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Test Code AIAES Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum


Ordering Guidance


Multiple neurological phenotype-specific autoimmune/paraneoplastic evaluations are available. For more information as well as phenotype-specific testing options, see Autoimmune Neurology Test Ordering Guide.

 

When more than one evaluation is ordered on the same order number, the duplicate test will be canceled.

 

For a list of antibodies performed with each evaluation, see Autoimmune Neurology Antibody Matrix.

 

This test should not be requested for patients who have recently received radioisotopes, therapeutically or diagnostically, because of potential assay interference. The specific waiting period before specimen collection will depend on the isotope administered, the dose given, and the clearance rate in the individual patient. Specimens will be screened for radioactivity prior to analysis. Radioactive specimens received in the laboratory will be held 1 week and assayed if sufficiently decayed or canceled if radioactivity remains.



Necessary Information


Provide the following information:

-Relevant clinical information

-Ordering provider name, phone number, mailing address, and e-mail address



Specimen Required


Patient Preparation:

For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication or intravenous immunoglobulin (IVIg) treatment.

Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 4 mL

Collection Instructions: Centrifuge and aliquot serum into a plastic vial.


Useful For

Evaluation of patients who present with a subacute neurological disorder of undetermined etiology, especially those with known risk factors for cancer

 

Directing a focused search for cancer

 

Investigating neurological symptoms that appear during, or after, cancer therapy, and are not explainable by metastasis

 

Differentiating autoimmune neuropathies from neurotoxic effects of chemotherapy

 

Detecting early evidence of cancer recurrence in previously seropositive patients

Profile Information

Test ID Reporting Name Available Separately Always Performed
AIAEI Autoimmune Axonal Interp, S No Yes
AMPHS Amphiphysin Ab, S No Yes
ANN1S Anti-Neuronal Nuclear Ab, Type 1 No Yes
ANN3S Anti-Neuronal Nuclear Ab, Type 3 No Yes
AGN1S Anti-Glial Nuclear Ab, Type 1 No Yes
APBIS AP3B2 IFA, S No Yes
CS2CS CASPR2-IgG CBA, S No Yes
CRMWS CRMP-5-IgG Western Blot, S Yes Yes
GFAIS GFAP IFA, S No Yes
IG5CS IgLON5 CBA, S No Yes
LG1CS LGI1-IgG CBA, S No Yes
NIFIS NIF IFA, S No Yes
PCABP Purkinje Cell Cytoplasmic Ab Type 1 No Yes
PCAB2 Purkinje Cell Cytoplasmic Ab Type 2 No Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AGNBS AGNA-1 Immunoblot, S No No
AINCS Alpha Internexin CBA, S No No
AMIBS Amphiphysin Immunoblot, S No No
AN1BS ANNA-1 Immunoblot, S No No
AN1TS ANNA-1 Titer, S No No
AN2BS ANNA-2 Immunoblot, S No No
AN3TS ANNA-3 Titer, S No No
APBCS AP3B2 CBA, S No No
APBTS AP3B2 IFA Titer, S No No
APHTS Amphiphysin Ab Titer, S No No
CRMTS CRMP-5-IgG Titer, S No No
GFACS GFAP CBA, S No No
GFATS GFAP IFA Titer, S No No
NFHCS NIF Heavy Chain CBA, S No No
NFLCS NIF Light Chain CBA, S No No
NIFTS NIF IFA Titer, S No No
PC1BS PCA-1 Immunoblot, S No No
PC1TS PCA-1 Titer, S No No
PC2TS PCA-2 Titer, S No No
AGNTS AGNA-1 Titer, S No No
IG5TS IgLON5 IFA Titer, S No No

Testing Algorithm

If the indirect immunofluorescence assay (IFA) patterns suggest antiglial nuclear antibody-1 (AGNA-1), then the AGNA-1 antibody IFA titer and AGNA-1 antibody immunoblot will be performed at an additional charge.

 

If the IFA patterns suggest antineuronal nuclear antibody type 1 (ANNA-1), then the ANNA-1 IFA titer, ANNA-1 immunoblot, and ANNA-2 immunoblot will be performed at an additional charge.

 

If the client requests or the IFA pattern suggests ANNA-3 antibody, then the ANNA-3 IFA titer will be performed at an additional charge.

 

If the IFA pattern suggests Purkinje cytoplasmic antibody type 1 (PCA-1), then the PCA-1 antibody IFA titer and PCA-1 antibody immunoblot will be performed at an additional charge.

 

If the IFA pattern suggests PCA-2 antibody, then the PCA-2 antibody IFA titer will be performed at an additional charge.

 

If the IFA patterns suggest amphiphysin antibody, then the amphiphysin antibody IFA titer and amphiphysin antibody immunoblot will be performed at an additional charge.

