Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum - Rochester Regional Health Laboratories

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, it will be canceled and automatically reordered by the laboratory as PCDES / Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Serum. The pediatric autoimmune central nervous system 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 for 1 week and assayed if sufficiently decayed or canceled if radioactivity remains.

Necessary Information

Provide the following information:

-Relevant clinical information

-Ordering healthcare professional's name, phone number, mailing address, and email address

Specimen Required

Patient Preparation: For optimal antibody detection, collect specimen before starting immunosuppressant medication or intravenous immunoglobulin (IVIg) 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 Serum

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)

-Microbiology Test Request (T244)

Secondary ID

92116

Useful For

Evaluating, using serum specimens, 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

The following accompaniments should increase 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
AK5IS	AK5 Ab IFA, 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
GBACS	GABA-A-R Ab CBA, S	Yes	Yes
GABCS	GABA-B-R Ab CBA, S	No	Yes
GD65S	GAD65 Ab Assay, S	Yes	Yes
IG5CS	IgLON5 CBA, S	No	Yes
KLHCS	KLHL11-IgG CBA, S	Yes	Yes
LG1CS	LGI1-IgG CBA, S	No	Yes
MA2ES	Ma2 Ab ELISA, S	Yes	Yes
GL1IS	mGluR1 Ab IFA, S	No	Yes
GL5IS	mGluR5 Ab IFA, S	No	Yes
NCDIS	Neurochondrin IFA, S	No	Yes
NMDCS	NMDA-R Ab CBA, S	No	Yes
PDEIS	PDE10A Ab IFA, 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
T46IS	TRIM46 Ab IFA, S	No	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AGNBS	AGNA-1 Immunoblot, S	No	No
AGNTS	AGNA-1 Titer, S	No	No
AK5CS	AK5 Ab CBA, S	No	No
AK5TS	AK5 Ab IFA Titer, S	No	No
AMPIS	AMPA-R Ab IF Titer Assay, S	No	No
APHTS	Amphiphysin Ab Titer, S	No	No
AMIBS	Amphiphysin Immunoblot, S	No	No
AN1BS	ANNA-1 Immunoblot, S	No	No
AN1TS	ANNA-1 Titer, S	No	No
AN2BS	ANNA-2 Immunoblot, 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
DPPTS	DPPX Ab IFA Titer, S	No	No
IG5TS	IgLON5 IFA Titer, S	No	No
K11TS	KLHL11 Ab IFA Titer, S	No	No
MP1BS	MAP1B Immunoblot, S	No	No
GL1CS	mGluR1 Ab CBA, S	No	No
GL1TS	mGluR1 Ab IFA Titer, S	No	No
GL5CS	mGluR5 Ab CBA, S	No	No
GL5TS	mGluR5 Ab IFA Titer, S	No	No
NCDCS	Neurochondrin CBA, S	No	No
NCDTS	Neurochondrin IFA Titer, S	No	No
NMDIS	NMDA-R Ab IF Titer Assay, S	No	No
PC1BS	PCA-1 Immunoblot, S	No	No
PC1TS	PCA-1 Titer, S	No	No
PC2TS	PCA-2 Titer, S	No	No
PCTBS	PCA-Tr Immunoblot, S	No	No
PCTTS	PCA-Tr Titer, S	No	No
PDETS	PDE10A Ab IFA Titer, S	No	No
SP7CS	Septin-7 CBA, S	No	No
SP7TS	Septin-7 IFA Titer, S	No	No
T46CS	TRIM46 Ab CBA, S	No	No
T46TS	TRIM46 Ab IFA Titer, S	No	No
CRMWS	CRMP-5-IgG Western Blot, S	No	No
GABIS	GABA-B-R Ab IF Titer Assay, 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 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 pattern suggests adenylate kinase 5 protein (AK5) antibody, then AK5 IFA titer and AK5 cell-binding assay (CBA) 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 cell 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 and microtubule-associated protein 1 (MAP1B) 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 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 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 mGluR5 antibody, then the mGluR5 antibody CBA and mGluR5 IFA titer will be performed at an additional charge.

