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Test ID: PNEFS Neuroimmunology Antibody Follow-up, Serum

Reporting Name

Neuroimmunology Ab Follow-up, S

Useful For

Monitoring patients who have previously tested positive for 1 or more antibodies within the past 5 years in a Mayo Neuroimmunology Laboratory serum evaluation

Clinical Information

Paraneoplastic autoimmune neurological disorders reflect a patient's humoral and cellular immune responses to cancer. The cancer may be new or recurrent, is usually limited in metastatic volume, and is often occult by standard imaging procedures. Autoantibodies specific for onconeural proteins found in the plasma membrane, cytoplasm, and nucleus of neurons or muscle are generated in this immune response and serve as serological markers of paraneoplastic autoimmunity. The most commonly recognized cancers in this context are small-cell lung carcinoma (SCLC), thymoma, ovarian (or related mullerian) carcinoma, breast carcinoma, and Hodgkin lymphoma. Pertinent childhood neoplasms recognized thus far include neuroblastoma, thymoma, Hodgkin lymphoma, and chondroblastoma. An individual patient's autoantibody profile can predict a specific neoplasm with 90% certainty, but not the neurological syndrome.

 

Four classes of autoantibodies are recognized in serum analysis:

-Neuronal nuclear (antineuronal nuclear antibody-type 1 [ANNA-1], ANNA-2, ANNA-3)

-Neuronal and muscle cytoplasmic (Purkinje cell cytoplasmic antibody, type 1 [PCA-1], PCA-2, PCA-Tr, CRMP-5, amphiphysin, and striational)

-Glial nuclear (antiglial nuclear antibody)

-Plasma membrane cation channel antibodies (neuronal P/Q-type and N-type calcium channel and muscle acetylcholine receptor autoantibodies). These autoantibodies are potential effectors of neurological dysfunction.

 

Seropositive patients usually present with subacute neurological symptoms and signs. The patient may present with encephalopathy, cerebellar ataxia, myelopathy, radiculopathy, plexopathy, sensory, sensorimotor, or autonomic neuropathy, with or without coexisting evidence of a neuromuscular transmission disorder: Lambert-Eaton syndrome (LES), myasthenia gravis, or neuromuscular hyperexcitability. Initial signs may be subtle, but a subacute multifocal and progressive syndrome usually evolves. Sensorimotor neuropathy and cerebellar ataxia are common presentations, but the clinical picture in some patients is dominated by striking gastrointestinal dysmotility, limbic encephalopathy, basal ganglionitis, or cranial neuropathy (especially loss of vision, hearing, smell, or taste). Cancer risk factors include past or family history of cancer, history of smoking or social/environmental exposure to carcinogens. Early diagnosis and treatment of the neoplasm favor less neurological morbidity and offer the best hope for survival.

Interpretation

Antibodies directed at onconeural proteins shared by neurons, 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 symptoms and signs. Several autoantibodies have a syndromic association, but no known autoantibody predicts a specific neurological syndrome. Conversely, a positive autoantibody profile has 80% to 90% predictive value for a specific cancer. It is not uncommon for more than 1 paraneoplastic autoantibody to be detected, each predictive of the same cancer.

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
ARMO ACh Receptor (Muscle) Modulating Ab No No
GANG AChR Ganglionic Neuronal Ab, S No No
AMPCS AMPA-R Ab CBA, S No No
AMPIS AMPA-R Ab IF Titer Assay, S No No
AMPHS Amphiphysin Ab, S No No
AGN1S Anti-Glial Nuclear Ab, Type 1 No No
ANN1S Anti-Neuronal Nuclear Ab, Type 1 No No
ANN2S Anti-Neuronal Nuclear Ab, Type 2 No No
ANN3S Anti-Neuronal Nuclear Ab, Type 3 No No
CS2CS CASPR2-IgG CBA, S No No
CRMS CRMP-5-IgG, S No No
DPPCS DPPX Ab CBA, S No No
DPPTS DPPX Ab IFA Titer, S No No
DPPIS DPPX Ab IFA, S No No
GABCS GABA-B-R Ab CBA, S No No
GABIS GABA-B-R Ab IF Titer Assay, S No No
LG1CS LGI1-IgG CBA, S No No
GL1CS mGluR1 Ab CBA, S No No
GL1TS mGluR1 Ab IFA Titer, S No No
GL1IS mGluR1 Ab IFA, S No No
VGKC Neuronal (V-G) K+ Channel Ab, S No No
NMDCS NMDA-R Ab CBA, S No No
NMDIS NMDA-R Ab IF Titer Assay, S No No
CCN N-Type Calcium Channel Ab No No
CCPQ P/Q-Type Calcium Channel Ab No No
PCABP Purkinje Cell Cytoplasmic Ab Type 1 No No
PCAB2 Purkinje Cell Cytoplasmic Ab Type 2 No No
PCATR Purkinje Cell Cytoplasmic Ab Type Tr No No

Analytic Time

Varies

Day(s) and Time(s) Performed

ANN1S, ANN2S, ANN3S, AGN1S, PCABP, PCAB2, PCATR, AMPHS, CRMS, AMPIS, GABIS, NMDIS, DPPIS, DPPTS, GL1IS, GL1TS:

Monday through Friday; 11:30 a.m. and 8 p.m.

Saturday and Sunday 8 a.m.

 

AMPCS, GABCS, NMDCS. LG1CS, CS2CS, DPPCS, GL1CS:

Monday through Friday; 6 a.m.

 

WBN, ABLOT:

Monday, Wednesday, Friday; 8 a.m.

 

CCPQ, CCN, GANG, VGKC:

Monday through Friday; 11 a.m. and 6 p.m.

