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HelpTest Code PCDEC Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid
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.
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.
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
Useful For
Evaluating children with autoimmune central nervous system disorders using cerebrospinal fluid specimens
Profile Information
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| PCCI | Peds Autoimmune CNS Interp, CSF | No | Yes |
| AMPCC | AMPA-R Ab CBA, CSF | No | Yes |
| ANN1C | Anti-Neuronal Nuclear Ab, Type 1 | No | Yes |
| CS2CC | CASPR2-IgG CBA, 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 |
| LG1CC | LGI1-IgG CBA, CSF | No | Yes |
| GL1IC | mGluR1 Ab IFA, CSF | No | Yes |
| NCDIC | Neurochondrin IFA, CSF | No | Yes |
| NMDCC | NMDA-R Ab CBA, CSF | No | Yes |
| NMOFC | NMO/AQP4 FACS, CSF | Yes | Yes |
| PCTRC | Purkinje Cell Cytoplasmc Ab Type Tr | No | Yes |
Reflex Tests
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| AMPIC | AMPA-R Ab IF Titer Assay, CSF | No | No |
| AN1BC | ANNA-1 Immunoblot, CSF | No | No |
| AN2BC | ANNA-2 Immunoblot, CSF | No | 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 |
| GL1CC | mGluR1 Ab CBA, CSF | No | No |
| GL1TC | mGluR1 Ab IFA Titer, CSF | No | No |
| NMDIC | NMDA-R Ab IF Titer Assay, CSF | No | No |
| NMOTC | NMO/AQP4 FACS Titer, CSF | No | No |
| PCTBC | PCA-Tr Immunoblot, CSF | No | No |
| AN1TC | ANNA-1 Titer, CSF | No | No |
| NCDCC | Neurochondrin CBA, CSF | No | No |
| NCDTC | Neurochondrin IFA Titer, CSF | No | No |
| PCTTC | PCA-Tr Titer, CSF | No | No |
Testing Algorithm
If the indirect immunofluorescence assay (IFA) pattern suggests antineuronal nuclear antibodies (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 cell 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 bind 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 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 cell-binding assay (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 Central Nervous System Demyelinating Disease Diagnostic Algorithm
Method Name
AMPCC, CS2CC, DPPCC, GABCC, GFACC, LG1CC, GL1CC, NCDCC, NMDCC: Cell Binding Assay (CBA)
NMOFC, NMOTC: Flow Cytometry
AMPIC, ANN1C, AN1TC, DPPTC, GABIC, GFAIC, GFATC, GL1IC, GL1TC, NCDIC, NCDTC, NMDIC, PCTRC, PCTTC: Indirect Immunofluorescence Assay (IFA)
GD65C: Radioimmunoassay (RIA)
AN1BC, AN2BC, PCTBC: Immunoblot (IB)
PCCI: Medical Interpretation
Reporting Name
Peds Autoimm Enceph CNS, 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 |
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, although 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 |
|
PCCI |
Peds Autoimmune CNS Interp, CSF |
Medical interpretation |
Interpretive report |
|
AMPCC |
AMPA-R Ab CBA, CSF |
CBA |
Negative |
|
ANN1C |
Anti-Neuronal Nuclear Ab, Type 1 |
IFA |
Negative |
|
CS2CC |
CASPR2-IgG CBA, CSF |
CBA |
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 |
|
LG1CC |
LGI1-IgG CBA, CSF |
CBA |
Negative |
|
NCDIC |
Neurochondrin IFA, CSF |
IFA |
Negative |
|
NMDCC |
NMDA-R Ab CBA, CSF |
CBA |
Negative |
|
NMOFC |
NMO/AQP4 FACS, CSF |
FACS |
Negative |
|
PCTRC |
Purkinje Cell Cytoplasmc Ab Type Tr |
IFA |
Negative |
Reflex Information:
|
Test ID |
Reporting name |
Methodology |
Reference value |
|
AMPIC |
AMPA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
|
AN1BC |
ANNA-1 Immunoblot, CSF |
IB |
Negative |
|
AN1TC |
ANNA-1 Titer, CSF |
IFA |
<1:2 |
|
AN2BC |
ANNA-2 Immunoblot, CSF |
IB |
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 |
|
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 |
|
NMDIC |
NMDA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
|
NMOTC |
NMO/AQP4 FACS Titer, CSF |
FACS |
<1:2 |
|
PCTTC |
PCA-Tr Titer, CSF |
IFA |
<1:2 |
|
PCTBC |
PCA-Tr Immunoblot, CSF |
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 or PCA-Tr may be reported as "unclassified antineuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."
Interpretation
This profile is consistent with an autoimmune central nervous system disorder.
Cautions
Negative results do not exclude a diagnosis of an autoimmune central nervous system disorder.