 

If the IFA pattern suggests glial fibrillary acidic protein (GFAP) antibody, then the GFAP antibody cell binding assay (CBA) and GFAP antibody IFA titer will be performed at an additional charge.

 

If the collapsin response-mediator protein-5 (CRMP-5)-IgG antibody Western blot is positive, then the CRMP-5-IgG antibody IF titer will be performed at an additional charge.

 

If the IFA pattern suggests adaptor protein 3 beta 2 (AP3B2) antibody, then the AP3B2 antibody IFA titer and AP3B2 antibody CBA will be performed at an additional charge.

 

If the IFA pattern suggests neuronal intermediate filament (NIF) antibody, then the alpha internexin CBA, NIF heavy chain CBA, NIF light chain CBA, and NIF IFA titer will be performed at an additional charge.

 

If the IgLON family member 5 antibody (IgLON5) by CBA result is positive, then the IgLON5 antibody IFA titer will be performed at an additional charge.

 

For more information see:

-Autoimmune/Paraneoplastic Axonal Neuropathy Evaluation Algorithm.

-Acquired Neuropathy Diagnostic Algorithm

Method Name

APBCS, CS2CS, LG1CS, AINCS, NFLCS, NFHCS, GFACS, IG5CS: Cell Binding Assay (CBA)

AGN1S, AGNTS, AMPHS, APHTS, ANN1S, AN1TS, ANN3S, AN3TS, APBIS, APBTS, NIFIS, NIFTS, PCABP, PCAB2, PC1TS, PC2TS, GFAIS, GFATS, CRMTS, IG5TS: Indirect Immunofluorescence Assay (IFA)

 

CRMWS; Western Blot (WB)

 

AGNBS, AMIBS, AN1BS, PC1BS, AN2BS: Immunoblot (IB)

Reporting Name

Axonal, Autoimm/Paraneo, S

Specimen Type

Serum

Specimen Minimum Volume

2 mL

Specimen Stability Information

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

Reference Values

Test ID

Reporting name

Methodology

Reference value

AIAEI

Autoimmune Axonal Interp, S

Medical interpretation

NA

AGN1S

Anti-Glial Nuclear Ab, Type 1

IFA

Negative

AMPHS

Amphiphysin Ab, S

IFA

Negative

ANN1S

ANNA-1, S

IFA

Negative

ANN3S

ANNA-3, S

IFA

Negative

APBIS

AP3B2 IFA, S

IFA

Negative

CRMWS

CRMP-5-IgG Western Blot, S

WB

Negative

CS2CS

CASPR2-IgG CBA, S

CBA

Negative

IG5CS

IgLON5 CBA, S

CBA

Negative

LG1CS

LGI1-IgG CBA, S

CBA

Negative

NIFIS

NIF IFA, S

IFA

Negative

PCAB2

PCA-2, S

IFA

Negative

PCABP

PCA-1, S

IFA

Negative

GFAIS

GFAP IFA, S

IFA

Negative

 

Reflex Information:

Test ID

Reporting name

Methodology

Reference value

AGNBS

AGNA-1 Immunoblot, S

IB

Negative

AGNTS

AGNA-1 Titer, S

IFA

<1:240

AINCS

Alpha Internexin CBA, S

CBA

Negative

AMIBS

Amphiphysin Immunoblot, S

IB

Negative

AN1BS

ANNA-1 Immunoblot, S

IB

Negative

AN1TS

ANNA-1 Titer, S

IFA

<1:240

AN2BS

ANNA-2 Immunoblot, S

IB

Negative

AN3TS

ANNA-3 Titer, S

IFA

<1:240

APBCS

AP3B2 CBA, S

CBA

Negative

APBTS

AP3B2 IFA Titer, S

IFA

<1:240

APHTS

Amphiphysin Ab Titer, S

IFA

<1:240

CRMTS

CRMP-5-IgG Titer, S

IFA

<1:240

GFACS

GFAP CBA, S

CBA

Negative

GFATS

GFAP IFA Titer, S

IFA

<1:240

IG5TS

IgLON5 IFA Titer, S

IFA

<1:240

NFHCS

NIF Heavy Chain CBA, S

CBA

Negative

NFLCS

NIF Light Chain CBA, S

CBA

Negative

NIFTS

NIF IFA Titer, S

IFA

<1:240

PC1BS

PCA-1 Immunoblot, S

IB

Negative

PC1TS

PCA-1 Titer, S

IFA

<1:240

PC2TS

PCA-2 Titer, S

IFA

<1:240

 

*Methodology abbreviations:

Immunofluorescence assay (IFA)

Cell-binding assay (CBA)

Western blot (WB)

Immunoblot (IB)

 

Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, CRMP-5-IgG, PCA-1, or PCA-2 may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

Interpretation

Antibodies directed at onconeural proteins shared by neurons, glia, muscle, and certain cancers are valuable serological markers of a patient's immune response to cancer. They are not found in healthy subjects and are usually accompanied by subacute neurological signs and symptoms. Several autoantibodies have a syndromic association, but no autoantibody predicts a specific neurological syndrome. More than one paraneoplastic autoantibody may be detected and associated with specific cancers.

