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
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 in 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.
Collection Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Submission Container/Tube: Plastic vial
Specimen Volume: 4 mL
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 |
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 |
---|---|---|---|
AGN1S | Anti-Glial Nuclear Ab, Type 1 | No | No |
AGNBS | AGNA-1 Immunoblot, S | No | No |
AMIBS | Amphiphysin Immunoblot, S | No | No |
AMPCS | AMPA-R Ab CBA, S | No | No |
AMPHS | Amphiphysin Ab, S | No | No |
AMPIS | AMPA-R Ab IF Titer Assay, S | No | No |
AN1BS | ANNA-1 Immunoblot, S | No | No |
AN2BS | ANNA-2 Immunoblot, S | No | No |
ANN2S | Anti-Neuronal Nuclear Ab, Type 2 | No | No |
ANN3S | Anti-Neuronal Nuclear Ab, Type 3 | 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 |
PC1BS | PCA-1 Immunoblot, S | No | No |
PCAB2 | Purkinje Cell Cytoplasmic Ab Type 2 | No | No |
PCABP | Purkinje Cell Cytoplasmic Ab Type 1 | No | No |
PCTBS | PCA-Tr Immunoblot, S | 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 charge.
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 antibody, 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 patterns suggest amphiphysin antibody, then amphiphysin immunoblot and amphiphysin 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 titer 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.
If myelin oligodendrocyte glycoprotein (MOG) FACS screen assay requires further investigation, then MOG FACS titration assay is 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, GL1CS, LG1CS, NMDCS: Cell-Binding Assay (CBA)
MOGFS, MOGTS, NMOFS, NMOTS: Flow Cytometry
AMPHS, AGN1S, AMPIS, ANN1S, ANN2S, ANN3S, DPPIS, DPPTS, GFAIS, GFATS, GL1IS, GL1TS, NMDIS, PCAB2, PCABP, PCATR: Indirect Immunofluorescence (IFA)
GD65S: Radioimmunoassay (RIA)
AGNBS, AMIBS, AN1BS, AN2BS, PC1BS, 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 | Special Container |
---|---|---|---|
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 | ANNA-1, S | IFA | <1:240** |
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 |
MOGFS | MOG FACS, S | FACS | Negative |
NMDCS | NMDA-R Ab CBA, S | CBA | Negative |
NMOFS | NMO/AQP4 FACS, S | FACS | Negative |
PCATR | PCA-Tr, S | IFA | <1:240** |
Reflex Information:
Test ID | Reporting name | Methodology | Reference value |
AGN1S | Anti-Glial Nuclear Ab, Type 1 | IFA | <1:240 |
AGNBS | AGNA-1 Immunoblot, S | IB | Negative |
AMIBS | Amphiphysin Immunoblot, s | IB | Negative |
AMPCS | AMPA-R Ab CBA, S | CBA | Negative |
AMPHS | Amphiphysin Ab, S | IFA | <1:240 |
AMPIS | AMPA-R Ab IF Titer Assay, S | IFA | <1:120 |
AN1BS | ANNA-1 Immunoblot, S | IB | Negative |
AN2BS | ANNA-2 Immunoblot, S | IB | Negative |
ANN2S | ANNA-2, S | IFA | <1:240** |
ANN3S | ANNA-3, S | IFA | <1:240 |
DPPCS | DPPX Ab CBA, S | CBA | Negative |
DPPTS | DPPX Ab IFA Titer, 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 |
NMDIS | NMDA-R Ab IF Titer Assay, S | IFA | <1:120 |
NMOTS | NMO/AQP4 FACS Titer, S | FACS | <1:5 |
PC1BS | PCA-1 Immunoblot, S | IB | Negative |
PCAB2 | PCA-2, S | IFA | <1:240** |
PCABP | PCA-1, 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, PCA-1, PCA-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.
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. doi: 10.1016/j.jneuroim.2018.04.016
4. Philipps G, Alisanski SB, Pranzatelli M, Clardy SL, Lennon VA, McKeon A: 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. doi: 10.1002/ana.25310
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. doi: 10.1001/jamaneurol.2018.1814
7. Clardy SL, Lennon VA, Dalmau J: Childhood onset of stiff-man syndrome. JAMA Neurol. 2013 Dec;70(12):1531-1536. doi: 10.1001/jamaneurol.2013.4442
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. doi: 10.1212/01.wnl.0000284600.80782.d5
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
10 to 13 days
Test 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
86341
86255 x9
86053
86363
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 |
61519 | GABA-B-R Ab CBA, S | 93428-1 |
605131 | Peds Autoimmune CNS Interp, S | 69048-7 |
80150 | ANNA-1, S | 94342-3 |
81596 | GAD65 Ab Assay, S | 94345-6 |
83076 | PCA-Tr, S | 94352-2 |
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 |
64930 | DPPX Ab IFA, S | 82976-2 |
64928 | mGluR1 Ab IFA, S | 94347-2 |
605155 | GFAP IFA, S | 94346-4 |
36349 | 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.