Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Serum - Mayo Clinic Laboratories Extended Catalog

Ordering Guidance

Multiple neurological phenotype-specific autoimmune/paraneoplastic evaluations are available. For more information as well as phenotype-specific testing options, refer to Autoimmune Neurology Test Ordering Guide.

For a list of antibodies performed with each evaluation, see Autoimmune Neurology Antibody Matrix.

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 a Neurology Specialty Testing Client Test Request (T732) with the specimen.

Secondary ID

605129

Useful For

Evaluating children with autoimmune central nervous system disorders using serum specimens

Profile Information

Test ID	Reporting Name	Available Separately	Always Performed
PCSI	Peds Autoimmune CNS Interp, S	No	Yes
ANN1S	Anti-Neuronal Nuclear Ab, Type 1	No	Yes
CS2CS	CASPR2-IgG CBA, 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
LG1CS	LGI1-IgG CBA, S	No	Yes
GL1IS	mGluR1 Ab IFA, S	No	Yes
MOGFS	MOG FACS, S	Yes	Yes
NCDIS	Neurochondrin IFA, S	No	Yes
NMDCS	NMDA-R Ab CBA, S	No	Yes
NMOFS	NMO/AQP4 FACS, S	Yes	Yes
PCATR	Purkinje Cell Cytoplasmic Ab Type Tr	No	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AN1BS	ANNA-1 Immunoblot, S	No	No
AN2BS	ANNA-2 Immunoblot, S	No	No
DPPCS	DPPX Ab CBA, S	No	No
DPPTS	DPPX Ab IFA Titer, S	No	No
GFACS	GFAP CBA, S	No	No
GFATS	GFAP IFA Titer, S	No	No
GL1CS	mGluR1 Ab CBA, S	No	No
GL1TS	mGluR1 Ab IFA Titer, S	No	No
MOGTS	MOG FACS Titer, S	No	No
NMDIS	NMDA-R Ab IF Titer Assay, S	No	No
NMOTS	NMO/AQP4 FACS Titer, S	No	No
PCTBS	PCA-Tr Immunoblot, S	No	No
AN1TS	ANNA-1 Titer, S	No	No
GABIS	GABA-B-R Ab IF Titer Assay, S	No	No
NCDCS	Neurochondrin CBA, S	No	No
NCDTS	Neurochondrin IFA Titer, S	No	No
PCTTS	PCA-Tr Titer, S	No	No

Testing Algorithm

If the indirect immunofluorescence assay (IFA) pattern suggests antineuronal nuclear antibody type 1 (ANNA-1), then ANNA-1 immunoblot, ANNA-1 IFA titer and ANNA-2 immunoblot will be performed at an additional charge.

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

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

If gamma-aminobutyric acid B receptor (GABA-B-R) antibody CBA is positive, then GABA-B-R IFA titer 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 glial fibrillary acidic protein (GFAP) antibody, then GFAP antibody CBA and GFAP IFA titer will be performed at an additional charge.

If the neuromyelitis optica/aquaporin-4-IgG (NMO/AQP4-IgG) fluorescence-activated cell sorting (FACS) screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay will be performed at an additional charge.

If the myelin oligodendrocyte glycoprotein (MOG) FACS screen assay requires further investigation, then MOG FACS titration assay will be performed at an additional charge.

If IFA pattern suggests neurochondrin antibody, then neurochondrin antibody CBA and neurochondrin IFA titer will be performed at an additional charge.

For more information, see the following:

Pediatric Autoimmune Encephalopathy/Central Nervous System Disorders Evaluation Algorithm-Serum

Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm

Special Instructions

Method Name

CS2CS, DPPCS, GABCS, GFACS, LG1CS, GL1CS, NCDCS, NMDCS: Cell Binding Assay (CBA)

MOGFS, MOGTS, NMOFS, NMOTS: Flow Cytometry

ANN1S, AN1TS, DPPIS, DPPTS, GABIS, GFAIS, GFATS, GL1IS, GL1TS, NCDIS, NCDTS, NMDIS, PCATR, PCTTS: Indirect Immunofluorescence (IFA)

GD65S: Radioimmunoassay (RIA)

AN1BS, AN2BS, PCTBS: Immunoblot (IB)

Reporting Name

Peds Autoimm Enceph CNS, S

Specimen Type

Serum

Specimen Minimum Volume

2 mL

Specimen Stability Information

Specimen Type	Temperature	Time
Serum	Refrigerated (preferred)	28 days
	Frozen	28 days
	Ambient	72 hours

Clinical Information

Autoimmune encephalitis and myelitis is increasingly recognized as a cause of central nervous system disease in children and adolescents. N-methyl-D-aspartate receptor antibody (NMDA-R) encephalitis and myelin oligodendrocyte glycoprotein (MOG) autoimmunity are most common, though other entities, including aquaporin-4 autoimmunity, contactin-associated protein-like 2 (CASPR2) autoimmunity, autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, and paraneoplastic encephalomyelopathies, may also occur in children.

Reference Values

Test ID	Reporting name	Methodology*	Reference value
PCSI	Peds Autoimmune CNS Interp, S	Medical interpretation	NA
ANN1S	Anti-Neuronal Nuclear Ab, Type 1	IFA	Negative
CS2CS	CASPR2-IgG CBA, S	CBA	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
LG1CS	LGI1-IgG CBA, S	CBA	Negative
GL1IS	mGluR1 Ab IFA, S	IFA	Negative
NCDIS	Neurochondrin IFA, S	IFA	Negative
MOGFS	MOG FACS, S	FACS	Negative
NMDCS	NMDA-R Ab CBA, S	CBA	Negative
NMOFS	NMO/AQP4 FACS, S	FACS	Negative
PCATR	Purkinje Cell Cytoplasmic Ab Type Tr	IFA	Negative

Reflex Information:

Test ID	Reporting name	Methodology	Reference value
AN1BS	ANNA-1 Immunoblot, S	IB	Negative
AN1TS	ANNA-1 Titer, S	IFA	<1:240
AN2BS	ANNA-2 Immunoblot, S	IB	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
GL1CS	mGluR1 Ab CBA, S	CBA	Negative
GL1TS	mGluR1 Ab IFA Titer, S	IFA	<1:240
MOGTS	MOG FACS Titer, S	FACS	<1:20
NCDCS	Neurochondrin CBA, S	CBA	Negative
NCDTS	Neurochondrin IFA Titer, S	IFA	<1:240
NMDIS	NMDA-R Ab IF Titer Assay, S	IFA	<1:240
NMOTS	NMO/AQP4 FACS Titer, S	FACS	<1:5
PCTTS	PCA-Tr Titer, S	IFA	<1:240
PCTBS	PCA-Tr Immunoblot, S	IB	Negative

*Methodology abbreviations:

Immunofluorescence assay (IFA)

Cell-binding assay (CBA)

Fluorescence activated cell sorting assay (FACS)

Radioimmunoassay (RIA)

Immunoblot (IB)

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

Cautions

Negative results do not exclude a diagnosis of an autoimmune central nervous system disorder.

Intravenous immunoglobulin (IVIg) treatment prior to the serum collection may cause a false-positive result.

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

86341

86363

86053

86255 x 10

84182 AN1BS (if appropriate)

86256 AN1TS (if appropriate)

84182 AN2BS (if appropriate)

86255 DPPCS (if appropriate)

86256 DPPTS (if appropriate)

86256 GABIS (if appropriate)

86255 GFACS (if appropriate)

86256 GFATS (if appropriate)

86255 GL1CS (if appropriate)

86256 GL1TS (if appropriate)

86363 MOGTS (if appropriate)

86255 NCDCS (if appropriate)

86256 NCDTS (if appropriate)

86256 NMDIS (if appropriate)

86053 NMOTS (if appropriate)

84182 PCTBS (if appropriate)

86256 PCTTS (if appropriate)

NY State Approved

Yes

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Test ID PCDES Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Serum