Test Code LAB93546 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid
Additional Codes
ENC2
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
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-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
Special Instructions
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, CSFSpecimen Type
CSFSpecimen 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 daysPerforming Laboratory
Mayo Clinic Laboratories in RochesterTest 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 |