Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid

Ordering Guidance

Multiple neurological phenotype-specific autoimmune/paraneoplastic evaluations are available. For more information as well as phenotype-specific testing options, see Autoimmune Neurology Test Ordering Guide.

When more than one evaluation is ordered on the same order number, the duplicate test will be canceled.

For a list of antibodies performed with each evaluation, see Autoimmune Neurology Antibody Matrix.

This test is intended to be ordered for adult patients. If this test is ordered for a patient younger than 18 years, it will be canceled and automatically reordered by the laboratory as PCDEC / Pediatric Autoimmune Encephalopathy/CNS Disorder Evaluation, Spinal Fluid. The pediatric autoimmune CNS disorders evaluation is part of an evolving approach to testing for autoimmune neurological disorders using phenotypic-specific evaluations that include multiple antibodies known for their disease association.

Necessary Information

Provide the following information:

-Relevant clinical information

-Ordering healthcare professional's name, phone number, mailing address, and email address

Specimen Required

Container/Tube: Sterile vial

Preferred: Collection vial number 1

Acceptable: Any collection 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)

-Microbiology Test Request (T244)

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 using spinal fluid specimens

The following accompaniments should increase 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 (20 or more 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 during or after 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
AK5IC	AK5 Ab IFA, 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
DPPCC	DPPX Ab CBA, CSF	No	Yes
GBACC	GABA-A-R Ab CBA, CSF	Yes	Yes
GABCC	GABA-B-R Ab CBA, CSF	No	Yes
GD65C	GAD65 Ab Assay, CSF	Yes	Yes
GFAIC	GFAP IFA, CSF	No	Yes
IG5CC	IgLON5 CBA, CSF	No	Yes
KLHCC	KLHL11-IgG CBA, CSF	Yes	Yes
LG1CC	LGI1-IgG CBA, CSF	No	Yes
MA2EC	Ma2 Ab ELISA, CSF	Yes	Yes
GL1IC	mGluR1 Ab IFA, CSF	No	Yes
GL5IC	mGluR5 Ab IFA, CSF	No	Yes
NCDIC	Neurochondrin IFA, CSF	No	Yes
NIFIC	NIF IFA, CSF	No	Yes
NMDCC	NMDA-R Ab CBA, CSF	No	Yes
PDEIC	PDE10A Ab IFA, 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
SP7IC	Septin-7 IFA, CSF	No	Yes
T46IC	TRIM46 Ab IFA, CSF	No	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AGNBC	AGNA-1 Immunoblot, CSF	No	No
AGNTC	AGNA-1 Titer, CSF	No	No
AK5CC	AK5 Ab CBA, CSF	No	No
AK5TC	AK5 Ab IFA Titer, CSF	No	No
AINCC	Alpha Internexin CBA, CSF	No	No
AMPIC	AMPA-R Ab IF Titer Assay, CSF	No	No
APHTC	Amphiphysin Ab Titer, CSF	No	No
AMIBC	Amphiphysin Immunoblot, CSF	No	No
AN1BC	ANNA-1 Immunoblot, CSF	No	No
AN1TC	ANNA-1 Titer, CSF	No	No
AN2BC	ANNA-2 Immunoblot, CSF	No	No
AN2TC	ANNA-2 Titer, CSF	No	No
AN3TC	ANNA-3 Titer, CSF	No	No
CRMTC	CRMP-5-IgG Titer, 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
IG5TC	IgLON5 IFA Titer, CSF	No	No
K11TC	KLHL11 Ab IFA Titer, CSF	No	No
MP1BC	MAP1B Immunoblot, CSF	No	No
GL1CC	mGluR1 Ab CBA, CSF	No	No
GL1TC	mGluR1 Ab IFA Titer, CSF	No	No
GL5CC	mGluR5 Ab CBA, CSF	No	No
GL5TC	mGluR5 Ab IFA Titer, CSF	No	No
NCDCC	Neurochondrin CBA, CSF	No	No
NCDTC	Neurochondrin 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
PC1TC	PCA-1 Titer, CSF	No	No
PC2TC	PCA-2 Titer, CSF	No	No
PCTBC	PCA-Tr Immunoblot, CSF	No	No
PCTTC	PCA-Tr Titer, CSF	No	No
PDETC	PDE10A Ab IFA Titer, CSF	No	No
SP7CC	Septin-7 CBA, CSF	No	No
SP7TC	Septin-7 IFA Titer, CSF	No	No
T46CC	TRIM46 Ab CBA, CSF	No	No
T46TC	TRIM46 Ab IFA Titer, CSF	No	No
CRMWC	CRMP-5-IgG Western Blot, CSF	No	No