If the Kelch-like protein 11 (KLHL11) CBA result is reactive, then the KLHL11 antibody 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

Special Instructions

Method Name

AEESI: Technical Interpretation

AGN1S, AK5IS, AMPHS, ANN1S, ANN2S, ANN3S, CRMS, GABIS, GL1IS, GL5IS, NCDIS, PCAB2, PCABP, PCATR, PDEIS, SP7IS, T46IS, AGNTS, AK5TS, AMPIS, AN1TS, AN2TS, AN3TS, APHTS, CRMTS, DPPTS, GL1TS, GL5TS, IG5TS, K11TS, NCDTS, NMDIS, PC1TS, PC2TS, PCTTS, PDETS, SP7TS, T46TS: Indirect Immunofluorescence Assay (IFA)

AMPCS, CS2CS, DPPCS, GABCS, GBACS, IG5CS, LG1CS, NMDCS, KLHCS, AK5CS, GL1CS, GL5CS, NCDCS, SP7CS, T46CS: Cell Binding Assay (CBA)

MA2ES: Enzyme-Linked Immunosorbent Assay (ELISA)

GD65S: Radioimmunoassay (RIA)

CRMWS: Western Blot (WB)

AGNBS, AMIBS, AN1BS, AN2BS, MP1BS, PC1BS, PCTBS: Immunoblot (IB)

Reporting Name

Encephalopathy, Autoimm/Paraneo, S

Specimen Type

Serum

Specimen Minimum Volume

Serum: 2.5 mL

Specimen Stability Information

Specimen Type	Temperature	Time
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	Technical interpretation	Interpretive report
AK5IS	AK5 Ab IFA, S	IFA	Negative
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
GBACS	GABA-A-R Ab CBA, S	CBA	Negative
GABCS	GABA-B-R Ab CBA, S	CBA	Negative
GD65S	GAD65 Ab Assay, S	RIA	≤0.02 nmol/L
IG5CS	IgLON5 CBA, S	CBA	Negative
KLHCS	KLHL11-IgG CBA, S	CBA	Negative
LG1CS	LGI1-IgG CBA, S	CBA	Negative
MA2ES	Ma2 Ab ELISA, S	ELISA	Negative
GL1IS	mGluR1 Ab IFA, S	IFA	Negative
GL5IS	mGluR5 Ab IFA, S	IFA	Negative
NCDIS	Neurochondrin IFA, S	IFA	Negative
NMDCS	NMDA-R Ab CBA, S	CBA	Negative
PDEIS	PDE10A Ab IFA, S	IFA	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
T46IS	TRIM46 Ab 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
AK5CS	AK5 Ab CBA, S	CBA	Negative
AK5TS	AK5 Ab IFA Titer, S	IFA	<1:240
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
IG5TS	IgLON5 IFA Titer, S	IFA	<1:240
K11TS	KLHL11 Ab IFA Titer, S	IFA	<1:240
MP1BS	MAP1B Immunoblot, S	IB	Negative
GL1CS	mGluR1 Ab CBA, S	CBA	Negative
GL1TS	mGluR1 Ab IFA Titer, S	IFA	<1:240
GL5CS	mGluR5 Ab CBA, S	CBA	Negative
GL5TS	mGluR5 Ab IFA Titer, S	IFA	<1:240
NCDCS	Neurochondrin CBA, S	CBA	Negative
NCDTS	Neurochondrin IFA Titer, S	IFA	<1:240
NMDIS	NMDA-R Ab IF Titer Assay, S	IFA	<1:120
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 Ab CBA, S	CBA	Negative
T46TS	TRIM46 Ab IFA Titer, S	IFA	<1:240

*Methodology abbreviations:

Cell-binding assay (CBA)

Enzyme-linked immunosorbent assay (ELISA)

Immunoblot (IB)

Immunofluorescence assay (IFA)

Radioimmunoassay (RIA)

Western blot (WB)

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 24

86341

0432U

83516

84182 AGNBS (if appropriate)

86256 AGNTS (if appropriate)

86255 AK5CS (if appropriate)

86256 AK5TS (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)

86256 IG5TS (if appropriate)

86256 K11TS (if appropriate)

84182 MP1BS (if appropriate)

86255 GL1CS (if appropriate)

86256 GL1TS (if appropriate)

86255 GL5CS (if appropriate)

86256 GL5TS (if appropriate)

86255 NCDCS (if appropriate)

86256 NCDTS (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 PDETS (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
620232	GABA-A-R Ab CBA, S	101874-6
623395	mGluR5 Ab IFA, S	In Process
623399	AK5 Ab IFA, S	In Process
616445	TRIM46 Ab IFA, S	105525-0
620068	PDE10A Ab IFA, S	103842-1
622117	KLHL11-IgG CBA, S	99072-1
615867	Neurochondrin IFA, S	101452-1
615875	Septin-7 IFA, S	101465-3
605970	Ma2 Ab ELISA, S	101868-8
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
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	94345-6
83138	PCA-2, S	84925-7
9477	PCA-1, S	84924-0
83076	PCA-Tr, S	84926-5
618896	IFA Notes	48767-8

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Test Code ENS2 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum