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Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers
Andrew McKeon1,2, Shahar Shelly2, Cecilia Zivelonghi1,3
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Annals of Clinical and Translational Neurology
|December 28, 2020
Summary
Neuronal intermediate filament (NIF) autoimmunity, identified by NIF-IgG in cerebrospinal fluid (CSF), presents as treatable axonopathies. These conditions can be associated with cancer or infections, highlighting the importance of CSF testing for diagnosis.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Neuronal intermediate filament (NIF) autoimmunity is a rare neurological condition.
- Cerebrospinal fluid (CSF) analysis is crucial for diagnosing autoimmune neurological disorders.
Purpose of the Study:
- To describe the characteristics of patients with CSF-defined NIF-IgG autoimmunity.
- To investigate the clinical phenotypes, associated conditions, and treatment responses in NIF-IgG autoimmunity.
Main Methods:
- Identified 41 patients with NIF-IgG CSF positivity between 1996-2019 using indirect immunofluorescence assay (IFA) and cell-based assays (CBAs).
- Analyzed patient demographics, clinical syndromes, MRI findings, neurophysiologic testing, and immunological perturbations (cancer, infections, immunotherapy).
Main Results:
- NIF-IgG autoimmunity presented with encephalopathy, cerebellar ataxia, or myeloradiculoneuropathies, often multifocal.
- 73% of patients had associated immunological conditions, including cancer (22), infections (7), and immunotherapy (4).
- Neuroendocrine-lineage carcinomas were frequently associated with NIF-IgG, particularly NF-L-IgG.
Conclusions:
- CSF-detected NIF-IgGs define a spectrum of treatable neurological axonopathies.
- These conditions can be paraneoplastic or parainfectious, emphasizing the need for comprehensive etiological investigation.
