Test Catalog

Test Id : CIDP

Chronic Inflammatory Demyelinating Polyradiculoneuropathy/Nodopathy Evaluation, Serum

Useful For
Suggests clinical disorders or settings where the test may be helpful

Evaluating for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and related demyelinating peripheral neuropathies

Profile Information
A profile is a group of laboratory tests that are ordered and performed together under a single Mayo Test ID. Profile information lists the test performed, inclusive of the test fee, when a profile is ordered and includes reporting names and individual availability.

Test Id Reporting Name Available Separately Always Performed
CIDPI CIDP/NP Interpretation, S No Yes
CONCS Contactin-1 IgG CBA, S No Yes
NF4FS Neurofascin-155 IgG4, S No Yes

Method Name
A short description of the method used to perform the test

CONCS: Cell Binding Assay (CBA)

NF4FS: Flow Cytometry (FCM)

CIDPI: Medical Interpretation

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

CIDP/NP Evaluation, S

Aliases
Lists additional common names for a test, as an aid in searching

Nodopathy

Paranodopathy

CIDP

CNTN1

Contactin-1

Contactin

Demyelinating neuropathy

Polyneuropathy

Polyradiculopathy

Polyradiculoneuropathy

Immune Neuropathy

Autoimmune Peripheral neuropathy

CISP

Anti-CNTN1

Specimen Type
Describes the specimen type validated for testing

Serum

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Patient Preparation: For optimal antibody detection, it is recommended to collect the specimen before initiation of immunosuppressant medication.

Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Specimen Volume: 3 mL

Forms

If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

2 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus Reject

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 28 days
Frozen 28 days
Ambient 72 hours

Useful For
Suggests clinical disorders or settings where the test may be helpful

Evaluating for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and related demyelinating peripheral neuropathies

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

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.

 

NF155 IgG antibodies are present in approximately 5% to 10% of patients with CIDP like presentations and, more rarely, in those with more acute forms of demyelinating polyradiculoneuropathy. NF155 IgG positive cases are more likely to present with distal weakness, gait disturbance, tremor, and dysarthria as compared to classic CIDP. Most patients who are seropositive for NF155 IgG have been reported to be refractory to intravenous immune globulin (IVIG) therapy and often require second line treatment that includes B-cell depleting therapies such as rituximab. Studies in animal models, as well as the disease pathology indicate NF155 IgG4 antibodies directly disrupt the paranodal axoglial junction ultimately leading to demyelination. IgG4 is the predominant antibody subclass found in these patients and associates with poorer treatment responses to IVIG. The detection of NF155 IgG4 is a highly specific finding and has not been reported in other disease mimics such as hereditary neuropathies, distal acquired demyelinating symmetric neuropathy, and motor neuron disease.

 

CNTN1 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 IVIG. 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.

Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

Contactin-1 IgG: Negative

Neurofascin-155 IgG4: Negative

Interpretation
Provides information to assist in interpretation of the test results

Seropositivity for contactin-1 IgG is consistent with an immune-mediated demyelinating polyradiculoneuropathy/polyradiculopathy.

 

Seropositivity for neurofascin-155 IgG4 is consistent with an immune-mediated demyelinating polyradiculoneuropathy.

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

A negative result does not exclude an immune mediated demyelinating neuropathy.

 

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 this assay.

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. Dubey D, Honorat JA, Shelly S, et al: Contactin-1 autoimmunity: Serologic, neurologic, and pathologic correlates. Neurol Neuroimmunol Neuroinflamm. 2020 May 27;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 Nov 21;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 Jun;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 Dec;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 Oct;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 Nov 21;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 Mar 11;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

Method Description
Describes how the test is performed and provides a method-specific reference

Contactin-1 IgG:

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)

 

Neurofascin-155 IgG4:

This cell-binding assay utilizes flow cytometry to detect neurofascin 155 (NF155) IgG4 antibodies in patient sera. Briefly, a stable HEK293 cell line expressing human NF155 on the cell surface is premixed with parental HEK293 cells that do not express human NF155. The 2 cell populations are distinguished using a green fluorescent protein marker, which is only expressed in NF155 expressing cells. The mixture of cells is incubated with diluted patient sera to allow antibodies present in the sample to bind target antigens. Next the cells are incubated with a human IgG4 specific secondary antibody conjugated to AlexaFluor 647 to detect cell bound human IgG4 antibodies. The AlexaFluor 647 signal intensity of the different cell populations is measured using a flow cytometer. The IBI (IgG binding index) is then calculated as the median fluorescent intensity (MFI) of AlexaFluor 647 of the NF155 expressing cells divided by the MFI of the parental non-NF155 expressing cells. When the IBI is greater than or equal to 2.0 the result is considered positive for NF155 IgG4 antibodies.(Unpublished Mayo method)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

CONCS: Monday through Thursday, Sunday

NF4FS: Monday, Friday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

5 to 10 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

28 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

  • Authorized users can sign in to Test Prices for detailed fee information.
  • Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
  • Prospective clients should contact their Regional Manager. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

86255 x 2

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
CIDP CIDP/NP Evaluation, S In Process
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.
614591 Neurofascin-155 IgG4, S 100845-7
616444 CIDP/NP Interpretation, S 69048-7
616442 Contactin-1 IgG CBA, S In Process

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports

Test Update Resources

Change Type Effective Date
New Test 2022-08-16