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Test Code ENC2 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid


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 is intended to be ordered for adult patients. If this test is ordered for a patient younger than 18 years of age, it will be canceled and automatically reordered by the laboratory as PCDEC / Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid. The pediatric autoimmune CNS disorders evaluation is part of an evolving approach to testing for autoimmune neurological disorders using phenotypic-specific evaluations that include multiple antibodies known for their disease association.



Necessary Information


Provide the following information:

-Relevant clinical information

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



Specimen Required


Container/Tube: Sterile vial

Preferred: Collection vial number 1

Acceptable: Any collection vial

Specimen Volume: 4 mL


Forms

If not ordering electronically, complete, print, and send 1 of the following with the specimen:

-Neurology Specialty Testing Client Test Request (T732)

-General Test Request (T239)

Useful For

Evaluating new onset encephalopathy (noninfectious or metabolic) comprising confusional states, psychosis, delirium, memory loss, hallucinations, movement disorders, sensory or motor complaints, seizures, dyssomnias, ataxias, nausea, vomiting, inappropriate antidiuresis, coma, dysautonomias, or hypoventilation using spinal fluid specimens

 

The following accompaniments should increase of suspicion for autoimmune encephalopathy:

-Headache

-Autoimmune stigmata (personal or family history or signs of diabetes mellitus, thyroid disorder, vitiligo, poliosis [premature graying], myasthenia gravis, rheumatoid arthritis, systemic lupus erythematosus)

-History of cancer

-Smoking history (20 or more pack-years) or other cancer risk factors

-Inflammatory cerebrospinal fluid (or isolated protein elevation)

-Neuroimaging signs suggesting inflammation

 

Evaluating limbic encephalitis (noninfectious)

 

Directing a focused search for cancer

 

Investigating encephalopathy appearing during or after cancer therapy and not explainable by metastasis or drug effect

Profile Information

Test ID Reporting Name Available Separately Always Performed
AEECI Encephalopathy, Interpretation, CSF No Yes
AMPCC AMPA-R Ab CBA, CSF No Yes
AMPHC Amphiphysin Ab, CSF No Yes
AGN1C Anti-Glial Nuclear Ab, Type 1 No Yes
ANN1C Anti-Neuronal Nuclear Ab, Type 1 No Yes
ANN2C Anti-Neuronal Nuclear Ab, Type 2 No Yes
ANN3C Anti-Neuronal Nuclear Ab, Type 3 No Yes
CS2CC CASPR2-IgG CBA, CSF No Yes
CRMC CRMP-5-IgG, CSF No Yes
DPPCC DPPX Ab CBA, CSF No Yes
GABCC GABA-B-R Ab CBA, CSF No Yes
GD65C GAD65 Ab Assay, CSF Yes Yes
GFAIC GFAP IFA, CSF No Yes
GL1IC mGluR1 Ab IFA, CSF No Yes
IG5CC IgLON5 CBA, CSF No Yes
LG1CC LGI1-IgG CBA, CSF No Yes
NCDIC Neurochondrin IFA, CSF No Yes
NIFIC NIF IFA, CSF No Yes
NMDCC NMDA-R Ab CBA, CSF No Yes
PCTRC Purkinje Cell Cytoplasmc Ab Type Tr No Yes
PCA1C Purkinje Cell Cytoplasmic Ab Type 1 No Yes
PCA2C Purkinje Cell Cytoplasmic Ab Type 2 No Yes
PDEIC PDE10A Ab IFA, CSF No Yes
SP7IC Septin-7 IFA, CSF No Yes
T46IC TRIM46 Ab IFA, CSF No Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AGNBC AGNA-1 Immunoblot, CSF No No
AINCC Alpha Internexin CBA, CSF No No
AMPIC AMPA-R Ab IF Titer Assay, CSF No No
AMIBC Amphiphysin Immunoblot, CSF No No
AN1BC ANNA-1 Immunoblot, CSF No No
AN2BC ANNA-2 Immunoblot, CSF No No
CRMWC CRMP-5-IgG Western Blot, CSF Yes No
DPPTC DPPX Ab IFA Titer, CSF No No
GABIC GABA-B-R Ab IF Titer Assay, CSF No No
GFACC GFAP CBA, CSF No No
GFATC GFAP IFA Titer, CSF No No
IG5TC IgLON5 IFA Titer, CSF No No
GL1CC mGluR1 Ab CBA, CSF No No
GL1TC mGluR1 Ab IFA Titer, CSF No No
NFHCC NIF Heavy Chain CBA, CSF No No
NIFTC NIF IFA Titer, CSF No No
NFLCC NIF Light Chain CBA, CSF No No
NMDIC NMDA-R Ab IF Titer Assay, CSF No No
PC1BC PCA-1 Immunoblot, CSF No No
PCTBC PCA-Tr Immunoblot, CSF No No
AGNTC AGNA-1 Titer, CSF No No
AN1TC ANNA-1 Titer, CSF No No
AN2TC ANNA-2 Titer, CSF No No
AN3TC ANNA-3 Titer, CSF No No
APHTC Amphiphysin Ab Titer, CSF No No
CRMTC CRMP-5-IgG Titer, CSF No No
NCDCC Neurochondrin CBA, CSF No No
NCDTC Neurochondrin IFA Titer, CSF No No
PC1TC PCA-1 Titer, CSF No No
PC2TC PCA-2 Titer, CSF No No
PCTTC PCA-Tr Titer, CSF No No
PDETC PDE10A Ab IFA Titer, CSF No No
SP7CC Septin-7 CBA, CSF No No
SP7TC Septin-7 IFA Titer, CSF No No
T46CC TRIM46 Ab CBA, CSF No No
T46TC TRIM46 Ab IFA Titer, CSF No No

Testing Algorithm

If client requests or if the immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then the CRMP-5-IgG IFA titer and CRMP-5-IgG Western blot will be performed at an additional charge.

 

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

 

If the IFA pattern suggests antiglial nuclear antibody (AGNA-1), then the AGNA-1 IFA titer and AGNA-1 immunoblot will be performed at an additional charge.

 

If the IFA pattern suggests 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 IFA pattern suggests ANNA-2 antibody, then the ANNA-2 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 antibodies, then the ANNA-3 titer will be performed at an additional charge.

 

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

 

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

 

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

 

If the IgLON5 antibody cell binding assay (CBA) result is positive, then the IgLON5 IFA titer will be performed at an additional charge.

 

If the AMPA (alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid) receptor antibody CBA result is positive, then AMPA-receptor antibody IFA titer assay will be performed at an additional charge.

 

If the gamma-aminobutyric acid B (GABA-B) receptor antibody CBA result is positive, then the GABA-B-receptor antibody IFA titer assay will be performed at an additional charge.

 

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

 

If the N-methyl-D-aspartate (NMDA) receptor antibody CBA result is positive, then NMDA-receptor antibody IFA titer assay will be performed at an additional charge.

 

If the dipeptidyl-peptidase-like protein-6 (DPPX) antibody CBA result is positive, then the DPPX IFA titer will be performed at an additional charge.

 

If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then the mGluR1 antibody CBA and mGluR1 IFA titer 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 IFA pattern suggests neurochondrin antibody, then the neurochondrin antibody CBA and neurochondrin IFA titer will be performed at an additional charge.

 

If the IFA pattern suggests septin-7 antibody, then the septin-7 antibody CBA and septin-7 IFA titer will be performed at an additional charge.

 

If the IFA pattern suggests tripartite motif-containing protein 46 (TRIM46) antibody, then the TRIM46 antibody CBA and TRIM46 IFA titer will be performed at an additional charge.

 

If the IFA pattern suggests phosphodiesterase 10A (PDE10A) antibody, then the PDE10A antibody IFA titer will be performed at an additional charge.

 

For more information, see the following algorithms:

-Autoimmune/Paraneoplastic Encephalopathy Evaluation Algorithm-Spinal Fluid

-Central Nervous System Demyelinating Disease Diagnostic Algorithm

Method Name

AEECI: Medical Interpretation

 

AGN1C, AGNTC, AMPIC, AMPHC, APHTC, ANN1C, AN1TC, ANN2C, AN2TC, ANN3C, AN3TC, CRMTC, CRMC, DPPTC, GABIC, GFAIC, GFATC, IG5TC, GL1IC, GL1TC, NCDIC, NCDTC, NIFIC, NIFTC, NMDIC, PCA1C, PC1TC, PCA2C, PC2TC, PCTRC, PCTTC, PDEIC, PDETC, SP7IC, SP7TC, T46IC, T26TC: Indirect Immunofluorescence Assay (IFA)

