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Test 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 Encephalopathy/Central Nervous System Disorders Evaluation Algorithm-Spinal Fluid

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, CSF

Specimen Type

CSF

Specimen 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 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

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