All patients eventually developed parasomnia, sleep apnea, insomnia, or excessive daytime sleepiness. HLA-DRB1*10:01 and HLA-DQB1*05:01 were positive in 13/15 (87%) patients; the DRB1*10:01 allele was 36 times more prevalent than in the general population. Among 16 patients with paired serum and CSF samples, 14 had IgLON5 antibodies in both, and 2 only in serum (both had a PSP-like syndrome). Twenty of 21 patients had IgG1 and IgG4 antibodies; the latter predominated in 16.<br />Conclusions: Patients with IgLON5 antibodies develop a characteristic sleep disorder preceded or accompanied by bulbar symptoms, gait abnormalities, oculomotor problems, and, less frequently, cognitive decline. IgG4 subclass antibodies predominate over IgG1; we confirm a strong association with the HLA-DRB1*10:01 allele.<br /> (© 2017 American Academy of Neurology.)
- Subjects :
- Aged
Aged, 80 and over
Autoantibodies metabolism
Autoimmune Diseases therapy
Biomarkers blood
Biomarkers cerebrospinal fluid
Brain diagnostic imaging
Carrier Proteins metabolism
HLA-DRB1 Chains metabolism
Humans
Immunoglobulin G metabolism
Immunotherapy
Middle Aged
Retrospective Studies
Sleep Wake Disorders therapy
Autoimmune Diseases diagnosis
Autoimmune Diseases physiopathology
Cell Adhesion Molecules, Neuronal immunology
Sleep Wake Disorders diagnosis
Sleep Wake Disorders physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1526-632X
- Volume :
- 88
- Issue :
- 18
- Database :
- MEDLINE
- Journal :
- Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 28381508
- Full Text :
- https://doi.org/10.1212/WNL.0000000000003887