Saturday, Sunday; 6 a.m.

 

ARMO:

Monday through Thursday, Saturday; 12 p.m.

Sunday; 8 a.m.

Clinical Reference

Lancaster E, Martinez-Hernandez E, Dalmau J: Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology 2011;77(2):179-189

Method Name

ANN1S, ANN2S, ANN3S, AGN1S, PCABP, PCAB2, PCATR, AMPHS, CRMS, AMPIS, GABIS, NMDIS, DPPIS, DPPTS, GL1IS, GL1TS: Indirect Immunofluorescence (IFA)

AMPCS, GABCS, NMDCS, LG1CS, CS2CS, DPPCS, GL1CS: Cell-Binding Assay (CBA)

WBN, ABLOT: Western Blot

CCPQ, CCN, GANG, VGKC, ARMO: Radioimmunoassay (RIA)

Specimen Type

Serum


Advisory Information


This test is only appropriate for follow-up in patients who have previously tested positive in a serum test. If patients have not previously been positive in a serum test, order 1 of the following:

-PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum

-GID1 / Autoimmune Gastrointestinal Dysmotility Evaluation, Serum

-DYS1 / Autoimmune Dysautonomia Evaluation, Serum

-DMS1 / Dementia, Autoimmune Evaluation, Serum

-ENS1 / Encephalopathy, Autoimmune Evaluation, Serum

-EPS1 / Epilepsy, Autoimmune Evaluation, Serum

-MDS1 / Movement Disorder Evaluation, Serum

-MGL1 / Myasthenia Gravis (MG)/Lambert-Eaton Syndrome (LES) Evaluation

-MGA1 / Myasthenia Gravis (MG) Evaluation, Adult

-MGP1 / Myasthenia Gravis (MG) Evaluation, Pediatric

-MGT1 / Myasthenia Gravis (MG) Evaluation, Thymoma

-MGRM / Myasthenia Gravis Evaluation with MuSK Reflex, Serum



Specimen Required


Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: 13- x 75-mm plastic screw-top vial.

Specimen Volume: 4 mL

Collection Instructions: Centrifuge within 2 hours. Aliquot and ship in 13- x 75-mm plastic screw-top vial.


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

Reference Value

GANG

AChR Ganglionic Neuronal Ab, S

<0.02

AMPCS

AMPA-R Ab CBA, S

Negative

AMPIS

AMPA-R Ab IF Titer Assay, S

<1:120

AMPHS

Amphiphysin Ab, S

<1:240

ABLOT

Amphiphysin Western Blot, S

Negative

AGN1S

Anti-Glial Nuclear Ab, Type 1

<1:240

ANN1S

Anti-Neuronal Nuclear Ab, Type 1

<1:240

ANN2S

Anti-Neuronal Nuclear Ab, Type 2

<1:240

ANN3S

Anti-Neuronal Nuclear Ab, Type 3

<1:240

CS2CS

CASPR2-IgG CBA, S

Negative

CRMS

CRMP-5-IgG, S

<1:240

GABCS

GABA-B-R Ab CBA, S

Negative

GABIS

GABA-B-R Ab IF Titer Assay, S

<1:120

LG1CS

LGI1-IgG CBA, S

Negative

VGKC

Neuronal (V-G) K+ Channel Ab, S

<0.02

NMDCS

NMDA-R Ab CBA, S

Negative

NMDIS

NMDA-R Ab IF Titer Assay, S

<1:120

NMOTS

NMO/AQP4 FACS Titer, S

<1:5

CCN

N-Type Calcium Channel Ab

<0.03

CCPQ

P/Q-Type Calcium Channel Ab

<0.02

WBN

Paraneoplastic Autoantibody WBlot,S

Negative

PCABP

Purkinje Cell Cytoplasmic Ab Type 1

<1:240

PCAB2

Purkinje Cell Cytoplasmic Ab Type 2

<1:240

PCATR

Purkinje Cell Cytoplasmic Ab Type Tr

<1:240

Test Classification

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 U.S. Food and Drug Administration.

CPT Code Information

83519-59-ACh receptor (muscle) modulating antibodies (if appropriate)

83519-59-AChR ganglionic neuronal antibody (if appropriate)

83519-59-N-type calcium channel antibody (if appropriate)

83519-59-P/Q-type calcium channel antibody (if appropriate)

83519-VGKC (if appropriate)

84182-CRMP-5-IgG Western blot (if appropriate)

84182-Paraneoplastic autoantibody Western blot confirmation (if appropriate)

86255-Amphiphysin (if appropriate)

86255-ANNA-1 (if appropriate)

86255-ANNA-2 (if appropriate)

86255-ANNA-3 (if appropriate)

86255-CRMP-5-IgG (if appropriate)

86255-PCA-1 (if appropriate)

86255-PCA-2 (if appropriate)

86255-PCA-Tr (if appropriate)

86255-DPPCS (if appropriate)

86255-DPPIS (if appropriate)

86255-GL1CS (if appropriate)

86255-GL1IS (if appropriate)

86255-NMDCS (if appropriate)

86255-AMPCS (if appropriate)

86255-GABCS (if appropriate)

86256-NMDIS (if appropriate)

86256-AMPIS (if appropriate)

86256-GABIS (if appropriate)

86256-DPPTS (if appropriate)

86256-GL1TS (if appropriate)

86255-LG1CS (if appropriate)

86255-CS2CS (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
PNEFS Neuroimmunology Ab Follow-up, S 80615-8

 

Result ID Test Result Name Result LOINC Value
84300 Neuroimmunology Ab Follow-up, S 80615-8

Forms

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

Mayo Clinic Laboratories | Endocrinology Catalog Additional Information:

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