Method Description
Cell-Binding Assay:
Patient specimen is applied to a composite slide containing transfected and nontransfected HEK-293 cells. After incubation and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the presence of patient IgG binding.(Package insert: IIFT: Neurology Mosaics, Instructions for the indirect immunofluorescence test. EUROIMMUN; FA_112d-1_A_UK_C13, 02/2019)
Fluorescence-Activated Cell Sorting Assay:
Human embryonic kidney cells (HEK 293) are transfected transiently with a plasmid (pIRES2-Aequorea coerulescens green fluorescent protein [AcGFP]) encoding both green fluorescent protein (AcGFP) and AQP4-M1. After 36 hours, a mixed population of cells (transfected expressing AQP4 or MOG on the surface and AcGFP in the cytoplasm and nontransfected lacking AQP4 or MOG and AcGFP) are lifted and resuspended in live cell-binding buffer. Cells are incubated with patient serum and an AlexaFluor 647-labeled secondary antibody is added. Two populations are gated on the basis of AcGFP expression: positive (high AQP4 or MOG expression) and negative (low or no AQP4 or MOG expression). Positivity is based on the ratio (Positive >2.0) of the average median fluorescence intensity (MFI) of each cell population (MFI GFP positive:MFI GFP negative).(Unpublished Mayo method)
Indirect Immunofluorescence Assay:
The patient's sample is tested by a standardized immunofluorescence assay that uses a composite frozen section of mouse cerebellum, kidney, and gut tissues. After incubation with sample and washing, fluorescein-conjugated goat-antihuman IgG is applied. Neuron-specific autoantibodies are identified by their characteristic fluorescence staining patterns. Samples that are scored positive for any neuronal nuclear or cytoplasmic autoantibody are titrated to an endpoint. Interference by coexisting non-neuron-specific autoantibodies can usually be eliminated by serologic absorption.(Honorat JA, Komorowski L, Josephs KA, et al. IgLON5 antibody: neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017;4(5):e385. doi:10.1212/NXI.0000000000000385)
Radioimmunoassay:
(125)I-labeled recombinant human antigens or labeled receptors are incubated with patient specimen. After incubation, anti-human IgG is added to form an immunoprecipitate. The amount of (125)I-labeled antigen in the immunoprecipitate is measured using a gamma-counter. The amount of gamma emission in the precipitate is proportional to the amount of antigen-specific IgG in the specimen. Results are reported as units of precipitated antigen (nmol) per liter of patient sample.(Griesmann GE, Kryzer TJ, Lennon VA. Autoantibody profiles of myasthenia gravis and Lambert-Eaton myasthenic syndrome. In: Rose NR, Hamilton RG, et al, eds. Manual of Clinical and Laboratory Immunology. 6th ed ASM Press; 2002:1005-1012; Walikonis JE, Lennon VA. Radioimmunoassay for glutamic acid decarboxylase [GAD65] autoantibodies as a diagnostic aid for stiff-man syndrome and a correlate of susceptibility to type 1 diabetes mellitus. Mayo Clin Proc 19983[12]:1161-1166; Jones AL, Flanagan EP, Pittock SJ, et al. Responses to and Outcomes of Treatment of Autoimmune Cerebellar Ataxia in Adults. JAMA Neurol 2015;72[11]:1304-1312. doi:10.1001/jamaneurol.2015.2378)
Immunoblot:
All steps are performed at ambient temperature (18-28° C) utilizing the EUROBlot One instrument.
Diluted patient serum (1:101) is added to test strips (strips containing recombinant antigen manufactured and purified using biochemical methods) in individual channels and incubated for 30 minutes. Positive serums will bind to the purified recombinant antigen and negative serums will not bind. Strips are washed to remove unbound antibodies and then incubated with antihuman IgG antibodies (alkaline phosphatase-labeled) and incubated for 30 minutes. The strips are again washed to remove unbound antihuman IgG antibodies and nitroblue tetrazolium chloride/5-bromo-4-chloro-3-indolyl phosphate (NBT/BCIP) substrate is added. Alkaline phosphatase enzyme converts the soluble substrate into a colored insoluble product on the membrane to produce a black band. Strips are digitized via picture capture on the EUROBlot One instrument and evaluated with the EUROLineScan software. (O'Connor K, Waters P, Komorowski L, et al: GABAA receptor autoimmunity: A multicenter experience. Neurol Neuroimmunol Neuroinflamm. 2019;6[3]:e552 doi:10.1212/NXI.0000000000000552)
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
8 to 12 daysPerforming 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 11
86341
86053
86256 AMPIC (if appropriate)
84182 AN1BC (if appropriate)
86256 AN1TC (if appropriate)
84182 AN2BC (if appropriate)
86256 DPPTC (if appropriate)
86256 GABIC (if appropriate)
86255 GFACC (if appropriate)
86256 GFATC (if appropriate)
86255 GL1CC (if appropriate)
86256 GL1TC (if appropriate)
86255 NCDCC (if appropriate)
86256 NCDTC (if appropriate)
86256 NMDIC (if appropriate)
86053 NMOTC (if appropriate)
84182 PCTBC (if appropriate)
86256 PCTTC (if appropriate)
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PCDEC | Peds Autoimm Enceph CNS, CSF | 101416-6 |
| 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 |
| 38325 | NMO/AQP4-IgG FACS, CSF | 46718-3 |
| 605132 | Peds Autoimmune CNS Interp, CSF | 69048-7 |
| 3852 | ANNA-1, CSF | 44768-0 |
| 21631 | PCA-Tr, CSF | 90845-9 |
| 21702 | GAD65 Ab Assay, CSF | 94359-7 |
| 615866 | Neurochondrin IFA, CSF | 101451-3 |
| 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 |
| 618906 | IFA Notes | 48767-8 |