Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid - Mayo Clinic Laboratories

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

Preferred: Vial number 1

Acceptable: Any 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
NCDIC	Neurochondrin IFA, CSF	No	Yes
NMDCC	NMDA-R Ab CBA, CSF	No	Yes
PCTRC	Purkinje Cell Cytoplasmc Ab Type Tr	No	Yes
NMOFC	NMO/AQP4 FACS, CSF	Yes	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AN1BC	ANNA-1 Immunoblot, CSF	No	No
AN2BC	ANNA-2 Immunoblot, CSF	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
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 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 PCA-Tr immunoblot and PCA-Tr IFA titer will be performed at an additional charge.

If N-methyl-D-aspartate receptor (NMDA-R) antibody CBA is positive, then NMDA-R IFA titer will be performed at an additional charge.

If gamma-aminobutyric acid B receptor (GABA-B-R) antibody CBA is positive, then GABA-B-R IFA titer will be performed at an additional charge.

If the IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX IFA titer will be performed at an additional charge.

If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then 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 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 NMO/AQP4-IgG FACS titration assay will be performed at an additional charge.

If the IFA pattern suggests neurochondrin antibody, then neurochondrin antibody CBA and neurochondrin IFA titer will be 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

CS2CS, DPPCC, GABCC, GFACC, LG1CC, GL1CC, NCDCC, NMDCC: Cell Binding Assay (CBA)

NMOFC, NMOTC: Flow Cytometry

ANN1C, AN1TC, DPPIC, DPPTC, GABIC, GFAIC, GFATC, GL1IC, GL1TC, NCDIC, NCDTC, NMDIC, PCTRC, PCTTC: Indirect Immunofluorescence Assay (IFA)

GD65C: Radioimmunoassay (RIA)

AN1BC, AN2BC, PCTBC: Immunoblot (IB)

Reporting Name

Peds Autoimm Enceph CNS, CSF

Specimen Type

CSF

Specimen 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	NA
ANN1C	Anti-Neuronal Nuclear Ab, Type 1	IFA	Negative
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
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
AN1BC	ANNA-1 Immunoblot, CSF	IB	Negative
AN1TC	ANNA-1 Titer, CSF	IFA	<1:2
AN2BC	ANNA-2 Immunoblot, CSF	IB	Negative
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
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 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 12 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 10

86341

86053

84182 AN1BC (if appropriate)

86256 AN1TC (if appropriate)

84182 AN2BC (if appropriate)

86255 DPPCC (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
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
615866	Neurochondrin IFA, CSF	101451-3
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
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.

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Test ID: PCDEC Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid