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Test Code LAB93544 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum

Additional Codes

ENS2


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 PCDES / Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Serum. 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.

 

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 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

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 serum 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 cerebral spinal 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
AEESI Encephalopathy, Interpretation, S No Yes
AMPCS AMPA-R Ab CBA, S No Yes
AMPHS Amphiphysin Ab, S No Yes
AGN1S Anti-Glial Nuclear Ab, Type 1 No Yes
ANN1S Anti-Neuronal Nuclear Ab, Type 1 No Yes
ANN2S Anti-Neuronal Nuclear Ab, Type 2 No Yes
ANN3S Anti-Neuronal Nuclear Ab, Type 3 No Yes
CS2CS CASPR2-IgG CBA, S No Yes
CRMS CRMP-5-IgG, S No Yes
DPPCS DPPX Ab CBA, S No Yes
GABCS GABA-B-R Ab CBA, S No Yes
GD65S GAD65 Ab Assay, S Yes Yes
GFAIS GFAP IFA, S No Yes
GL1IS mGluR1 Ab IFA, S No Yes
IG5CS IgLON5 CBA, S No Yes
LG1CS LGI1-IgG CBA, S No Yes
NCDIS Neurochondrin IFA, S No Yes
NIFIS NIF IFA, S No Yes
NMDCS NMDA-R Ab CBA, S No Yes
PCABP Purkinje Cell Cytoplasmic Ab Type 1 No Yes
PCAB2 Purkinje Cell Cytoplasmic Ab Type 2 No Yes
PCATR Purkinje Cell Cytoplasmic Ab Type Tr No Yes
PDEIS PDE10A Ab IFA, S No Yes
SP7IS Septin-7 IFA, S No Yes
T46IS TRIM46 Ab IFA, S 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
AMPIS AMPA-R Ab IF Titer Assay, S No No
AMIBS Amphiphysin Immunoblot, S No No
AN1BS ANNA-1 Immunoblot, S No No
AN2BS ANNA-2 Immunoblot, S No No
CRMWS CRMP-5-IgG Western Blot, S Yes No
DPPTS DPPX Ab IFA Titer, S No No
GABIS GABA-B-R Ab IF Titer Assay, S No No
GFACS GFAP CBA, S No No
GFATS GFAP IFA Titer, S No No
IG5TS IgLON5 IFA Titer, S No No
GL1CS mGluR1 Ab CBA, S No No
GL1TS mGluR1 Ab IFA Titer, S No No
NFHCS NIF Heavy Chain CBA, S No No
NIFTS NIF IFA Titer, S No No
NFLCS NIF Light Chain CBA, S No No
NMDIS NMDA-R Ab IF Titer Assay, S No No
PC1BS PCA-1 Immunoblot, S No No
PCTBS PCA-Tr Immunoblot, S No No
AGNTS AGNA-1 Titer, S No No
APHTS Amphiphysin Ab Titer, S No No
AN1TS ANNA-1 Titer, S No No
AN2TS ANNA-2 Titer, S No No
AN3TS ANNA-3 Titer, S No No
CRMTS CRMP-5-IgG Titer, S No No
NCDCS Neurochondrin CBA, S No No
NCDTS Neurochondrin IFA Titer, S No No
PC1TS PCA-1 Titer, S No No
PC2TS PCA-2 Titer, S No No
PCTTS PCA-Tr Titer, S No No
SP7CS Septin-7 CBA, S No No
SP7TS Septin-7 IFA Titer, S No No
PDETS PDE10A Ab IFA Titer, S No No
T46CS TRIM46 Ab CBA, S No No
T46TS TRIM46 Ab IFA Titer, S No No

Testing Algorithm

To determine the necessity of laboratory testing for patients with suspected autoimmune encephalitis, epilepsy or dementia, see the Antibody Prevalence in Epilepsy and Encephalopathy (APE2) scorecard.

