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


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 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.


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
AMPCS AMPA-R Ab CBA, S No Yes
ANN1S Anti-Neuronal Nuclear Ab, Type 1 No Yes
CS2CS CASPR2-IgG CBA, 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
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
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
AMPIS AMPA-R Ab IF Titer Assay, S No No

Testing Algorithm

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

 

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

 

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

 

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

 

If alpha-amino-3-hydroxy-5 methyl-4-isoxazolepropionic acid (AMPA)-receptor antibody CBA is positive, then AMPA-receptor antibody IFA titer assay 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

Method Name

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

MOGFS, MOGTS, NMOFS, NMOTS: Flow Cytometry

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

GD65S: Radioimmunoassay (RIA)

AN1BS, AN2BS, PCTBS: Immunoblot (IB)

PCSI: Medical Interpretation

Reporting Name

Peds Autoimm Enceph CNS, S

Specimen Type

Serum

Specimen Minimum Volume

2 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

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

Interpretive report

AMPCS

AMPA-R Ab CBA, S

CBA

Negative

ANN1S

Anti-Neuronal Nuclear Ab, Type 1

IFA

Negative

CS2CS

CASPR2-IgG CBA, S

CBA

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

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

AMPIS

AMPA-R Ab IF Titer Assay, S

IFA

<1:240

AN1BS

ANNA-1 Immunoblot, S

IB

Negative

AN1TS

ANNA-1 Titer, S

IFA

<1:240

AN2BS

ANNA-2 Immunoblot, S

IB

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."

Interpretation

This profile is consistent with an autoimmune central nervous system disorder.

Day(s) Performed

Profile tests: Monday through Sunday; Reflex tests: Varies

Report Available

8 to 12 days

Specimen Retention Time

28 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 11

86256 AMPIS (if appropriate)

84182 AN1BS (if appropriate)

86256 AN1TS (if appropriate)

84182 AN2BS (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)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
PCDES Peds Autoimm Enceph CNS, S 101417-4

 

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
38324 NMO/AQP4 FACS, S 43638-6
64279 LGI1-IgG CBA, S 94287-0
64281 CASPR2-IgG CBA, S 94285-4
65563 MOG FACS, S 90248-6
64933 DPPX Ab CBA, S 94676-4
64928 mGluR1 Ab IFA, S 94347-2
605155 GFAP IFA, S 94346-4
605131 Peds Autoimmune CNS Interp, S 69048-7
80150 ANNA-1, S 33615-6
81596 GAD65 Ab Assay, S 30347-9
83076 PCA-Tr, S 84926-5
615867 Neurochondrin IFA, S 101452-1
618907 IFA Notes 48767-8