 

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

 

CRMWC: Western Blot (WB)

 

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

 

GD65C: Radioimmunoassay (RIA)

Reporting Name

Enceph, Autoimm/Paraneo, CSF

Specimen Type

CSF

Specimen Minimum Volume

2 mL

Specimen Stability Information

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

Reference Values

Test ID

Reporting name

Methodology*

Reference value

AEECI

Encephalopathy, Interpretation, CSF

Medical interpretation

Interpretive report

AMPCC

AMPA-R Ab CBA, CSF

CBA

Negative

AMPHC

Amphiphysin Ab, CSF

IFA

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

CS2CC

CASPR2-IgG CBA, CSF

CBA

Negative

CRMC

CRMP-5-IgG, CSF

IFA

Negative

DPPCC

DPPX Ab CBA, CSF

CBA

Negative

GABCC

GABA-B-R Ab CBA, CSF

CBA

Negative

GD65C

GAD65 Ab Assay, CSF

RIA

≤0.02 nmol/L

Reference values apply to all ages.

GFAIC

GFAP IFA, CSF

IFA

Negative

GL1IC

mGluR1 Ab IFA, CSF

IFA

Negative

IG5CC

IgLON5 CBA, CSF

CBA

Negative

LG1CC

LGI1-IgG CBA, CSF

CBA

Negative

NCDIC

Neurochondrin IFA, CSF

IFA

Negative

NIFIC

NIF IFA, CSF

IFA

Negative

NMDCC

NMDA-R Ab CBA, CSF

CBA

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

PDEIC

PDE10A Ab IFA, CSF

IFA

Negative

SP7IC

Septin-7 IFA, CSF

IFA

Negative

T46IC

TRIM46 IFA, CSF

IFA

Negative

 

Reflex Information:

Test ID

Reporting name

Methodology*

Reference value

AGNBC

AGNA-1 Immunoblot, CSF

IB

Negative

AGNTC

AGNA-1 Titer, CSF

IFA

<1:2

AINCC

Alpha Internexin CBA, CSF

CBA

Negative

AMPIC

AMPA-R Ab IF Titer Assay, CSF

IFA

<1:2

AMIBC

Amphiphysin Immunoblot, CSF

IB

Negative

AN1BC

ANNA-1 Immunoblot, CSF

IB

Negative

AN1TC

ANNA-1 Titer, CSF

IFA

<1:2

AN2BC

ANNA-2 Immunoblot, CSF

IB

Negative

AN2TC

ANNA-2 Titer, CSF

IFA

<1:2

AN3TC

ANNA-3 Titer, CSF

IFA

<1:2

APHTC

Amphiphysin Ab Titer, CSF

IFA

<1:2

CRMTC

CRMP-5-IgG Titer, CSF

IFA

<1:2

CRMWC

CRMP-5-IgG Western Blot, CSF

WB

Negative

DPPTC

DPPX Ab IFA Titer, CSF

IFA

<1:2

GABIC

GABA-B-R Ab IF Titer Assay, CSF

IFA

<1:2

GFACC

GFAP CBA, CSF

CBA

Negative

GFATC

GFAP IFA Titer, CSF

IFA

<1:2

IG5TC

IgLON5 IFA Titer, CSF

IFA

<1:2

GL1CC

mGluR1 Ab CBA, CSF

CBA

Negative

GL1TC

mGluR1 Ab IFA Titer, CSF

IFA

<1:2

NCDCC

Neurochondrin CBA, CSF

CBA

Negative

NCDTC

Neurochondrin IFA Titer, CSF

IFA

<1:2

NFLCC

NIF Light Chain CBA, CSF

CBA

Negative

NFHCC

NIF Heavy Chain CBA, CSF

CBA

Negative

NIFTC

NIF IFA Titer, CSF

IFA

<1:2

NMDIC

NMDA-R Ab IF Titer Assay, CSF

IFA

<1:2

PC1BC

PCA-1 Immunoblot, CSF

IB

Negative

PC1TC

PCA-1 Titer, CSF

IFA

<1:2

PC2TC

PCA-2 Titer, CSF

IFA

<1:2

PCTBC

PCA-Tr Immunoblot, CSF

IB

Negative

PCTTC

PCA-Tr Titer, CSF

IFA

<1:2

PDETC

PDE10A Ab IFA Titer, CSF

IFA

<1:2

SP7CC

Septin-7 CBA, CSF

CBA

Negative

SP7IC

Septin-7 IFA Titer, CSF

IFA

<1:2

T46CC

TRIM46 CBA, CSF

CBA

Negative

T46TC

TRIM46 IFA Titer, CSF

IFA

<1:2

 