 

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-2 immunoblot, and ANNA-1 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 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 the 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 is positive, then the 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 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-Serum

-Central Nervous System Demyelinating Disease Diagnostic Algorithm

Method Name

AEESI: Medical Interpretation

 

AGN1S, AGNTS, AMPHS, APHTS, AMPIS, ANN1S, AN1TS, ANN2S, AN2TS, AN3TS, ANN3S, CRMS, CRMTS, DPPTS, GABIS, GFAIS, GFATS, IG5TS, GL1IS, GL1TS, NCDIS, NCDTS, NIFIS, NIFTS, NMDIS, PCABP, PC1TS, PCAB2, PC2TS, PCATR, PCTTS, PDEIS, PDETS, SP7IS, SP7TS, T46IS, T46TS: Indirect Immunofluorescence Assay (IFA)

 

AMPCS, CS2CS, DPPCS, GABCS, GFACS, IG5CS, LG1CS, GL1CS, NCDCS, AINCS, NFLCS, NFHCS, NMDCS, SP7CS, T46CS: Cell Binding Assay (CBA)

 

CRMWS: Western Blot (WB)

 

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

 

GD65S: Radioimmunoassay (RIA)

Reporting Name

Encephalopathy, Autoimm/Paraneo, S

Specimen Type

Serum

Specimen Minimum Volume

2.5 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

AEESI

Encephalopathy, Interpretation, S

Medical interpretation

Interpretive report

AMPCS

AMPA-R Ab CBA, S

CBA

Negative

AMPHS

Amphiphysin Ab, S

IFA

Negative

AGN1S

Anti-Glial Nuclear Ab, Type 1

IFA

Negative

ANN1S

Anti-Neuronal Nuclear Ab, Type 1

IFA

Negative

ANN2S

Anti-Neuronal Nuclear Ab, Type 2

IFA

Negative

ANN3S

Anti-Neuronal Nuclear Ab, Type 3

IFA

Negative

CS2CS

CASPR2-IgG CBA, S

CBA

Negative

CRMS

CRMP-5-IgG, S

IFA

Negative

DPPCS

DPPX Ab CBA, S

CBA

Negative

GABCS

GABA-B-R Ab CBA, S

CBA

Negative

GD65S

GAD65 Ab Assay, S

RIA

≤0.02 nmol/L

Reference values apply to all ages.

GFAIS

GFAP IFA, S

IFA

Negative

GL1IS

mGluR1 Ab IFA, S

IFA

Negative

IG5CS

IgLON5 CBA, S

CBA

Negative

LG1CS

LGI1-IgG CBA, S

CBA

Negative

NCDIS

Neurochondrin IFA, S

IFA

Negative

NIFIS

NIF IFA, S

IFA

Negative

NMDCS

NMDA-R Ab CBA, S

CBA

Negative

PCABP

Purkinje Cell Cytoplasmic Ab Type 1

IFA

Negative

PCAB2

Purkinje Cell Cytoplasmic Ab Type 2

IFA

Negative

PCATR

Purkinje Cell Cytoplasmic Ab Type Tr

IFA

Negative

PDEIS

PDE10A Ab IFA, S

IFA

Negative

SP7IS

Septin-7 IFA, S

IFA

Negative

T46IS

TRIM46 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

AMPIS

AMPA-R Ab IF Titer Assay, S

IFA

<1:240

APHTS

Amphiphysin Ab Titer, S

IFA

<1:240

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

AN2TS

ANNA-2 Titer, S

IFA

<1:240

AN3TS

ANNA-3 Titer, S

IFA

<1:240

CRMTS

CRMP-5-IgG Titer, S

IFA

<1:240

CRMWS

CRMP-5-IgG Western Blot, S

WB

Negative

DPPTS

DPPX Ab IFA Titer, S

IFA

<1:240

GABIS

GABA-B-R Ab IF Titer Assay, 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

