Axonal autoimmune neuropathies
A Targeted testing approach
Patients with axonal autoimmune neuropathy have variable sensory and motor disturbances and can present with symptoms that result from injury to the distal nerves, roots, ganglia, or their gathering points. Patients may have symmetric or asymmetric involvements of the extremities, trunk, and head, including extraocular muscles. Subacute onset and asymmetric involvements more commonly indicate inflammatory or immune causes rather than inherited or metabolic forms. Other parts of the nervous system may also be affected based on the specific inflammatory or immune-mediated causes.
Because of the overlap of clinical symptoms, we recommend a panel-based approach rather than individual antibody testing.
Axonal autoimmune neuropathies Test menu
Axonal antibody panel
Key testing
- AIAES | Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
- Evaluates specific antibodies with clinical relevance to autoimmune or paraneoplastic neuropathies.
- Provides clarity on etiology and prognosis.
- Facilitates selection of targeted treatment.
Highlights
Collaborating with Mayo Clinic Laboratories, UNC Health streamlined neuroimmunology test ordering — eliminating outmoded testing and elevating diagnostic accuracy.
Anastasia Zekeridou, M.D., Ph.D., explains how Mayo Clinic Laboratories' updated panels and methodology boost the accuracy and efficiency of testing for three autoimmune neurology biomarkers. Early diagnosis is key to managing debilitating conditions associated with these antibodies.
Div Dubey, M.B.B.S., explains Mayo Clinic Laboratories' phenotypic testing approach for autoimmune axonal neuropathy. The comprehensive, serologic panel detects for antibodies with clinical relevance to the disease to provide clarity on etiology, prognosis, and treatment choices.
Comprehensive Antibody Panel
Our comprehensive antibody panel uses multiple leading-edge methods to detect 19 clinically actionable autoantibodies related to autoimmune and paraneoplastic neuropathies with axonal and demyelinating causes.
By including antibodies targeting peripheral nerves, dorsal root ganglia, and nodal and paranodal antigens, this evaluation supports diagnosis across a range of neuropathy phenotypes, including subacute, progressive, atypical, or asymmetric presentations; autonomic involvement; and suspected immune-mediated or paraneoplastic causes.
The panel includes CASPR1, contactin-1 (CNTN1), and their antibody complex to broaden the detection of antigen-specific autoimmune nodopathies. It helps distinguish chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) from mimics and identify Guillain-Barré syndrome (GBS), vasculitis, and other nodopathies.
- CASPR1 antibodies are often associated with rapidly progressive sensorimotor neuropathy characterized by prominent neuropathic pain, distal weakness, and sensory symptoms.
- CASPR1/contactin-1 complex antibodies are linked to prominent sensory ataxia, often with acute or subacute onset, asymmetric distribution, and occasional GBS-like features.
- NF155 antibodies are typically associated with younger age at onset, distal weakness, pronounced sensory ataxia, and tremor.
- CNTN1 antibodies are often linked to older age at onset, severe sensorimotor involvement, and sensory ataxia. Associated membranous nephropathy or proteinuria may provide additional supporting evidence.
In cases of antibody positivity, reflex testing is performed to confirm and further characterize the antibody, which enhances the diagnostic sensitivity of the assay.
Key testing
- CNES | Peripheral Nervous System Comprehensive Neuropathy, Autoimmune Evaluation, Serum
- Supports diagnosis of patients with suspected immune-mediated peripheral neuropathy.
- Enables informed treatment decisions.
- Includes a carefully curated panel of clinically actionable autoantibodies associated with axonal and demyelinating causes of peripheral neuropathy (e.g., CASPR1, CNTN1, NF155).
- Automatically reflexes when positive to confirmatory testing.
References
- Dubey D, Lennon V, Gadoth A, et al. Autoimmune CRMP5 neuropathy phenotype and outcome defined from 105 cases. Neurology. 2018 Jan 9;90(2):e103-e110. doi:10.1212/WNL.0000000000004803. Epub 2017 Dec 8.
- Dubey D, Jitprapaikulsan J, Bi H, et al. Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome. Neurology. 2019 Nov 12;93(20):e1873-e1880. doi:10.1212/WNL.0000000000008472. Epub 2019 Oct 17.
- McKeon A, Pittock S. Paraneoplastic encephalomyelopathies: pathology and mechanisms. Acta Neuropathol.2011 Oct;122(4):381-400. doi:10.1007/s00401-011-0876-1. Epub 2011 Sep 22.