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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


Advisory Information


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 1 of the following:

-PAC1 / Paraneoplastic, Autoantibody Evaluation, Spinal Fluid

-DMC1 / Dementia, Autoimmune Evaluation, Spinal Fluid

-ENC1 / Encephalopathy, Autoimmune Evaluation, Spinal Fluid

-EPC1 / Epilepsy, Autoimmune Evaluation, Spinal Fluid

-MDC1 / Movement Disorder Evaluation, Spinal Fluid



Specimen Required


Container/Tube: Sterile 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

Reference Value

AMPCC

AMPA-R Ab CBA, CSF

Negative

AMPIC

AMPA-R Ab IF Titer Assay, CSF

<1:2

AMPHC

Amphiphysin Ab, CSF

<1:2

ABLTC

Amphiphysin Western Blot, CSF

Negative

AGN1C

Anti-Glial Nuclear Ab, Type 1

<1:2

ANN1C

Anti-Neuronal Nuclear Ab, Type 1

<1:2

ANN2C

Anti-Neuronal Nuclear Ab, Type 2

<1:2

ANN3C

Anti-Neuronal Nuclear Ab, Type 3

<1:2

CS2CC

CASPR2-IgG CBA, CSF

Negative

CRMC

CRMP-5-IgG, CSF

<1:2

GABCC

GABA-B-R Ab CBA, CSF

Negative

GABIC

GABA-B-R Ab IF Titer Assay, CSF

<1:2

LG1CC

LGI1-IgG CBA, CSF

Negative

VGKCC

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

<0.02

NMDCC

NMDA-R Ab CBA, CSF

Negative

NMDIC

NMDA-R Ab IF Titer Assay, CSF

<1:2

NMOTC

NMO/AQP4 FACS Titer, CSF

<1:2

WBNC

Paraneoplastic Autoantibody WBlot, CSF

Negative

PCA1C

Purkinje Cell Cytoplasmic Ab Type 1

<1:2

PCA2C

Purkinje Cell Cytoplasmic Ab Type 2

<1:2

PCTRC

Purkinje Cell Cytoplasmic Ab Type Tr

<1:2

Day(s) and Time(s) Performed

ANN1C, ANN2C, ANN3C, AGN1C, PCA1C, PCA2C, PCTRC, AMPHC, CRMC, NMDIC, AMPIC, GABIC, DPPIC, DPPTC, GL1CC, GL1TC:

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

Saturday and Sunday 8 a.m.

 

AMPCC, GABCC, NMDCC, LG1CC, CS2CC, DPPCC, GL1CC:

Monday through Friday; 6 a.m.

 

WBNC, ABLTC:

Monday, Wednesday, Friday; 8 a.m.

 

VGKC:

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

Saturday, Sunday 6 a.m.

CPT Code Information

84182-Paraneoplastic autoantibody Western blot confirmation (if appropriate)

84182-Amphiphysin 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-AGNA-1 (if appropriate)

86256-AMPIC (if appropriate)

86256-GABIC (if appropriate)

86256-NMDIC (if appropriate)

86255-DPPIC (if appropriate)

86256-DPPTC (if appropriate)

86255-GL1IC (if appropriate)

86256-GL1TC (if appropriate)

86255-AMPCC (if appropriate)

86255-GABCC (if appropriate)

86255-NMDCC (if appropriate)

83519-VGKCC (if appropriate)

86255-LG1CC (if appropriate)

86255-CS2CC (if appropriate)

86255-DPPCC (if appropriate)

86255-GL1CC (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
PNEFC Neuroimmunology Ab Follow-up, CSF In Process

 

Result ID Test Result Name Result LOINC Value
84299 Neuroimmunology Ab Follow-up, CSF In Process

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.

 

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, CRMP-5, and amphiphysin)

-Glial nuclear (antiglial nuclear antibody: AGNA)

 

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 autoantibodies to be detected, each predictive of the same cancer.

Analytic Time

Varies

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AMPCC AMPA-R Ab CBA, CSF No No
AMPIC AMPA-R Ab IF Titer Assay, CSF No No
AMPHC Amphiphysin Ab, CSF No No
ABLTC Amphiphysin Western Blot, 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
GABIC GABA-B-R Ab IF Titer Assay, CSF No No
LG1CC LGI1-IgG CBA, CSF No No
NMDCC NMDA-R Ab CBA, CSF No No
NMDIC NMDA-R Ab IF Titer Assay, CSF No No
WBNC Paraneoplas Autoantibody WBlot,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
VGKCC VGKC-complex Ab IPA, CSF No No
DPPCC DPPX Ab CBA, CSF No No
DPPIC DPPX Ab IFA, CSF No No
DPPTC DPPX Ab IFA Titer, CSF No No
GL1CC mGluR1 Ab CBA, CSF No No
GL1IC mGluR1 Ab IFA, CSF No No
GL1TC mGluR1 Ab IFA Titer, CSF No No

Method Name

ANN1C, ANN2C, ANN3C, AGN1C, PCA1C, PCA2C, PCTRC, AMPHC, CRMC, NMDIC, AMPIC, GABIC, DPPIC, DPPTC, GL1CC, GL1TC: Indirect Immunofluorescence (IFA)

NMDCC, AMPCC, GABCC, LG1CC, CS2CC, DPPCC, GL1CC: Cell-Binding Assay (CBA)

WBNC, ABLTC: Western Blot

VGKCC: Radioimmunoassay (RIA)

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

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.

Forms

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