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Test ID: PNEFC Neuroimmunology Antibody Follow-up, Spinal Fluid

Reporting Name

Neuroimmunology Ab Follow-up, CSF

Useful For

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

Specimen Type

CSF


Ordering Guidance


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

-DMC2 / Dementia, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid

-ENC2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid

-EPC2 / Epilepsy, Autoimmune/Paraneoplastic Evaluation, Serum

-MAC1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid

-MDC2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid

-PCDEC / Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid

-SPPC / Stiff-Person Spectrum Disorders/PERM Evaluation, Spinal Fluid

 

This test should not be requested in 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.



Specimen Required


Container/Tube: Sterile vial

Preferred: Vial number 1

Acceptable: Any vial

Specimen Volume: 4 mL


Specimen Minimum Volume

2 mL

Specimen Stability Information

Specimen Type Temperature Time
CSF Refrigerated (preferred) 28 days
  Frozen  28 days
  Ambient  72 hours

Reference Values

Test ID

Reporting Name

Methodology*

Reference Value

AGNBC

AGNA-1 Immunoblot, CSF

IB

Negative

AINCC

Alpha Internexin CBA, CSF

CBA

Negative

AMPCC

AMPA-R Ab CBA, CSF

CBA

Negative

AMPHC

Amphiphysin Ab, CSF

IFA

Negative

AMIBC

Amphiphysin Immunoblot, CSF

IB

Negative

AN1BC

ANNA-1 Immunoblot, CSF

IB

Negative

AN2BC

ANNA-2 Immunoblot, CSF

IB

Negative

AGN1C

Anti-Glial Nuclear Ab Type 1

IFA

Negative

ANN1C

Anti-Neuronal Nuclear Ab, Type 1

IFA

Negative

ANN2C

Anti-Neuronal Nuclear Ab, Type 2

IFA

Negative

ANN3C

Anti-Neuronal Nuclear Ab, Type 3

IFA

Negative

APBCC

AP3B2 CBA, CSF

CBA

Negative

APBIC

AP3B2 IFA, CSF

IFA

Negative

CS2CC

CASPR2-IgG CBA, CSF

CBA

Negative

CRMWC

CRMP-5-IgG Western Blot, CSF

WB

Negative

CRMC

CRMP-5-IgG, CSF

IFA

Negative

DPPCC

DPPX Ab CBA, CSF

CBA

Negative

DPPIC

DPPX Ab IFA, CSF

IFA

Negative

GABCC

GABA-B-R Ab CBA, CSF

CBA

Negative

GFACC

GFAP CBA, CSF

CBA

Negative

GFAIC

GFAP IFA, CSF

IFA

Negative

GRFCC

GRAF1 CBA, CSF

CBA

Negative

GRFIC

GRAF1 IFA, CSF

IFA

Negative

IG5CC

IgLON5 CBA, CSF

CBA

Negative

IG5IC

IgLON5 IFA, CSF

IFA

Negative

ITPCC

ITPR1 CBA, CSF

CBA

Negative

ITPIC

ITPR1 IFA, CSF

IFA

Negative

LG1CC

LGI1-IgG CBA, CSF

CBA

Negative

GL1CC

mGluR1 Ab CBA, CSF

CBA

Negative

GL1IC

mGluR1 Ab IFA, CSF

IFA

Negative

NCDCC

Neurochondrin CBA, CSF

CBA

Negative

NCDIC

Neurochondrin IFA, CSF

IFA

Negative

NFHCC

NIF Heavy Chain CBA, CSF

CBA

Negative

NIFIC

NIF IFA, CSF

IFA

Negative

NFLCC

NIF Light Chain CBA, CSF

CBA

Negative

NMDCC

NMDA-R Ab CBA, CSF

CBA

Negative

PC1BC

PCA-1 Immunoblot, CSF

IB

Negative

PCTBC

PCA-Tr Immunoblot, CSF

IB

Negative

PCTRC

Purkinje Cell Cytoplasmc Ab Type Tr

IFA

Negative

PCA1C

