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Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers
- Source :
- Annals of Clinical and Translational Neurology, Annals of Clinical and Translational Neurology, Vol 8, Iss 2, Pp 425-439 (2021)
- Publication Year :
- 2020
- Publisher :
- Wiley, 2020.
-
Abstract
- Objectives To describe CSF‐defined neuronal intermediate filament (NIF) autoimmunity. Methods NIF‐IgG CSF‐positive patients (41, 0.03% of 118599 tested, 1996–2019) were included (serum was neither sensitive nor specific). Criteria‐based patient NIF‐IgG staining of brain and myenteric NIFs was detected by indirect immunofluorescence assay (IFA); NIF‐specificity was confirmed by cell‐based assays (CBAs, alpha internexin, neurofilament light [NF‐L]), heavy‐[NF‐H] chain). Results Sixty‐one percent of 41 patients were men, median age, 61 years (range, 21–88). Syndromes were encephalopathy predominant (23), cerebellar ataxia predominant (11), or myeloradiculoneuropathies (7). MRI abnormalities (T2 hyperintensities of brain, spinal cord white matter tracts. and peripheral nerve axons) and neurophysiologic testing (EEG, EMG, evoked potentials) co‐localized with clinical neurological phenotypes (multifocal in 29%). Thirty patients (73%) had ≥ 1 immunological perturbation: cancer (paraneoplastic), 22; systemic infection (parainfectious [including ehrlichosis, 3] or HIV), 7; checkpoint‐inhibitor cancer immunotherapy, 4; other, 5. Cancers were as follows: neuroendocrine‐lineage carcinomas, 12 (small cell, 6; Merkel cell, 5; pancreatic, 1 [11/12 had NF‐L‐IgG detected, versus 8/29 others, P = 0.0005]) and other, 11. Onset was predominantly subacute (92%) and accompanied by inflammatory CSF (75%), and immunotherapy response (77%). In contrast, CSF controls (15684 total) demonstrated NIF‐IgG negativity (100% of test validation controls), and low frequencies of autoimmune diagnoses (20% of consecutively referred clinical specimens) and neuroendocrine‐lineage carcinoma diagnosis (3.1% vs. 30% of NIF cases), P
- Subjects :
- Central Nervous System
Male
0301 basic medicine
Pathology
neuronal intermediate filament
medicine.medical_treatment
Intermediate Filaments
Autoimmunity
medicine.disease_cause
Autoantigens
Cohort Studies
Mice
0302 clinical medicine
Neurofilament Proteins
Research Articles
Aged, 80 and over
General Neuroscience
Middle Aged
Neurological autoimmunity
medicine.anatomical_structure
Disease Progression
Female
medicine.symptom
Merkel cell
RC321-571
Research Article
Adult
CSF-detected NIF-IgGs
medicine.medical_specialty
Encephalopathy
Neurosciences. Biological psychiatry. Neuropsychiatry
autoimmune axonopathy
Young Adult
03 medical and health sciences
Carcinoma
medicine
Animals
Humans
RC346-429
Aged
Autoantibodies
Cerebellar ataxia
business.industry
Ehrlichiosis
biomarkers
Immunotherapy
medicine.disease
Axons
Hyperintensity
Staining
030104 developmental biology
Immunoglobulin G
Neurology. Diseases of the nervous system
Neurology (clinical)
Nervous System Diseases
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 23289503
- Volume :
- 8
- Database :
- OpenAIRE
- Journal :
- Annals of Clinical and Translational Neurology
- Accession number :
- edsair.doi.dedup.....de4b9466e72cb9e9047f60fafb69ad51