GL1CS

mGluR1 Ab CBA, S

CBA

Negative

GL1TS

mGluR1 Ab IFA Titer, S

IFA

<1:240

NCDCS

Neurochondrin CBA, S

CBA

Negative

NCDTS

Neurochondrin IFA Titer, S

IFA

<1:240

NFHCS

NIF Heavy Chain CBA, S

CBA

Negative

NIFTS

NIF IFA Titer, S

IFA

<1:240

NFLCS

NIF Light Chain CBA, S

CBA

Negative

NMDIS

NMDA-R Ab IF Titer Assay, 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

PCTBS

PCA-Tr Immunoblot, S

IB

Negative

PCTTS

PCA-Tr Titer, S

IFA

<1:240

PDETS

PDE10A Ab IFA Titer, S

IFA

<1:240

SP7CS

Septin-7 CBA, S

CBA

Negative

SP7TS

Septin-7 IFA Titer, S

IFA

<1:240

T46CS

TRIM46 CBA, S

CBA

Negative

T46TS

TRIM46 IFA Titer, S

IFA

<1:240

 

*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, 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 1:240 are detectable by recombinant CRMP-5 Western blot analysis. CRMP-5 Western blot analysis will be done on request on stored serum (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 x 23

86341 x 1

84182-AGNBS (if appropriate)

86256 AGNTS (if appropriate)

86255-AINCS (if appropriate)

86256-AMPIS (if appropriate)

86256 APHTS (if appropriate)

84182-AMIBS (if appropriate)

84182-AN1BS (if appropriate)

86256 AN1TS (if appropriate)

84182-AN2BS (if appropriate)

86256 AN2TS (if appropriate)

86256 AN3TS (if appropriate)

86256 CRMTS (if appropriate)

84182-CRMWS (if appropriate)

86256-DPPTS (if appropriate)

86256-GABIS (if appropriate)

86255-GFACS (if appropriate)

86256-GFATS (if appropriate)

86256-IG5TS (if appropriate)

86255-GL1CS (if appropriate)

86256-GL1TS (if appropriate)

86255 NCDCS (if appropriate)

86256 NCDTS (if appropriate)

86255-NFHCS (if appropriate)

86256-NIFTS (if appropriate)

86255-NFLCS (if appropriate)

86256-NMDIS (if appropriate)

84182-PC1BS (if appropriate)

86256 PC1TS (if appropriate)

86256 PC2TS (if appropriate)

84182-PCTBS (if appropriate)

86256 PCTTS (if appropriate)

86256 PDETCS (if appropriate)

86255 SP7CS (if appropriate)

86256 SP7TS (if appropriate)

86255 T46CS (if appropriate)

86256 T46TS (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
ENS2 Encephalopathy, Autoimm/Paraneo, S 94697-0

 

Result ID Test Result Name Result LOINC Value
61516 NMDA-R Ab CBA, S 93503-1
61518 AMPA-R Ab CBA, S 93489-3
61519 GABA-B-R Ab CBA, S 93428-1
606950 IgLON5 CBA, S 96478-3
615867 Neurochondrin IFA, S 101452-1
615875 Septin-7 IFA, S 101465-3
616445 TRIM46 Ab IFA, S 103843-9
620068 PDE10A Ab IFA, S 103842-1
34257 Encephalopathy, Interpretation, S 69048-7
64279 LGI1-IgG CBA, S 94287-0
64281 CASPR2-IgG CBA, S 94285-4
64933 DPPX Ab CBA, S 94676-4
64928 mGluR1 Ab IFA, S 94347-2
605155 GFAP IFA, S 94346-4
606964 NIF IFA, S 96486-6
89080 AGNA-1, S 84927-3
81722 Amphiphysin Ab, S 72327-0
80150 ANNA-1, S 33615-6
80776 ANNA-2, S 43187-4
83137 ANNA-3, S 43102-3
83077 CRMP-5-IgG, S 72504-4
81596 GAD65 Ab Assay, S 30347-9
83138 PCA-2, S 84925-7
9477 PCA-1, S 84924-0
83076 PCA-Tr, S 84926-5
618896 IFA Notes 48767-8