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HelpTest ID: PCDEC Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid
Ordering Guidance
Multiple neuroimmunology profile tests are available. For testing that is performed with each profile, 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
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 |
| ANN1C | Anti-Neuronal Nuclear Ab, Type 1 | No | Yes |
| CS2CC | CASPR2-IgG CBA, CSF | No | Yes |
| DPPIC | DPPX Ab IFA, 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 |
| 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 |
|---|---|---|---|
| AGN1C | Anti-Glial Nuclear Ab, Type 1 | No | No |
| AGNBC | AGNA-1 Immunoblot, CSF | No | No |
| AMIBC | Amphiphysin Immunoblot, CSF | No | No |
| AMPCC | AMPA-R Ab CBA, CSF | No | No |
| AMPHC | Amphiphysin Ab, CSF | No | No |
| AMPIC | AMPA-R Ab IF Titer Assay, CSF | No | No |
| AN1BC | ANNA-1 Immunoblot, CSF | No | No |
| AN2BC | ANNA-2 Immunoblot, CSF | No | No |
| ANN2C | Anti-Neuronal Nuclear Ab, Type 2 | No | No |
| ANN3C | Anti-Neuronal Nuclear Ab, Type 3 | No | No |
| DPPCC | DPPX Ab CBA, 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 |
| PC1BC | PCA-1 Immunoblot, CSF | No | No |
| PCA1C | Purkinje Cell Cytoplasmic Ab Type 1 | No | No |
| PCA2C | Purkinje Cell Cytoplasmic Ab Type 2 | No | No |
| PCTBC | PCA-Tr Immunoblot, CSF | No | No |
Testing Algorithm
If indirect immunofluorescence assay (IFA) patterns suggest antineuronal nuclear antibodies (ANNA)-1, then ANNA-1 immunoblot and ANNA-2 immunoblot are performed at an additional charge.
If IFA patterns suggest ANNA-2 antibody, then ANNA-2 immunoblot, ANNA-1 immunoblot, and ANNA-2 antibody IFA are performed at an additional charged.
If IFA patterns suggest ANNA-3 antibody, then ANNA-3 IFA is performed at an additional charge.
If IFA patterns suggest Purkinje cytoplasmic antibody (PCA)-1, then PCA-1 immunoblot and PCA-1 IFA are performed at an additional charge.
If IFA patterns suggest PCA-2 antibody, then PCA-2 IFA is performed at an additional charge.
If IFA patterns suggest PCA-Tr antibody, then PCA-Tr immunoblot is performed at an additional charge.
If IFA pattern suggests amphiphysin antibody, then amphiphysin immunoblot and amphiphysin antibody IFA titer are performed at an additional charge.
If IFA pattern suggests anti-glial nuclear antibody (AGNA), then AGNA immunoblot and AGNA antibody IFA titer are performed at an additional charge.
If IFA pattern suggests N-methyl-D-aspartate receptor (NMDA-R) antibody, then NMDA-R IFA titer is performed at an additional charge.
If IFA pattern suggests alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor (AMPA-R) antibody, then AMPA-R antibody cell-binding assay (CBA) and AMPA-R IFA are performed at an additional charge.
If IFA pattern suggests gamma-aminobutyric acid B receptor (GABA-B-R) antibody, then GABA-B-R IFA titer is performed at an additional charge.
If IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX IFA titer are performed at an additional charge.
If IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1antibody CBA and mGluR1 IFA titer are performed at an additional charge.
If IFA pattern suggests glial fibrillary acidic protein (GFAP) antibody, then GFAP antibody CBA and GFAP IFA titer are performed at an additional charge.
If 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 is performed at an additional charge.
For more information, see the following:
Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm
Method Name
CS2CC, DPPCC, GABCC, AMPCC, GFACC, GL1CC, LG1CC, NMDCC: Cell-Binding Assay (CBA)
NMOFC, NMOTC: Flow Cytometry
AGN1C, AMPIC, ANN1C, ANN2C, ANN3C, DPPIC, DPPTC, GABIC, GFAIC, GFATC, GL1IC, GL1TC, NMDIC, PCA1C, PCA2C, PCTRC: Indirect Immunofluorescence (IFA)
GD65C: Radioimmunoassay (RIA)
AGNBC, AMIBC, AN1BC, AN2BC, PC1BC, PC2BC: Immunoblot (IB)
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 |
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 |
NA |
|
ANN1C |
Anti-Neuronal Nuclear Ab, Type 1 |
IFA |
Negative at <1:2* |
|
CS2CC |
CASPR2-IgG CBA, CSF |
CBA |
Negative |
|
DPPIC |
DPPX Ab IFA, CSF |
IFA |
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 |
|
NMDCC |
NMDA-R Ab CBA, CSF |
CBA |
Negative |
|
NMOFC |
NMO/AQP4 FACS, CSF |
FACS |
Negative |
|
PCTRC |
Purkinje Cell Cytoplasmc Ab Type Tr |
IFA |
<1:2* |
Reflex Information:
|
Test ID |
Reporting Name |
Methodology |
Reference Value |
|
AGN1C |
Anti-Glial Nuclear Ab Type 1 |
IFA |
<1:2 |
|
AGNBC |
AGNA-1 Immunoblot, CSF |
IB |
Negative |
|
AMIBC |
Amphiphysin Immunoblot, CSF |
IB |
Negative |
|
AMPCC |
AMPA-R Ab CBA, CSF |
CBA |
Negative |
|
AMPHC |
Amphiphysin Ab, CSF |
IFA |
<1:2 |
|
AMPIC |
AMPA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
|
ANN2C |
Anti-Neuronal Nuclear Ab, Type 2 |
IFA |
<1:2* |
|
AN1BC |
ANNA-1 Immunoblot, CSF |
IB |
Negative |
|
AN2BC |
ANNA-2 Immunoblot, CSF |
IB |
Negative |
|
ANN3C |
Anti-Neuronal Nuclear Ab, Type 3 |
IFA |
<1:2* |
|
DPPCC |
DPPX Ab CBA, CSF |
CBA |
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 |
|
NMDIC |
NMDA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
|
NMOTC |
NMO/AQP4 FACS Titer, CSF |
FACS |
<1:2 |
|
PC1BC |
PCA-1 Immunoblot, CSF |
IB |
Negative |
|
PCA1C |
Purkinje Cell Cytoplasmic Ab Type 1 |
IFA |
<1:2* |
|
PCA2C |
Purkinje Cell Cytoplasmic Ab Type 2 |
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, ANNA-2, ANNA-3, PCA-1, PCA-2, 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 Mar 1;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 Jul 8;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 Aug 15;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 Mar;71(3):347-349
5. Lopez-Chiriboga AS, Klein C, Zekeridou A, et al: LGI1 and CASPR2 neurological autoimmunity in children. Ann Neurol. 2018 Sep;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 Nov 1;75(11):1355-1363
7. Clardy SL, Lennon VA, Dalmau J: Childhood onset of stiff-man syndrome. JAMA Neurol. 2013 Dec;70(12):1531-1536
8. Banwell B, Tenembaum S, Lennon VA, et al: Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Neurology. 2008 Jan 29;70(5):344-352
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
8 to 11 daysTest Classification
This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86255 x 9
86053
86341
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PCDEC | Peds Autoimm Enceph CNS, CSF | In Process |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 61513 | NMDA-R Ab CBA, CSF | 93502-3 |
| 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 | 94356-3 |
| 21631 | PCA-Tr, CSF | 94362-1 |
| 21702 | GAD65 Ab Assay, CSF | 94359-7 |
| 64280 | LGI1-IgG CBA, CSF | 94288-8 |
| 64282 | CASPR2-IgG CBA, CSF | 94286-2 |
| 64929 | DPPX Ab IFA, CSF | 82989-5 |
| 64927 | mGluR1 Ab IFA, CSF | 94361-3 |
| 605156 | GFAP IFA, CSF | 94360-5 |
| 36429 | Reflex Added | 77202-0 |
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