*Methodology abbreviations:

Immunofluorescence assay (IFA)

Cell-binding assay (CBA)

Western blot (WB)

Radioimmunoassay (RIA)

Immunoblot (IB)

 

Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, CRMP-5-IgG, PCA-1, PCA-2, or PCA-Tr may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

 

Note: CRMP-5 titers lower than 1:2 are detectable by recombinant CRMP-5 Western blot analysis. CRMP-5 Western blot analysis will be done on request on stored spinal fluid (held 4 weeks). This supplemental testing is recommended in cases of chorea, vision loss, cranial neuropathy, and myelopathy. Call the Neuroimmunology Laboratory at 800-533-1710 to request CRMP-5 Western blot.

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 x23

86341 x1

 

84182 AGNBC (if appropriate)

86256 AGNTC (if appropriate)

86255 AINCC (if appropriate)

86256 AMPIC (if appropriate)

84182 AMIBC (if appropriate)

84182 AN1BC (if appropriate)

86256 AN1TC (if appropriate)

84182 AN2BC (if appropriate)

86256 AN2TC (if appropriate)

86256 AN3TC (if appropriate)

86256 APHTC (if appropriate)

86256 CRMTC (if appropriate)

84182 CRMWC (if appropriate)

86256 DPPTC (if appropriate)

86256 GABIC (if appropriate)

86255 GFACC (if appropriate)

86256 GFATC (if appropriate)

86256 IG5TC (if appropriate)

86255 GL1CC (if appropriate)

86256 GL1TC (if appropriate)

86255 NCDCC (if appropriate)

86256 NCDTC (if appropriate)

86255 NFHCC (if appropriate)

86256 NIFTC (if appropriate)

86255 NFLCC (if appropriate)

86256 NMDIC (if appropriate)

84182 PC1BC (if appropriate)

86256 PC1TC (if appropriate)

86256 PC2TC (if appropriate)

84182 PCTBC (if appropriate)

86256 PCTTC (if appropriate)

86256 PDETC (if appropriate)

86255 SP7CC (if appropriate)

86256 SP7TC (if appropriate)

86255 T46CC (if appropriate)

86256 T46TC (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
ENC2 Enceph, Autoimm/Paraneo, CSF 94708-5

 

Result ID Test Result Name Result LOINC Value
61513 NMDA-R Ab CBA, CSF 93502-3
61514 AMPA-R Ab CBA, CSF 93491-9
61515 GABA-B-R Ab CBA, CSF 93426-5
34256 Encephalopathy, Interpretation, CSF 69048-7
89079 AGNA-1, CSF 90827-7
5906 Amphiphysin Ab, CSF 90815-2
3852 ANNA-1, CSF 44768-0
7472 ANNA-2, CSF 56959-0
21633 ANNA-3, CSF 90836-8
21650 CRMP-5-IgG, CSF 63216-6
3988 PCA-1, CSF 90841-8
21632 PCA-2, CSF 90843-4
21631 PCA-Tr, CSF 90845-9
21702 GAD65 Ab Assay, CSF 94359-7
64280 LGI1-IgG CBA, CSF 94288-8
64282 CASPR2-IgG CBA, CSF 94286-2
64927 mGluR1 Ab IFA, CSF 94361-3
64934 DPPX Ab CBA, CSF 94283-9
605156 GFAP IFA, CSF 94360-5
606965 NIF IFA, CSF 96490-8
606951 IgLON5 CBA, CSF 96481-7
615866 Neurochondrin IFA, CSF 101451-3
615874 Septin-7 IFA, CSF 101464-6
620067 PDE10A Ab IFA, CSF 103842-1
616446 TRIM46 Ab IFA, CSF 103843-9
618895 IFA Notes 48767-8