Test Code ENS2 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
Additional Codes
Lab LIS Code |
ENS2 |
Epice Code |
LAB4736 |
Mayo Code |
ENS2 |
Ordering Guidance
Multiple neuroimmunology profile tests are available. For testing that is performed with each profile, 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.
Necessary Information
Provide the following information:
-Relevant clinical information
-Ordering provider name, phone number, mailing address, and e-mail address
Specimen Required
Patient Preparation:
1. For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication or intravenous immunoglobulin treatment.
2. 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.
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.
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 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 |
DPPIS | DPPX Ab IFA, 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 |
IG5IS | IgLON5 IFA, S | No | Yes |
LG1CS | LGI1-IgG CBA, S | No | Yes |
GL1IS | mGluR1 Ab IFA, 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 |
SP7IS | Septin-7 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 |
DPPCS | DPPX Ab CBA, S | No | 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 |
IG5CS | IgLON5 CBA, 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 |
Testing Algorithm
If client requests or if the immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then 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 amphiphysin IFA titer and amphiphysin immunoblot will be performed at an additional charge.
If the IFA pattern suggests antiglial nuclear antibody (AGNA)-1, then 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 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 ANNA-2 IFA titer, ANNA-2 immunoblot, and ANNA-1 immunoblot will be performed at an additional charge.
If the IFA pattern suggests Purkinje cytoplasmic antibody type 1 (PCA-1), then PCA-1 IFA titer and PCA-1 immunoblot will be performed at an additional charge.
If IFA pattern suggests PCA-Tr antibody, then PCA-Tr IFA titer and PCA-Tr immunoblot will be performed at an additional charge.
If the IFA pattern suggests IgLON5 antibody, then IgLON5 IFA titer and IgLON5 cell-binding assay (CBA) will be performed at an additional charge.
If AMPA (alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid) receptor antibody CBA is positive, then AMPA-receptor antibody IFA titer assay will be performed at an additional charge.
If gamma-aminobutyric acid B (GABA-B) receptor antibody is positive, then 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 GFAP IFA titer and GFAP CBA will be performed at an additional charge.
If N-methyl-D-aspartate (NMDA) receptor antibody CBA is positive, then NMDA-receptor antibody IFA titer assay will be performed at an additional charge.
If the IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX IFA titer will be performed at an additional charge.
If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then 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 alpha internexin CBA, NIF heavy chain CBA, NIF light chain CBA, and NIF 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
Special Instructions
Method Name
AGN1S, AGNTS, AMPHS, APHTS, AMPIS, ANN1S, AN1TS, ANN2S, AN2TS, AN3TS, ANN3S, CRMS, CRMTS, DPPIS, DPPTS, GABIS, GFAIS, GFATS, IG5IS, IG5TS, GL1IS, GL1TS, NCDIS, NCDTS, NIFIS, NIFTS, NMDIS, PCABP, PC1TS, PCAB2, PC2TS, PCATR, PCTTS, SP7IS, SP7TS: Indirect Immunofluorescence Assay (IFA)
AMPCS, CS2CS, DPPCS, GABCS, GFACS, IG5CS, LG1CS, GL1CS, NCDCS, AINCS, NFLCS, NFHCS, NMDCS, SP7CS: Cell Binding Assay (CBA)
CRMWS: Western Blot (WB)
AGNBS, AMIBS, AN1BS, AN2BS, PC1BS, PCTBS: Immunoblot (IB)
GD65S: Radioimmunoassay (RIA)
Reporting Name
Encephalopathy, Autoimm/Paraneo, SSpecimen Type
SerumSpecimen 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 |
Reject Due To
Gross hemolysis | Reject |
Gross lipemia | Reject |
Gross icterus | Reject |
Reference Values
Test ID |
Reporting name |
Methodology* |
Reference value |
AEESI |
Encephalopathy, Interpretation, S |
Medical interpretation |
NA |
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 |
DPPIS |
DPPX Ab IFA, S |
IFA |
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 |
IG5IS |
IgLON5 IFA, S |
IFA |
Negative |
LG1CS |
LGI1-IgG CBA, S |
CBA |
Negative |
GL1IS |
mGluR1 Ab IFA, S |
IFA |
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 |
SP7IS |
Septin-7 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 |
DPPCS |
DPPX Ab CBA, S |
CBA |
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 |
IG5CS |
IgLON5 CBA, S |
CBA |
Negative |
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 |
SP7CS |
Septin-7 CBA, S |
CBA |
Negative |
SP7TS |
Septin-7 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 daysPerforming Laboratory

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 US Food and Drug Administration.CPT Code Information
86255 x 21
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)
86255-DPPCS (if appropriate)
86256-DPPTS (if appropriate)
86256-GABIS (if appropriate)
86255-GFACS (if appropriate)
86256-GFATS (if appropriate)
86255-IG5CS (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)
86255 SP7CS (if appropriate)
86256 SP7TS (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 |
34257 | Encephalopathy, Interpretation, S | 69048-7 |
89080 | AGNA-1, S | 94341-5 |
81722 | Amphiphysin Ab, S | 94340-7 |
80150 | ANNA-1, S | 94342-3 |
80776 | ANNA-2, S | 94343-1 |
83137 | ANNA-3, S | 94344-9 |
83077 | CRMP-5-IgG, S | 94815-8 |
81596 | GAD65 Ab Assay, S | 30347-9 |
83138 | PCA-2, S | 94351-4 |
9477 | PCA-1, S | 94350-6 |
83076 | PCA-Tr, S | 94352-2 |
64279 | LGI1-IgG CBA, S | 94287-0 |
64281 | CASPR2-IgG CBA, S | 94285-4 |
64930 | DPPX Ab IFA, S | 82976-2 |
64928 | mGluR1 Ab IFA, S | 94347-2 |
605155 | GFAP IFA, S | 94346-4 |
606946 | IgLON5 IFA, S | 96476-7 |
606964 | NIF IFA, S | 96486-6 |
615867 | Neurochondrin IFA, S | 101452-1 |
615875 | Septin-7 IFA, S | In Process |
618896 | IFA Notes | 48767-8 |