Evaluating for chronic inflammatory demyelinating polyradiculoneuropathy and related demyelinating peripheral neuropathies
Determining contactin-1 IgG results as a part of a profile
Only orderable as part of a profile. For more information see:
-CIDP / Chronic Inflammatory Demyelinating Polyradiculoneuropathy/Nodopathy Evaluation, Serum
-DMNES / Peripheral Nervous System Demyelinating Neuropathy, Autoimmune Evaluation, Serum
Cell Binding Assay (CBA)
Nodopathy
Paranodopathy
CIDP
CNTN1
Contactin-1
Contactin
Demyelinating neuropathy
Polyneuropathy
Polyradiculopathy
Polyradiculoneuropathy
Immune Neuropathy
Autoimmune Peripheral neuropathy
CISP
Anti-CNTN1
Serum
Only orderable as part of a profile. For more information see:
-CIDP / Chronic Inflammatory Demyelinating Polyradiculoneuropathy/Nodopathy Evaluation, Serum
-DMNES / Peripheral Nervous System Demyelinating Neuropathy, Autoimmune Evaluation, Serum
0.5 mL
Gross hemolysis | Reject |
Gross lipemia | Reject |
Gross icterus | Reject |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 28 days | |
Frozen | 28 days | ||
Ambient | 72 hours |
Evaluating for chronic inflammatory demyelinating polyradiculoneuropathy and related demyelinating peripheral neuropathies
Determining contactin-1 IgG results as a part of a profile
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated condition effecting peripheral nerves and nerve roots and is characterized by electrodiagnostic features of demyelination with a chronic onset that leads to significant disability. The prevalence of CIDP is estimated at approximately 2 to 4 cases per 100,000 persons. Although a rarer cause of polyneuropathy, it is important to recognize as it is treatable with the appropriate use of immunomodulating therapies. Although the exact immunological trigger of CIDP remains unclear, a subset of patients with suspected CIDP have been identified with autoantibodies targeting nodal-paranodal proteins. These patients share common immunopathological mechanisms of disease, clinical features, and treatment responses that are distinct from classic CIDP. A common target of these autoantibodies is the neurofascin-155 (NF155)-contactin-1 (CNTN1) complex. NF155 is expressed at the paranodal loops of Schwann cells where it interacts with CNTN1 expressed on adjacent axons. This interaction stabilizes and allows the proper organization of the paranodal axoglial junction. Antibody-mediated disruption of this interaction in animal models recapitulates the pathophysiology observed in humans.
Contactin-1 IgG antibodies are present in approximately 2% of patients with CIDP like presentations. CNTN1 IgG-positive cases are more likely to present with neuropathic pain, sensory ataxia, and subacute progressive demyelinating polyradiculoneuropathy or polyradiculopathy. The majority of seropositive patients have been reported to be refractory to treatment with intravenous immune globulin. However, some of these patients respond well to B-cell depleting therapies such as rituximab. Up to half of CNTN1 IgG-positive patients with CIDP or CIDP-like presentations have been reported to develop membranous nephropathy and, thus, screening for proteinuria may be warranted.
Only orderable as part of a profile. For more information see:
-CIDP / Chronic Inflammatory Demyelinating Polyradiculoneuropathy/Nodopathy Evaluation, Serum
-DMNES / Peripheral Nervous System Demyelinating Neuropathy, Autoimmune Evaluation, Serum
Negative
Seropositivity for contactin-1 IgG is consistent with an immune-mediated demyelinating polyradiculoneuropathy/polyradiculopathy.
A negative result does not rule out an immune-mediated demyelinating disease.
This test should only be utilized in the appropriate clinical context.
The use of immunotherapy prior to sample collection may negatively impact the sensitivity of these assays.
1. Dubey D, Honorat JA, Shelly S, et al: Contactin-1 autoimmunity: Serologic, neurologic, and pathologic correlates. Neurol Neuroimmunol Neuroinflamm. 2020;7(4):e771
2. Cortese A, Lombardi R, Briani C, et al. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. Neurol Neuroimmunol Neuroinflamm. 2020;7(1):e639
3. Manso C, Querol L, Mekaouche M, Illa I, Devaux JJ. Contactin-1 IgG4 antibodies cause paranode dismantling and conduction defects. Brain. 2016;139(Pt 6):1700-1712
4. Le Quintrec M, Teisseyre M, Bec N, et al. Contactin-1 is a novel target antigen in membranous nephropathy associated with chronic inflammatory demyelinating polyneuropathy. Kidney Int. 2021;100(6):1240-1249
5. Ogata H, Yamasaki R, Hiwatashi A, et al. Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy. Ann Clin Transl Neurol. 2015;2(10):960-971
6. Cortese A, Lombardi R, Briani C, et al. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. Neurol Neuroimmunol Neuroinflamm. 2020;7(1):e639
7. Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology. 2014;82(10):879-886
8. Shelly S, Klein CJ, Dyck PJB, et al. Neurofascin-155 immunoglobulin subtypes: Clinicopathologic associations and neurologic outcomes. Neurology. 2021;97(24):e2392-e2403
Patient specimen is applied to a composite slide containing transfected and nontransfected HEK-293 cells. After incubation and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the presence of patient IgG binding.(Package insert: IIFT: Neurology Mosaics, Instructions for the indirect immunofluorescence test. EUROIMMUN; FA_112d-1_A_UK_C13, 02/2019)
Monday through Thursday, Sunday
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.
86255
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
CONCS | Contactin-1 IgG CBA, S | 101448-9 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
616442 | Contactin-1 IgG CBA, S | 101448-9 |