Purkinje Cell Cytoplasmic Ab Type 1

IFA

Negative

PCA2C

Purkinje Cell Cytoplasmic Ab Type 2

IFA

Negative

SP5CC

Septin-5 CBA, CSF

CBA

Negative

SP5IC

Septin-5 IFA, CSF

IFA

Negative

SP7CC

Septin-7 CBA, CSF

CBA

Negative

SP7IC

Septin-7 IFA, CSF

IFA

Negative

PDEIC

PDE10A Ab IFA, CSF

IFA

Negative

T46CC

TRIM46 Ab CBA, CSF

CBA

Negative

T46IC

TRIM46 Ab IFA, CSF

IFA

Negative

 

*Methodology abbreviations:

CBA: Cell-binding assay

IB: Immunoblot

IFA: Immunofluorescence assay

WB: Western blot (WB)

Day(s) Performed

Varies

CPT Code Information

84182 AGNBC (if appropriate)

86255 AINCC (if appropriate)

86255 AMPCC (if appropriate)

86255 AMPHC (if appropriate)

84182 AMIBC (if appropriate)

84182 AN1BC (if appropriate)

84182 AN2BC (if appropriate)

86255 AGN1C (if appropriate)

86255 ANN1C (if appropriate)

86255 ANN2C (if appropriate)

86255 ANN3C (if appropriate)

86255 APBCC (if appropriate)

86255 APBIC (if appropriate)

86255 CS2CC (if appropriate)

84182 CRMWC (if appropriate)

86255 CRMC (if appropriate)

86255 DPPCC (if appropriate)

86255 DPPIC (if appropriate)

86255 GABCC (if appropriate)

86255 GFACC (if appropriate)

86255 GFAIC (if appropriate)

86255 GRFCC (if appropriate)

86255 GRFIC (if appropriate)

86255 IG5CC (if appropriate)

86255 IG5IC (if appropriate)

86255 ITPCC (if appropriate)

86255 ITPIC (if appropriate)

86255 LG1CC (if appropriate)

86255 GL1CC (if appropriate)

86255 GL1IC (if appropriate)

86255 NCDCC (if appropriate)

86255 NCDIC (if appropriate)

86255 NFHCC (if appropriate)

86255 NIFIC (if appropriate)

86255 NFLCC (if appropriate)

86255 NMDCC (if appropriate)

84182 PC1BC (if appropriate)

84182 PCTBC (if appropriate)

86255 PCTRC (if appropriate)

86255 PCA1C (if appropriate)

86255 PCA2C (if appropriate)

86255 PDEIC (if appropriate)

86255 SP5CC (if appropriate)

86255 SP5IC (if appropriate)

86255 SP7CC (if appropriate)

86255 SP7IC (if appropriate)

86255 T46CC (if appropriate)

86255 T46IC (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
PNEFC Neuroimmunology Ab Follow-up, CSF 80615-8

 

Result ID Test Result Name Result LOINC Value
84299 Neuroimmunology Ab Follow-up, CSF 80615-8

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 recognized cancers in this context are small-cell lung carcinoma, 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.

 

Three classes of autoantibodies are recognized in the spinal fluid 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, collapsin response-mediator protein-5 [CRMP-5], and amphiphysin)

-Glial nuclear (antiglial nuclear antibody: AGNA)

 

Patients who are seropositive usually present with subacute neurological signs and symptoms. 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, 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 signs and symptoms. 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 one paraneoplastic autoantibodies to be detected, each predictive of the same cancer.