Clinical Reference

1. Klein CJ Autoimmune-mediated peripheral neuropathies and autoimmune pain. In: Pittock SJ, Vincent A, eds. Autoimmune Neurology. Elsevier; 2016:417-446. Aminoff MJ, Boller F, Swaab DF, eds. Handbook of Clinical Neurology; vol 133

2. Cutsforth-Gregory JK, McKeon A, Coon EA, et al. Ganglionic antibody level as a predictor of severity of autonomic failure. Mayo Clin Proc. 2018;93(10):1440-1447

3. Wei YC, Huang CC, Liu CH, Kuo HC, Lin JJ. Peripheral neuropathy in limbic encephalitis with anti-glutamate receptor antibodies: Case report and systematic literature review. Brain Behav. 2017;7(9):e00779

4. Lucchinetti CF, Kimmel DW, Lennon VA. Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. Neurology. 1998;50(3):652-657. doi:10.1212/wnl.50.3.652

5. Pittock SJ, Lucchinetti CF, Lennon VA. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments. Ann Neurol. 2003;53(5):580-587

6. Chan KH, Vernino S, Lennon VA. ANNA-3 anti-neuronal nuclear antibody: marker of lung cancer-related autoimmunity. Ann Neurol. 2001;50(3):301-311

7. Dubey D, Lennon VA, Gadoth A, et al. Autoimmune CRMP5 neuropathy phenotype and outcome defined from 105 cases. Neurology. 2018;90(2):e103-e110

8. Gadoth A, Pittock SJ, Dubey D, et al. Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients. Ann Neurol. 2017;82(1):79-92

9. Honnorat J, Trouillas P, Thivolet C, Aguera M, Belin MF. Autoantibodies to glutamate decarboxylase in a patient with cerebellar cortical atrophy, peripheral neuropathy, and slow eye movements. Arch Neurol. 1995;52(5):462-468

10. McKeon A, Tracy JA. GAD65 neurological autoimmunity. Muscle Nerve. 2017;56(1):15-27

11. Bradshaw MJ, Haluska P, McKeon A, Klein CJ. Multifocal neuropathy as the presenting symptom of Purkinje cell cytoplasmic autoantibody-1. Muscle Nerve. 2013;48:827-831

12. Pittock SJ, Lucchinetti CF, Parisi JE, et al. Amphiphysin autoimmunity: paraneoplastic accompaniments. Ann Neurol. 2005;58(1):96-107

Day(s) Performed

Profile tests: Monday through Sunday; Reflex tests: Varies

Report Available

8 to 12 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

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.

CPT Code Information

86255 x 12

84182

84182 AGNBS (if appropriate)

86256 AGNTS (if appropriate)

86255 AINCS (if appropriate)

84182 AMIBS (if appropriate)

84182 AN1BS (if appropriate)

86256 AN1TS (if appropriate)

84182 AN2BS (if appropriate)

86256 AN3TS (if appropriate)

86255 APBCS (if appropriate)

86256 APBTS (if appropriate)

86256 APHTS (if appropriate)

86256 CRMTS (if appropriate)

86255 GFACS (if appropriate)

86256 GFATS (if appropriate)

86256 IG5TS (if appropriate)

86255 NFHCS (if appropriate)

86255 NFLCS (if appropriate)

86256 NIFTS (if appropriate)

84182 PC1BS (if appropriate)

86256 PC1TS (if appropriate)

86256 PC2TS (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
AIAES Axonal, Autoimm/Paraneo, S 94695-4

 

Result ID Test Result Name Result LOINC Value
606950 IgLON5 CBA, S 96478-3
615863 AP3B2 IFA, S 101907-4
606964 NIF IFA, S 96486-6
89080 AGNA-1, S 84927-3
81722 Amphiphysin Ab, S 72327-0
80150 ANNA-1, S 33615-6
83137 ANNA-3, S 43102-3
83107 CRMP-5-IgG Western Blot, S 47401-5
83138 PCA-2, S 84925-7
9477 PCA-1, S 84924-0
64279 LGI1-IgG CBA, S 94287-0
64281 CASPR2-IgG CBA, S 94285-4
605155 GFAP IFA, S 94346-4
606975 Autoimmune Axonal Interp, S 69048-7
618900 IFA Notes 48767-8