Encephalopathy, Autoimmune/Paraneoplastic 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

Forms

If not ordering electronically, complete, print, and send 1 of the following with the specimen:

-Neurology Specialty Testing Client Test Request (T732)

-General Test Request (T239)

Useful For

Evaluating new onset encephalopathy (noninfectious or metabolic) comprising confusional states, psychosis, delirium, memory loss, hallucinations, movement disorders, sensory or motor complaints, seizures, dyssomnias, ataxias, nausea, vomiting, inappropriate antidiuresis, coma, dysautonomias, or hypoventilation in spinal fluid specimens

The following accompaniments should increase of suspicion for autoimmune encephalopathy:

-Headache

-Autoimmune stigmata (personal or family history or signs of diabetes mellitus, thyroid disorder, vitiligo, poliosis [premature graying], myasthenia gravis, rheumatoid arthritis, systemic lupus erythematosus)

-History of cancer

-Smoking history (over 20 pack-years) or other cancer risk factors

-Inflammatory cerebrospinal fluid (or isolated protein elevation)

-Neuroimaging signs suggesting inflammation

Evaluating limbic encephalitis (noninfectious)

Directing a focused search for cancer

Investigating encephalopathy appearing in the course or wake of cancer therapy and not explainable by metastasis or drug effect

Profile Information

Test ID	Reporting Name	Available Separately	Always Performed
AEECI	Encephalopathy, Interpretation, CSF	No	Yes
AMPCC	AMPA-R Ab CBA, CSF	No	Yes
AMPHC	Amphiphysin Ab, CSF	No	Yes
AGN1C	Anti-Glial Nuclear Ab, Type 1	No	Yes
ANN1C	Anti-Neuronal Nuclear Ab, Type 1	No	Yes
ANN2C	Anti-Neuronal Nuclear Ab, Type 2	No	Yes
ANN3C	Anti-Neuronal Nuclear Ab, Type 3	No	Yes
CS2CC	CASPR2-IgG CBA, CSF	No	Yes
CRMC	CRMP-5-IgG, 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
IG5IC	IgLON5 IFA, CSF	No	Yes
LG1CC	LGI1-IgG CBA, CSF	No	Yes
GL1IC	mGluR1 Ab IFA, CSF	No	Yes
NIFIC	NIF IFA, CSF	No	Yes
NMDCC	NMDA-R Ab CBA, CSF	No	Yes
PCTRC	Purkinje Cell Cytoplasmc Ab Type Tr	No	Yes
PCA1C	Purkinje Cell Cytoplasmic Ab Type 1	No	Yes
PCA2C	Purkinje Cell Cytoplasmic Ab Type 2	No	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AGNBC	AGNA-1 Immunoblot, CSF	No	No
AINCC	Alpha Internexin CBA, CSF	No	No
AMPIC	AMPA-R Ab IF Titer Assay, CSF	No	No
AMIBC	Amphiphysin Immunoblot, CSF	No	No
AN1BC	ANNA-1 Immunoblot, CSF	No	No
AN2BC	ANNA-2 Immunoblot, CSF	No	No
CRMWC	CRMP-5-IgG Western Blot, CSF	Yes	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
IG5CC	IgLON5 CBA, CSF	No	No
IG5TC	IgLON5 IFA Titer, CSF	No	No
GL1CC	mGluR1 Ab CBA, CSF	No	No
GL1TC	mGluR1 Ab IFA Titer, CSF	No	No
NFHCC	NIF Heavy Chain CBA, CSF	No	No
NIFTC	NIF IFA Titer, CSF	No	No
NFLCC	NIF Light Chain CBA, CSF	No	No
NMDIC	NMDA-R Ab IF Titer Assay, CSF	No	No
PC1BC	PCA-1 Immunoblot, CSF	No	No
PCTBC	PCA-Tr Immunoblot, CSF	No	No

Testing Algorithm

If client requests or if immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then CRMP-5-IgG Western blot is performed at an additional charge.

If IFA patterns suggest amphiphysin antibody, then amphiphysin immunoblot is performed at an additional charge.

If IFA pattern suggests antiglial nuclear antibody (AGNA-1) antibody, then AGNA-1 immunoblot is performed at an additional charge.

If IFA pattern suggests antineuronal nuclear antibody (ANNA-1) antibody, then ANNA-1 immunoblot is performed at an additional charge.

If IFA pattern suggests ANNA-2 antibody, then ANNA-2 immunoblot is performed at an additional charge.

If IFA pattern suggests Purkinje cytoplasmic antibody (PCA-1) antibody, then PCA-1 immunoblot is performed at an additional charge.

If IFA pattern suggests PCA-Tr antibody, then PCA-Tr immunoblot is performed at an additional charge.

If IFA pattern suggests IgLON5 antibody, then IgLON5 IFA titer IgLON5 cell-binding assay (CBA) is performed at an additional charge.

If IFA pattern suggests alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA)-receptor antibody, and AMPA-receptor antibody CBA is positive, then AMPA-receptor antibody IFA titer assay is performed at an additional charge.

If IFA pattern suggests gamma-aminobutyric acid B (GABA-B)-receptor antibody, and GABA-B-receptor antibody CBA is positive, then GABA-B-receptor antibody IFA titer assay is performed at an additional charge.

If IFA pattern suggests glial fibrillary acidic protein (GFAP) antibody, then GFAP IFA titer and GFAP CBA are performed at an additional charge.

If IFA pattern suggests N-methyl-D-aspartate (NMDA)-receptor antibody, and NMDA-receptor antibody CBA is positive, then NMDA-receptor antibody IFA titer assay is performed at an additional charge.

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

If IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 antibody CBA and mGluR1 titer are performed at an additional charge.

If IFA pattern suggests neuronal intermediate filament (NIF) antibody, then alpha internexin CBA, NIF heavy chain CBA, NIF light chain CBA, and NIF titer are performed at an additional charge.

For more information, see the following algorithms:

Autoimmune/Paraneoplastic Encephalopathy Evaluation Algorithm-Spinal Fluid

Central Nervous System Demyelinating Disease Diagnostic Algorithm

Special Instructions

Method Name

AGN1C, AMPHC, AMPIC, ANN1C, ANN2C, ANN3C, CRMC, DPPIC, DPPTC, GABIC, GFAIC, GFATC, GL1IC, GL1TC, IG5IC, IG5TC, NIFIC, NIFTC, NMDIC, PCA1C, PCA2C, PCTRC: Indirect Immunofluorescence Assay (IFA)

AINCC, AMPCC, CS2CC, DPPCC, GABCC, GFACC, GL1CC, IG5CC, LG1CC, NFHCC, NFLCC, NMDCC: Cell-Binding Assay (CBA)

CRMWC: Western Blot (WB)

AGNBC, AMIBC, AN1BC, AN2BC, PC1BC, PCTBC: Immunoblot (IB)

GD65C: Radioimmunoassay (RIA)

Reporting Name

Enceph, Autoimm/Paraneo, 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

Reference Values

Test ID	Reporting name	Methodology	Reference value
AEECI	Encephalopathy, Interpretation, CSF	Medical interpretation	NA
AMPCC	AMPA-R Ab CBA, CSF	CBA	Negative
AMPHC	Amphiphysin Ab, CSF	IFA	<1:2
AGN1C	Anti-Glial Nuclear Ab, Type 1	IFA	<1:2
ANN1C	Anti-Neuronal Nuclear Ab, Type 1	IFA	<1:2
ANN2C	Anti-Neuronal Nuclear Ab, Type 2	IFA	<1:2
ANN3C	Anti-Neuronal Nuclear Ab, Type 3	IFA	<1:2
CS2CC	CASPR2-IgG CBA, CSF	CBA	Negative
CRMC	CRMP-5-IgG, CSF	IFA	<1:2
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
IG5IC	IgLON5 IFA, CSF	IFA	Negative
LG1CC	LGI1-IgG CBA, CSF	CBA	Negative
GL1IC	mGluR1 Ab IFA, CSF	IFA	Negative
NIFIC	NIF IFA, CSF	IFA	Negative
NMDCC	NMDA-R Ab CBA, CSF	CBA	Negative
PCTRC	Purkinje Cell Cytoplasmc Ab Type Tr	IFA	<1:2
PCA1C	Purkinje Cell Cytoplasmic Ab Type 1	IFA	<1:2
PCA2C	Purkinje Cell Cytoplasmic Ab Type 2	IFA	<1:2

Reflex Information:

Test ID	Reporting name	Methodology	Reference value
AGNBC	AGNA-1 Immunoblot, CSF	IB	Negative
AINCC	Alpha Internexin CBA, CSF	CBA	Negative
AMPIC	AMPA-R Ab IF Titer Assay, CSF	IFA	<1:2
AMIBC	Amphiphysin Immunoblot, CSF	IB	Negative
AN1BC	ANNA-1 Immunoblot, CSF	IB	Negative
AN2BC	ANNA-2 Immunoblot, CSF	IB	Negative
CRMWC	CRMP-5-IgG Western Blot, CSF	WB	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
IG5CC	IgLON5 CBA, CSF	CBA	Negative
IG5TC	IgLON5 IFA Titer, CSF	IFA	<1:2
GL1CC	mGluR1 Ab CBA, CSF	CBA	Negative
GL1TC	mGluR1 Ab IFA Titer, CSF	IFA	<1:2
NFLCC	NIF Light Chain CBA, CSF	CBA	Negative
NMDIC	NMDA-R Ab IF Titer Assay, CSF	IFA	Negative at <1:2
PC1BC	PCA-1 Immunoblot, CSF	IB	Negative
PCTBC	PCA-Tr Immunoblot, CSF	IB	Negative

*Methodology abbreviations:

Immunofluorescence assay (IFA)

Cell-binding assay (CBA)

Western blot (WB)

Radioimmunoassay (RIA)

Immunoblot (IB)

Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, CRMP-5-IgG, PCA-1, PCA-2, or PCA-Tr may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

Note: CRMP-5 titers lower than 1:2 are detectable by recombinant CRMP-5 Western blot analysis. CRMP-5 Western blot analysis will be done on request on stored spinal fluid (held 4 weeks). This supplemental testing is recommended in cases of chorea, vision loss, cranial neuropathy, and myelopathy. Call the Neuroimmunology Laboratory at 800-533-1710 to request CRMP-5 Western blot.

Day(s) Performed

Profile tests: Monday through Sunday; Reflex tests: Varies

Report Available

5 to 10 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

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 x19

86341 x1

84182 AGNBC (if appropriate)

86255 AINCC (if appropriate)

86256 AMPIC (if appropriate)

84182 AMIBC (if appropriate)

84182 AN1BC (if appropriate)

84182 AN2BC (if appropriate)

84182 CRMWC (if appropriate)

86255 DPPCC (if appropriate)

86256 DPPTC (if appropriate)

86256 GABIC (if appropriate)

86255 GFACC (if appropriate)

86256 GFATC (if appropriate)

86255 IG5CC (if appropriate)

86256 IG5TC (if appropriate)

86255 GL1CC (if appropriate)

86256 GL1TC (if appropriate)

86255 NFHCC (if appropriate)

86256 NIFTC (if appropriate)

86255 NFLCC (if appropriate)

86256 NMDIC (if appropriate)

84182 PC1BC (if appropriate)

84182 PCTBC (if appropriate)

LOINC Code Information

Test ID	Test Order Name	Order LOINC Value
ENC2	Enceph, Autoimm/Paraneo, CSF	94708-5

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
34256	Encephalopathy, Interpretation, CSF	69048-7
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
606965	NIF IFA, CSF	96490-8
606947	IgLON5 IFA, CSF	96479-1
89079	AGNA-1, CSF	94355-5
5906	Amphiphysin Ab, CSF	94354-8
3852	ANNA-1, CSF	94356-3
7472	ANNA-2, CSF	94357-1
21633	ANNA-3, CSF	94358-9
21650	CRMP-5-IgG, CSF	94706-9
3988	PCA-1, CSF	94363-9
21632	PCA-2, CSF	94364-7
21631	PCA-Tr, CSF	94362-1
21702	GAD65 Ab Assay, CSF	94359-7
36429	Reflex Added	77202-0

NY State Approved

Yes

Mayo Clinic Laboratories Rochester

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Test ID ENC2 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid