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HelpTest ID: 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 healthcare professional's name, phone number, mailing address, and email 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 spinal 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-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 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 result 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 |
|---|---|---|
| CSF | 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, 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.
Clinical Reference
1. Dubey D, Pittock SJ, Krecke KN, et al. Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 2019;76(3):301-309 doi:10.1001/jamaneurol.2018.4053
2. McKeon A, Lennon VA, Lotze T, et al. CNS aquaporin-4 autoimmunity in children. Neurology. 2008;71(2):93-100
3. Dubey D, Hinson SR, Jolliffe EA, et al. Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year. J Neuroimmunol. 2018;321:157-163
4. Philipps G, Alisanski SB, Pranzatelli M, et al. Purkinje cell cytoplasmic antibody type 1 (anti-Yo) autoimmunity in a child with Down syndrome. JAMA Neurol. 2014;71(3):347-349
5. Lopez-Chiriboga AS, Klein C, Zekeridou A, et al. LGI1 and CASPR2 neurological autoimmunity in children. Ann Neurol. 2018;84(3):473-480
6. Lopez-Chiriboga AS, Majed M, Fryer J, et al. Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol. 2018;75(11):1355-1363
7. Clardy SL, Lennon VA, Dalmau J. Childhood onset of stiff-man syndrome. JAMA Neurol. 2013;70(12):1531-1536
8. Banwell B, Tenembaum S, Lennon VA, et al. Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Neurology. 2008;70(5):344-352
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
8 to 12 daysTest 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 |
Special Instructions
Forms
If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.
mml-neuroimmunology, mml-pediatric