Report Available

Varies

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AMPCC AMPA-R Ab CBA, CSF No No
AMPHC Amphiphysin Ab, CSF No No
AGN1C Anti-Glial Nuclear Ab, Type 1 No No
ANN1C Anti-Neuronal Nuclear Ab, Type 1 No No
ANN2C Anti-Neuronal Nuclear Ab, Type 2 No No
ANN3C Anti-Neuronal Nuclear Ab, Type 3 No No
CS2CC CASPR2-IgG CBA, CSF No No
CRMWC CRMP-5-IgG Western Blot, CSF No No
CRMC CRMP-5-IgG, CSF No No
GABCC GABA-B-R Ab CBA, CSF No No
LG1CC LGI1-IgG CBA, CSF No No
NMDCC NMDA-R Ab CBA, CSF No No
PCTRC Purkinje Cell Cytoplasmc Ab Type Tr No No
PCA1C Purkinje Cell Cytoplasmic Ab Type 1 No No
PCA2C Purkinje Cell Cytoplasmic Ab Type 2 No No
DPPCC DPPX Ab CBA, CSF No No
DPPIC DPPX Ab IFA, CSF No No
GL1CC mGluR1 Ab CBA, CSF No No
GL1IC mGluR1 Ab IFA, CSF No No
AGNBC AGNA-1 Immunoblot, CSF No No
AINCC Alpha Internexin CBA, CSF No No
AMIBC Amphiphysin Immunoblot, CSF No No
AN1BC ANNA-1 Immunoblot, CSF No No
AN2BC ANNA-2 Immunoblot, CSF No No
APBCC AP3B2 CBA, CSF No No
APBIC AP3B2 IFA, CSF No No
GFACC GFAP CBA, CSF No No
GFAIC GFAP IFA, CSF No No
GRFCC GRAF1 CBA, CSF No No
GRFIC GRAF1 IFA, CSF No No
IG5CC IgLON5 CBA, CSF No No
IG5IC IgLON5 IFA, CSF No No
ITPCC ITPR1 CBA, CSF No No
ITPIC ITPR1 IFA, CSF No No
NCDIC Neurochondrin IFA, CSF No No
NCDCC Neurochondrin CBA, CSF No No
NFHCC NIF Heavy Chain CBA, CSF No No
NIFIC NIF IFA, CSF No No
NFLCC NIF Light Chain CBA, CSF No No
PC1BC PCA-1 Immunoblot, CSF No No
PCTBC PCA-Tr Immunoblot, CSF No No
SP5CC Septin-5 CBA, CSF No No
SP5IC Septin-5 IFA, CSF No No
SP7CC Septin-7 CBA, CSF No No
SP7IC Septin-7 IFA, CSF No No
PDEIC PDE10A Ab IFA, CSF No No
T46CC TRIM46 Ab CBA, CSF No No
T46IC TRIM46 Ab IFA, CSF No No

Method Name

AMPHC, AGN1C, ANN1C, ANN2C, ANN3C, PCTRC, PCA1C, PCA2C, DPPTC, GL1IC, GFAIC, IG5IC, ITPIC, GRFIC, NIFIC, APBIC, SP5IC, SP7IC, CRMC, NCDIC, DPPIC, PDEIC, T46IC: Indirect Immunofluorescence Assay (IFA)

AMPCC, CS2CC, GABCC, LG1CC, NMDCC, DPPCC, GL1CC, GFACC, IG5CC, ITPCC, GRFCC, NFLCC, NFHCC, AINCC, APBCC, NCDCC, SP5CC, SP7CC, T46CC: Cell Binding Assay (CBA)

CRMWC: Western Blot (WB)

AN1BC, AN2BC, AMIBC, PC1BC, PCTBC, AGNBC: Immunoblot (IB)

Clinical Reference

1. Lancaster E, Martinez-Hernandez E, Dalmau J. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011;77(2):179-189. doi:10.1212/WNL.0b013e318224afde

2. Horta ES, Lennon VA, Lachance DH, et al. Neural autoantibody clusters aid diagnosis of cancer. Clin Cancer Res. 2014;20(14):3862-3869

3. Gilligan M, McGuigan C, McKeon A..Paraneoplastic neurologic Disorders. Curr Neurol Neurosci Rep. 2023;23(3):67-82. doi:10.1007/s11910-023-01250-w

4. Graus F, Vogrig A, Muniz-Castrillo S, et al. Updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm. 2021;8(4):e1014. doi:10.1212/NXI.0000000000001014

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.

Forms

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