Testing Algorithm

To determine the necessity of laboratory testing for patients with suspected autoimmune encephalitis, epilepsy or dementia, see the Antibody Prevalence in Epilepsy and Encephalopathy (APE2) scorecard.

If the immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then the CRMP-5-IgG IFA titer and CRMP-5-IgG Western blot will be performed at an additional charge.

If the IFA pattern suggests adenylate kinase 5 protein (AK5) antibody, then AK5 IFA titer and AK5 cell-binding assay (CBA) will be performed at an additional charge.

If the IFA patterns suggest amphiphysin antibody, then the amphiphysin IFA titer and amphiphysin immunoblot will be performed at an additional charge.

If the IFA pattern suggests antiglial nuclear antibody (AGNA-1), then the AGNA-1 IFA titer and AGNA-1 immunoblot will be performed at an additional charge.

If the IFA pattern suggests antineuronal nuclear antibody type 1 (ANNA-1), then the ANNA-1 IFA titer, ANNA-1 immunoblot, and ANNA-2 immunoblot will be performed at an additional charge.

If the IFA pattern suggests ANNA-2 antibody, then the ANNA-2 IFA titer, ANNA-1 immunoblot, and ANNA-2 immunoblot will be performed at an additional charge.

If the client requests or the IFA pattern suggests ANNA-3 antibodies, then the ANNA-3 titer will be performed at an additional charge.

If the IFA pattern suggests Purkinje cell cytoplasmic antibody type 1 (PCA-1), then the PCA-1 IFA titer and PCA-1 immunoblot will be performed at an additional charge.

If the IFA pattern suggests PCA-2 antibody, then the PCA-2 IFA titer and microtubule-associated protein 1B (MAP1B) immunoblot will be performed at an additional charge.

If the IFA pattern suggests PCA-Tr antibody, then the PCA-Tr IFA titer and PCA-Tr immunoblot will be performed at an additional charge.

If the IgLON5 antibody CBA result is positive, then the IgLON5 IFA titer will be performed at an additional charge.

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

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

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

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

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

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

If the IFA pattern suggests mGluR5 antibody, then the mGluR5 antibody CBA and mGluR5 IFA titer will be performed at an additional charge.

If the Kelch-like protein 11 (KLHL11) CBA result is reactive, then the KLHL11 antibody IFA titer will be performed at an additional charge.

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

If the IFA pattern suggests neurochondrin antibody, then the neurochondrin antibody CBA and neurochondrin IFA titer will be performed at an additional charge.

If the IFA pattern suggests septin-7 antibody, then the septin-7 antibody CBA and septin-7 IFA titer will be performed at an additional charge.

If the IFA pattern suggests tripartite motif-containing protein 46 (TRIM46) antibody, then the TRIM46 antibody CBA and TRIM46 IFA titer will be performed at an additional charge.

If the IFA pattern suggests phosphodiesterase 10A (PDE10A) antibody, then the PDE10A antibody IFA titer will be 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

AEECI: Technical Interpretation

AGN1C, AK5IC, AMPHC, ANN1C, ANN2C, ANN3C, CRMC, GFAIC, GL1IC, GL5IC, NCDIC, NIFIC, PCA1C, PCA2C, PCTRC, PDEIC, SP7IC, T46IC, AGNTC, AK5TC, AMPIC, AN1TC, AN2TC, AN3TC, APHTC, CRMTC, DPPTC, GABIC, GFATC, GL1TC, GL5TC, IG5TC, K11TC, NCDTC, NIFTC, NMDIC, PC1TC, PC2TC, PCTTC, PDETC, SP7TC, T46TC: Indirect Immunofluorescence Assay (IFA)

AMPCC, CS2CC, DPPCC, GABCC, GBACC, IG5CC, LG1CC, NMDCC, KLHCC, AINCC, AK5CC, GFACC, GL1CC, GL5CC, NCDCC, NFHCC, NFLCC, SP7CC, T46CC: Cell Binding Assay (CBA)

MA2EC: Enzyme-Linked Immunosorbent Assay (ELISA)

CRMWC: Western Blot (WB)

GD65C: Radioimmunoassay (RIA)

AGNBC, AMIBC, AN1BC, AN2BC, MP1BC, PC1BC, PCTBC: Immunoblot (IB)

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

Reject Due To

Gross hemolysis	Reject
Gross lipemia	Reject
Gross icterus	Reject

Reference Values

Test ID	Reporting name	Methodology*	Reference value
AEECI	Encephalopathy, Interpretation, CSF	Technical interpretation	Interpretive report
AK5IC	AK5 Ab IFA, CSF	IFA	Negative
AMPCC	AMPA-R Ab CBA, CSF	CBA	Negative
AMPHC	Amphiphysin Ab, CSF	IFA	Negative
AGN1C	Anti-Glial Nuclear Ab, Type 1	IFA	Negative
ANN1C	Anti-Neuronal Nuclear Ab, Type 1	IFA	Negative
ANN2C	Anti-Neuronal Nuclear Ab, Type 2	IFA	Negative
ANN3C	Anti-Neuronal Nuclear Ab, Type 3	IFA	Negative
CS2CC	CASPR2-IgG CBA, CSF	CBA	Negative
CRMC	CRMP-5-IgG, CSF	IFA	Negative
DPPCC	DPPX Ab CBA, CSF	CBA	Negative
GBACC	GABA-A-R Ab CBA, CSF	CBA	Negative
GABCC	GABA-B-R Ab CBA, CSF	CBA	Negative
GD65C	GAD65 Ab Assay, CSF	RIA	≤0.02 nmol/L
GFAIC	GFAP IFA, CSF	IFA	Negative
IG5CC	IgLON5 CBA, CSF	CBA	Negative
KLHCC	KLHL11-IgG CBA, CSF	CBA	Negative
LG1CC	LGI1-IgG CBA, CSF	CBA	Negative
MA2EC	Ma2 Ab ELISA, CSF	ELISA	Negative
GL1IC	mGluR1 Ab IFA, CSF	IFA	Negative
GL5IC	mGluR5 Ab IFA, CSF	IFA	Negative
NCDIC	Neurochondrin IFA, CSF	IFA	Negative
NIFIC	NIF IFA, CSF	IFA	Negative
NMDCC	NMDA-R Ab CBA, CSF	CBA	Negative
PDEIC	PDE10A Ab IFA, CSF	IFA	Negative
PCTRC	Purkinje Cell Cytoplasmc Ab Type Tr	IFA	Negative
PCA1C	Purkinje Cell Cytoplasmic Ab Type 1	IFA	Negative
PCA2C	Purkinje Cell Cytoplasmic Ab Type 2	IFA	Negative
SP7IC	Septin-7 IFA, CSF	IFA	Negative
T46IC	TRIM46 Ab IFA, CSF	IFA	Negative

Reflex Information:

Test ID	Reporting name	Methodology*	Reference value
AGNBC	AGNA-1 Immunoblot, CSF	IB	Negative
AGNTC	AGNA-1 Titer, CSF	IFA	<1:2
AK5CC	AK5 Ab CBA, CSF	CBA	Negative
AK5TC	AK5 Ab IFA Titer, CSF	IFA	<1:2
AINCC	Alpha Internexin CBA, CSF	CBA	Negative
AMPIC	AMPA-R Ab IF Titer Assay, CSF	IFA	<1:2
APHTC	Amphiphysin Ab Titer, CSF	IFA	<1:2
AMIBC	Amphiphysin Immunoblot, CSF	IB	Negative
AN1BC	ANNA-1 Immunoblot, CSF	IB	Negative
AN1TC	ANNA-1 Titer, CSF	IFA	<1:2
AN2BC	ANNA-2 Immunoblot, CSF	IB	Negative
AN2TC	ANNA-2 Titer, CSF	IFA	<1:2
AN3TC	ANNA-3 Titer, CSF	IFA	<1:2
CRMTC	CRMP-5-IgG Titer, CSF	IFA	<1:2
CRMWC	CRMP-5-IgG Western Blot, CSF	WB	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
IG5TC	IgLON5 IFA Titer, CSF	IFA	<1:2
K11TC	KLHL11 Ab IFA Titer, CSF	IFA	<1:2
MP1BC	MAP1B Immunoblot, CSF	IB	Negative
GL1CC	mGluR1 Ab CBA, CSF	CBA	Negative
GL1TC	mGluR1 Ab IFA Titer, CSF	IFA	<1:2
GL5CC	mGluR5 Ab CBA, CSF	CBA	Negative
GL5TC	mGluR5 Ab IFA Titer, CSF	IFA	<1:2
NCDCC	Neurochondrin CBA, CSF	CBA	Negative
NCDTC	Neurochondrin IFA Titer, CSF	IFA	<1:2
NFHCC	NIF Heavy Chain CBA, CSF	CBA	Negative
NIFTC	NIF IFA Titer, CSF	IFA	<1:2
NFLCC	NIF Light Chain CBA, CSF	CBA	Negative
NMDIC	NMDA-R Ab IF Titer Assay, CSF	IFA	<1:2
PC1BC	PCA-1 Immunoblot, CSF	IB	Negative
PC1TC	PCA-1 Titer, CSF	IFA	<1:2
PC2TC	PCA-2 Titer, CSF	IFA	<1:2
PCTBC	PCA-Tr Immunoblot, CSF	IB	Negative
PCTTC	PCA-Tr Titer, CSF	IFA	<1:2
PDETC	PDE10A Ab IFA Titer, CSF	IFA	<1:2
SP7CC	Septin-7 CBA, CSF	CBA	Negative
SP7TC	Septin-7 IFA Titer, CSF	IFA	<1:2
T46CC	TRIM46 Ab CBA, CSF	CBA	Negative
T46TC	TRIM46 Ab IFA Titer, CSF	IFA	<1:2

*Methodology abbreviations:

Cell-binding assay (CBA)

Enzyme-linked immunosorbent assay (ELISA)

Immunoblot (IB)

Immunofluorescence assay (IFA)

Radioimmunoassay (RIA)

Western blot (WB)

Day(s) Performed

Profile tests: Monday through Sunday; Reflex tests: Varies

Report Available

8 to 12 days

CPT Code Information

86255 x 26

86341

0432U

83516

84182 AGNBC (if appropriate)

86256 AGNTC (if appropriate)

86255 AK5CC (if appropriate)

86256 AK5TC (if appropriate)

86255 AINCC (if appropriate)

86256 AMPIC (if appropriate)

86256 APHTC (if appropriate)

84182 AMIBC (if appropriate)

84182 AN1BC (if appropriate)

86256 AN1TC (if appropriate)

84182 AN2BC (if appropriate)

86256 AN2TC (if appropriate)

86256 AN3TC (if appropriate)

86256 CRMTC (if appropriate)

84182 CRMWC (if appropriate)

86256 DPPTC (if appropriate)

86256 GABIC (if appropriate)

86255 GFACC (if appropriate)

86256 GFATC (if appropriate)

86256 IG5TC (if appropriate)

86256 K11TC (if appropriate)

84182 MP1BC (if appropriate)

86255 GL1CC (if appropriate)

86256 GL1TC (if appropriate)

86255 GL5CC (if appropriate)

86256 GL5TC (if appropriate)

86255 NCDCC (if appropriate)

86256 NCDTC (if appropriate)

86255 NFHCC (if appropriate)

86256 NIFTC (if appropriate)

86255 NFLCC (if appropriate)

86256 NMDIC (if appropriate)

84182 PC1BC (if appropriate)

86256 PC1TC (if appropriate)

86256 PC2TC (if appropriate)

84182 PCTBC (if appropriate)

86256 PCTTC (if appropriate)

86256 PDETC (if appropriate)

86255 SP7CC (if appropriate)

86256 SP7TC (if appropriate)

86255 T46CC (if appropriate)

86256 T46TC (if appropriate)

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Orderable Name ENC2 Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid