Autoimmune Encephalitis Panel, Serum (Sendout)

General Information

Lab Name

Autoimmune Encephalitis Panel, Serum (Sendout)

Lab Code

RSAENP

Epic Name

Autoimmune Encephalitis Panel, Serum (Sendout)

External Test Id

ENS2

Description

    Test Information: Encephalitis is a debilitating neurologic condition caused by infectious and autoimmune conditions that result in inflammation of the brain parenchyma. The identification of autoantibodies may augment a diagnosis of autoimmune encephalitis, but is not required for the diagnosis or initiation of therapy. The most frequently identified antibodies target NMDA-R. We recommend ordering NMDA Receptor IgG Antibody with Reflex, Serum (Sendout) [RNMDA] as first-line testing for autoimmune encephalitis prior to ordering this full panel.

    Monitoring titers over time for these antibodies is not recommended; however, this recommendation is based on limited currently available literature.

    Test Algorithm: Refer to Mayo's algorithm (linked below) for additional detail on tests and potential reflex tests included in this panel. Reflex testing may be performed at additional charge.

    Ordering Guidance: Refer to Autoimmune Encephalitis Testing Recommendations or consult the Laboratory Medicine Resident on-call.

    Ordering Requirements: Orders from UW Medicine Neurology Clinics are pre-approved. For all other orders, Laboratory Medicine Resident (LMR) approval is required.

    References

    Synonyms

    AGNA-1, AMPA-R Ab CBA, Amphiphysin Ab, ANNA-1, ANNA-2, ANNA-3, Anti-Glial Nuclear Antibody, Anti-Neuronal Nuclear Antibody Type 1, Anti-Neuronal Nuclear Antibody Type 2, Anti-Neuronal Nuclear Antibody Type 3, CASPR2 IgG, Contactin-Associated Protein-Like-2 (CASPR2) IgG, CRMP-5 IgG, Dipeptidyl-Peptidase-Like Protein-6 (DPPX) Antibody, DPPX, Encephalitis, Encephalopathy, Encephalopathy Autoimmune Paraneoplastic Evaluation, Encephalopathy Autoimmune/Paraneoplastic Evaluation Serum, ENS2, GABA-B-R Antibody CBA, GAD65, GFAP, Glutamic Acid Decarboxylase, IgLON Family Member 5 Antibody, IGLON5, Leucine-Rich Glioma Inactivated Protein-1 IgG, LGI1 IgG, Limbic encephalitis, Neurochondrin, NIF, NMDA-R Ab CBA, PCA-1, PCA-2, PCA-Tr, PDE10A, Phosphodiesterase 10A (PDE10A) IgG, Purkinje Cell Cytoplasmc Antibody Type Tr, Purkinje Cell Cytoplasmic Antibody Type 1, Purkinje Cell Cytoplasmic Antibody Type 2, Septin-7, TRIM46, Tripartite Motif-Containing Protein 46 (TRIM46) IgG

    Components

    Code Name
    RAEESI Encephalopathy Interpretation, Serum
    RAESIF IFA Notes
    RAMPCS AMPA-R Antibody CBA
    RAMPHS Amphiphysin Antibody
    RAGNA1 Anti Glial Nuclear Antibody 1
    RANN1 Anti Neuronal Nuclear Antibody 1
    RANN2 Anti Neuronal Nuclear Antibody 2
    RANN3 Anti Neuronal Nuclear Antibody 3
    RCS2CS CASPR2 IgG CBA
    RCRMP CRMP 5 IgG
    RDPPCS DPPX Antibody CBA
    RGABCS GABA B R Antibody CBA
    RGAD65 GAD65 Antibody Assay
    RGFAIS GFAP IFA
    RIG5CS IgLON5 CBA
    RLG1CS LGI1 IgG CBA
    RMGL1S mGluR1 Antibody IFA
    RNCDIS Neurochondrin IFA
    RNIFIS NIF IFA
    RNMDCS NMDA R Antibody CBA
    RPCA1 Purkinje Cell Cytoplasmic Antibody 1
    RPCA2 Purkinje Cell Cytoplasmic Antibody 2
    RPCATR Purkinje Cell Cytoplasmic Antibody Tr
    RPDEIS PDE10A Antibody IFA
    RSP7IS Septin 7 IFA
    RTRM46 TRIM46 Antibody IFA

    Interpretation

    Method

    Immunofluorescence assay (IFA): Amphiphysin Ab, Anti-Glial Nuclear Ab Type 1, Anti-Neuronal Nuclear Ab Type 1, Anti-Neuronal Nuclear Ab Type 2, Anti-Neuronal Nuclear Ab Type 3, CRMP-5-IgG, GFAP, mGluR1 Ab, Neurochondrin, NIF, Purkinje Cell Cytoplasmic Ab Type 1, Purkinje Cell Cytoplasmic Ab Type 2, Purkinje Cell Cytoplasmic Ab Type Tr, Septin-7, TRIM46 Ab, PDE10A Ab.

    Cell-binding assay (CBA): AMPA-R Ab, CASPR2-IgG, GABA-B-R Ab, LGI1-IgG, NMDA-R Ab, IgLON5 Ab, DPPX Ab.

    Radioimmunoassay (RIA): GAD65 Ab Assay

    Reference Range

    See individual components

    Ref. Range Notes

    Reference Values:

    AMPA-R Ab CBA: Negative
    Amphiphysin Ab: Negative
    Anti-Glial Nuclear Ab, Type 1: Negative
    Anti-Neuronal Nuclear Ab, Type 1: Negative
    Anti-Neuronal Nuclear Ab, Type 2: Negative
    Anti-Neuronal Nuclear Ab, Type 3: Negative
    CASPR2-IgG CBA: Negative
    CRMP-5-IgG: Negative
    DPPX Ab CBA: Negative
    GABA-B-R Ab CBA: Negative
    GAD65 Ab Assay: < or =0.02 nmol/L
    GFAP IFA: Negative
    mGluR1 Ab IFA: Negative
    IgLON5 CBA: Negative
    LGI1-IgG CBA: Negative
    Neurochondrin IFA: Negative
    NIF IFA: Negative
    NMDA-R Ab CBA: Negative
    Purkinje Cell Cytoplasmic Ab Type 1: Negative
    Purkinje Cell Cytoplasmic Ab Type 2: Negative
    Purkinje Cell Cytoplasmic Ab Type Tr: Negative
    PDE10A Ab IFA: Negative
    Septin-7 IFA: Negative
    TRIM46 IFA: Negative

    Note: Includes reference values for panel tests only.

    Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, 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."

    CRMP-5 titers lower than 1:240 are detectable by recombinant CRMP-5 Western blot analysis. CRMP-5 Western blot analysis will be done on request on stored serum (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.

    Interpretation: Neuronal, glial, and muscle autoantibodies are valuable serological markers of autoimmune encephalopathy and of a patient's immune response to cancer. These autoantibodies are usually accompanied by subacute neurological symptoms and signs are not found in healthy subjects. It is not uncommon for more than 1 of the following autoantibody specificities to be detected in patients with an autoimmune encephalopathy:

    • Plasma membrane autoantibodies: N-methyl-D-aspartate (NMDA) receptor; 2-amino-3-(5-methyl-3-oxo-1,2- oxazol-4-yl) propanoic acid (AMPA) receptor; gamma-amino butyric acid (GABA-B) receptor; neuronal ACh receptor. These are all potential effectors of neurological dysfunction.

    • Neuronal nuclear autoantibodies, type 1 (ANNA-1), type 2 (ANNA-2), or type 3 (ANNA-3)

    • Neuronal or muscle cytoplasmic antibodies: amphiphysin, Purkinje cell antibodies (PCA-1) and PCA-2, CRMP-5, GAD65, or striational

    Interferences and Limitations

    Cautions:

    Negative results do not exclude autoimmune encephalopathy or cancer.

    This test does not detect Ma1 or Ma2 antibodies (also known as MaTa), which are sometimes associated with brainstem and limbic encephalitis in the context of testicular germ cell neoplasms. Scrotal ultrasound is advised for men who present with unexplained subacute encephalitis. To order Ma2 Antibody, refer to separate test guide #577.

    Intravenous immunoglobulin (IVIg) treatment prior to the serum collection may cause a false-positive result.

    Guidelines

    Ordering & Collection

    Specimen Type

    Blood

    Collection

    12 mL blood in RED TOP or GOLD SST tubes

    Approval Required

    Orders from UW Medicine Neurology Clinics are pre-approved. For all other orders, Laboratory Medicine Resident (LMR) approval is required.

    Handling Instructions

    Outside Laboratories: Centrifuge samples and transfer serum to a separate plastic vial. Refrigerate serum while awaiting shipment. Transport with a cold pack.

    Stability: Refrigerated (preferred): 28 days; Frozen: 28 days; Ambient: 72 hours.

    Reject Due To: Gross hemolysis, gross lipemia, gross icterus, insufficient sample volume.

    Quantity

    requested: 4 mL serum
    minimum: 2.5 mL serum

    Processing

    Centrifuge samples and transfer 4 mL serum (2.5 mL minimum) to a separate plastic aliquot. Refrigerate serum.

    Sendouts:

    • Order Mayo Test: ENS2
    • Interfaced: Yes [Interface: 601; Worksheet: MARF]

    Stability: Refrigerated (preferred): 28 days; Frozen: 28 days; Ambient: 72 hours.

    Reject Due To: Gross hemolysis, gross lipemia, gross icterus, insufficient sample volume.

    Performance

    LIS Dept Code

    Sendouts Mayo Lab (RF) (MARF)

    Performing Location(s)

    Sendout Mayo Clinic Laboratories
    800-533-1710

    200 First Street Southwest
    Rochester, MN 55901

    Frequency

    Performed: Varies. Report available: 10-13 days from sample receipt at Mayo Clinic Laboratories.

    Available STAT?

    No

    Billing & Coding

    CPT codes

    86255x23, 86341

    Billing Comments

    Reflex testing may be performed at additional charge.

    LOINC

    94697-0

    Interfaced Order Code

    UOW4912