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Encephalopathy, Autoimmune Evaluation, Spinal Fluid

Additional Codes

Primary ID

Epic Code

Mayo Test ID

ENAEC2

LAB3710 ENC2


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 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 (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 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 AGNA-1 antibody, then AGNA-1 immunoblot is performed at an additional charge.

 

If IFA pattern suggests 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 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 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 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 GFAP antibody, then GFAP IFA titer and GFAP CBA are performed at an additional charge.

 

If IFA pattern suggests 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 DPPX antibody, then DPPX antibody CBA and DPPX titer are performed at an additional charge.

 

If IFA pattern suggests mGluR1 antibody, then mGluR1 antibody CBA and mGluR1 titer are performed at an additional charge.

 

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

 

See Encephalopathy Autoimmune Evaluation Algorithm-Spinal Fluid in 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

Encephalopathy-Autoimmune Eval, 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

Reject Due To

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus Reject

Reference Values

 Test ID

Reporting name

Methodology

Reference value

AMPCC

AMPA-R Ab CBA, CSF

Cell-binding assay (CBA)

Negative

AMPHC

Amphiphysin Ab, CSF

Indirect Immunofluorescence (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

Radioimmunoassay (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

Immunoblot (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

Western Blot

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

 

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) and Time(s) Performed

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

Monday through Friday; 5 a.m., 7 a.m., 5 p.m.

Saturday, Sunday; 6 a.m.

 

AMPCC, CS2CC, DPPCC, GABCC, LG1CC, NMDCC:

Monday through Friday; 10 p.m.

Sunday; 3 p.m.

 

GL1CC, IG5CC:

Monday, Thursday; 6 p.m.

 

AINCC, NFHCC, NFLCC:

Tuesday, Thursday; 6 p.m.

 

GFACC:

Monday, Wednesday, Friday; 6 p.m.

 

CRMWC:

Monday through Thursday; 8 a.m.

 

AGNBC, AMIBC, AN1BC, AN2BC, PC1BC, PCTBC:

Monday through Friday; 6 p.m.

 

GD65C:

Monday through Friday; 5 a.m., 2 p.m.

Saturday, Sunday; 7 a.m.

Analytic Time

8 days

Specimen Retention Time

28 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 U.S. 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 Encephalopathy-Autoimmune Eval, 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 In Process
606947 IgLON5 IFA, CSF In Process
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