| Web: | mayocliniclabs.com |
|---|---|
| Email: | mcl@mayo.edu |
| Telephone: | 800-533-1710 |
| International: | +1 855-379-3115 |
| Values are valid only on day of printing | |
Test Id : ENS2
Encephalopathy, Autoimmune Evaluation, Serum
Useful For
Suggests clinical disorders or settings where the test may be helpful
Suggests clinical disorders or settings where the test may be helpful
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 serum 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 cerebral spinal 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
A profile is a group of laboratory tests that are ordered and performed together under a single Mayo Test ID. Profile information lists the test performed, inclusive of the test fee, when a profile is ordered and includes reporting names and individual availability.
A profile is a group of laboratory tests that are ordered and performed together under a single Mayo Test ID. Profile information lists the test performed, inclusive of the test fee, when a profile is ordered and includes reporting names and individual availability.
| Test Id | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| AEESI | Encephalopathy, Interpretation, S | No | Yes |
| AMPCS | AMPA-R Ab CBA, S | No | Yes |
| AMPHS | Amphiphysin Ab, S | No | Yes |
| AGN1S | Anti-Glial Nuclear Ab, Type 1 | No | Yes |
| ANN1S | Anti-Neuronal Nuclear Ab, Type 1 | No | Yes |
| ANN2S | Anti-Neuronal Nuclear Ab, Type 2 | No | Yes |
| ANN3S | Anti-Neuronal Nuclear Ab, Type 3 | No | Yes |
| CS2CS | CASPR2-IgG CBA, S | No | Yes |
| CRMS | CRMP-5-IgG, S | No | Yes |
| DPPIS | DPPX Ab IFA, S | No | Yes |
| GABCS | GABA-B-R Ab CBA, S | No | Yes |
| GD65S | GAD65 Ab Assay, S | Yes | Yes |
| GFAIS | GFAP IFA, S | No | Yes |
| IG5IS | IgLON5 IFA, S | No | Yes |
| LG1CS | LGI1-IgG CBA, S | No | Yes |
| GL1IS | mGluR1 Ab IFA, S | No | Yes |
| NIFIS | NIF IFA, S | No | Yes |
| NMDCS | NMDA-R Ab CBA, S | No | Yes |
| PCABP | Purkinje Cell Cytoplasmic Ab Type 1 | No | Yes |
| PCAB2 | Purkinje Cell Cytoplasmic Ab Type 2 | No | Yes |
| PCATR | Purkinje Cell Cytoplasmic Ab Type Tr | No | Yes |
Reflex Tests
Lists tests that may or may not be performed, at an additional charge, depending on the result and interpretation of the initial tests.
Lists tests that may or may not be performed, at an additional charge, depending on the result and interpretation of the initial tests.
| Test Id | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| ARBI | ACh Receptor (Muscle) Binding Ab | Yes | No |
| AGNBS | AGNA-1 Immunoblot, S | No | No |
| AINCS | Alpha Internexin CBA, S | No | No |
| AMPIS | AMPA-R Ab IF Titer Assay, S | No | No |
| AMIBS | Amphiphysin Immunoblot, S | No | No |
| AN1BS | ANNA-1 Immunoblot, S | No | No |
| AN2BS | ANNA-2 Immunoblot, S | No | No |
| CRMWS | CRMP-5-IgG Western Blot, S | Yes | No |
| DPPCS | DPPX Ab CBA, S | No | No |
| DPPTS | DPPX Ab IFA Titer, S | No | No |
| GABIS | GABA-B-R Ab IF Titer Assay, S | No | No |
| GFACS | GFAP CBA, S | No | No |
| GFATS | GFAP IFA Titer, S | No | No |
| IG5CS | IgLON5 CBA, S | No | No |
| IG5TS | IgLON5 IFA Titer, S | No | No |
| GL1CS | mGluR1 Ab CBA, S | No | No |
| GL1TS | mGluR1 Ab IFA Titer, S | No | No |
| NFHCS | NIF Heavy Chain CBA, S | No | No |
| NIFTS | NIF IFA Titer, S | No | No |
| NFLCS | NIF Light Chain CBA, S | No | No |
| NMDIS | NMDA-R Ab IF Titer Assay, S | No | No |
| PC1BS | PCA-1 Immunoblot, S | No | No |
| PCTBS | PCA-Tr Immunoblot, S | No | No |
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If client requests or if immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are 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, then AGNA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests antineuronal nuclear antibody (ANNA)-1, 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, 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 and 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 are performed at an additional charge.
If AMPA-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and acetylcholine (Ach) receptor (muscle) binding antibody are performed at an additional charge.
If contactin-associated protein-like-2 (CASPR2)-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are performed at an additional charge.
If IFA pattern suggests gamma-aminobutyric acid B (GABA-B)-receptor antibody, and GABA-B-receptor antibody 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.
See Encephalopathy Autoimmune Evaluation Algorithm-Serum in Special Instructions.
Special Instructions
Library of PDFs including pertinent information and forms related to the test
Library of PDFs including pertinent information and forms related to the test
Method Name
A short description of the method used to perform the test
A short description of the method used to perform the test
AGN1S, AMPHS, AMPIS, ANN1S, ANN2S, ANN3S, CRMS, DPPIS, DPPTS, GABIS, GFAIS, GFATS, GL1IS, GL1TS, IG5IS, IG5TS, NIFIS, NIFTS, NMDIS, PCAB2, PCABP, PCATR: Indirect Immunofluorescence Assay (IFA)
AINCS, AMPCS, CS2CS, DPPCS, GABCS, GFACS, GL1CS, IG5CS, LG1CS, NFHCS, NFLCS, NMDCS: Cell-Binding Assay (CBA)
CRMWS: Western Blot (WB)
AGNBS, AMIBS, AN1BS, AN2BS, PC1BS, PCTBS: Immunoblot (IB)
ARBI, GD65S: Radioimmunoassay (RIA)
NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Yes
Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test
Lists a shorter or abbreviated version of the Published Name for a test
Encephalopathy-Autoimmune Eval, S
Aliases
Lists additional common names for a test, as an aid in searching
Lists additional common names for a test, as an aid in searching
ACh Receptor (Muscle) Binding Ab
AMPA-R Ab CBA
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
Behavioral change
CASPR2-IgG
Confusion
Contactin-Associated Protein-Like-2 (CASPR2)-IgG
CRMP-5-IgG
Encephalitis
GABA-B-R Ab CBA
Glutamic Acid Decarboxylase (GAD65)
Leucine-Rich Glioma Inactived Protein-1 IgG
LGI1-IgG
Limbic encephalitis
NMDA-R Ab CBA
Psychosis
Purkinje Cell Cytoplasmc Ab Type Tr
Purkinje Cell Cytoplasmic Ab Type 1
Purkinje Cell Cytoplasmic Ab Type 2
GFAP
IGLON5
NIF
ENCES_
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If client requests or if immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are 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, then AGNA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests antineuronal nuclear antibody (ANNA)-1, 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, 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 and 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 are performed at an additional charge.
If AMPA-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and acetylcholine (Ach) receptor (muscle) binding antibody are performed at an additional charge.
If contactin-associated protein-like-2 (CASPR2)-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are performed at an additional charge.
If IFA pattern suggests gamma-aminobutyric acid B (GABA-B)-receptor antibody, and GABA-B-receptor antibody 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.
See Encephalopathy Autoimmune Evaluation Algorithm-Serum in Special Instructions.
Specimen Type
Describes the specimen type validated for testing
Describes the specimen type validated for testing
Serum
Necessary Information
Provide the following information:
-Relevant clinical information
-Ordering provider name, phone number, mailing address, and e-mail address
Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing
Defines the optimal specimen required to perform the test and the preferred volume to complete testing
Patient Preparation:
1. For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication or intravenous immunoglobulin treatment.
2. This test should not be requested in patients who have recently received radioisotopes, therapeutically or diagnostically, because of potential assay interference. The specific waiting period before specimen collection will depend on the isotope administered, the dose given, and the clearance rate in the individual patient. Specimens will be screened for radioactivity prior to analysis. Radioactive specimens received in the laboratory will be held 1 week and assayed if sufficiently decayed, or canceled if radioactivity remains.
3. Patient should have no general anesthetic or muscle-relaxant drugs in the previous 24 hours.
Container/Tube:
Preferred: Red top
Acceptable: Serum gel
Specimen Volume: 4 mL
Special Instructions
Library of PDFs including pertinent information and forms related to the test
Library of PDFs including pertinent information and forms related to the test
Forms
-General Request (T239)
Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory
2.5 mL
Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected
Identifies specimen types and conditions that may cause the specimen to be rejected
| Gross hemolysis | Reject |
| Gross lipemia | Reject |
| Gross icterus | Reject |
Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 28 days | |
| Frozen | 28 days | ||
| Ambient | 72 hours |
Useful For
Suggests clinical disorders or settings where the test may be helpful
Suggests clinical disorders or settings where the test may be helpful
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 serum 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 cerebral spinal 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
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If client requests or if immunofluorescence (IFA) patterns suggest collapsin response-mediator protein-5-IgG (CRMP-5-IgG), then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are 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, then AGNA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests antineuronal nuclear antibody (ANNA)-1, 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, 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 and 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 are performed at an additional charge.
If AMPA-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and acetylcholine (Ach) receptor (muscle) binding antibody are performed at an additional charge.
If contactin-associated protein-like-2 (CASPR2)-receptor antibody CBA is positive, then CRMP-5-IgG Western blot, and ACh receptor (muscle) binding antibody are performed at an additional charge.
If IFA pattern suggests gamma-aminobutyric acid B (GABA-B)-receptor antibody, and GABA-B-receptor antibody 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.
See Encephalopathy Autoimmune Evaluation Algorithm-Serum in Special Instructions.
Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Autoimmune encephalopathies extend beyond the classically recognized clinical and radiological spectrum of "limbic encephalitis." They encompass a diversity of neurological presentations with subacute or insidious onset, including confusional states, psychosis, delirium, memory loss, hallucinations, movement disorders, sensory or motor complaints, seizures, dyssomnias, ataxias, eye movement problems, nausea, vomiting, inappropriate antidiuresis, coma, dysautonomias, or hypoventilation. A diagnosis of autoimmune encephalopathy should be suspected on the basis of clinical course, coexisting autoimmune disorder (eg, thyroiditis, diabetes), serological evidence of autoimmunity, spinal fluid evidence of intrathecal inflammation, neuroimaging or electroencephalographic abnormalities, and favorable response to trial of immunotherapy.
Detection of one or more neural autoantibodies aids the diagnosis of autoimmune encephalopathy and may guide a search for cancer. Pertinent autoantibody specificities include: 1) neurotransmitter receptors and ion channels such as neuronal voltage-gated potassium channels (and interacting synaptic and axonal proteins, leucine-rich glioma inactivated protein: LGI1 and contactin associated protein 2: CASPR2), ionotropic glutamate receptors (N-methyl-D-aspartate receptor: NMDA and 2-amino-3-[5-methyl-3-oxo-1,2- oxazol-4-yl] propanoic acid: AMPA), metabotropic gamma-aminobutyric acid (GABA)-B receptors; 2) enzymes, signaling molecules and RNA-regulatory proteins in the cytoplasm and nucleus of neurons (glutamic acid decarboxylase 65: GAD65, collapsin response-mediator protein-5 neuronal: CRMP-5, antineuronal nuclear antibody-type 1: ANNA-1, and ANNA-2).
Importantly, autoimmune encephalopathies are reversible. Misdiagnosis as a progressive (currently irreversible) neurodegenerative condition is not uncommon and has devastating consequences for the patient. Clinicians must consider the possibility of an autoimmune etiology in the differential diagnoses of encephalopathy. For example, a potentially reversible disorder justifies a trial of immunotherapy for the detection of neural autoantibodies in patients presenting with symptoms of personality change, executive dysfunction, and psychiatric manifestations.
A triad of clues helps to identify patients with an autoimmune encephalopathy: 1) clinical presentation (subacute symptoms, onset rapidly progressive course, and fluctuating symptoms) and radiological findings consistent with inflammation, 2) detection of neural autoantibodies in serum or cerebrospinal fluid (CSF), and 3) favorable response to a trial of immunotherapy.
Detection of neural autoantibodies in serum or CSF informs the physician of a likely autoimmune etiology, and may heighten suspicion for a paraneoplastic basis and guide the search for cancer. Neurological accompaniments of neural autoantibodies are generally not syndromic, but diverse and multifocal. For example, LGI1 antibody was initially considered to be specific for autoimmune limbic encephalitis, but over time other presentations have been reported, including rapidly progressive course of cognitive decline mimicking neurodegenerative dementia. Comprehensive antibody testing is more informative than selective testing for 1 or 2 neural antibodies. Some antibodies strongly predict an underlying cancer. For example; small-cell lung carcinoma (ANNA-1, CRMP-5-IgG), ovarian teratoma (NMDA-R), and thymoma (CRMP-5 IgG).
An individual patient's profile autoantibody may be informative for a specific cancer type. For example, in a patient presenting with encephalitis who has CRMP 5 IgG, and subsequent reflex reveals muscle acetylcholine receptor (AChR) binding antibody, the findings should raise a high suspicion for thymoma. Testing of CSF for autoantibodies is particularly helpful when serum testing is negative, though in some circumstances testing both serum and CSF simultaneously is pertinent. Testing of CSF is recommended for some antibodies in particular (such as NMDA-R antibody and GFAP-IgG) because CSF testing is both more sensitive and specific. In contrast, serum testing for LGI1 antibody is more sensitive than CSF testing.
Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
| Test ID | Reporting name | Methodology | Reference value |
| AMPCS | AMPA-R Ab CBA, S | Cell-binding assay (CBA) | Negative |
| AMPHS | Amphiphysin Ab, S | Indirect Immunofluorescence Assay (IFA) | <1:240 |
| AGN1S | Anti-Glial Nuclear Ab, Type 1 | IFA | <1:240 |
| ANN1S | Anti-Neuronal Nuclear Ab, Type 1 | IFA | <1:240 |
| ANN2S | Anti-Neuronal Nuclear Ab, Type 2 | IFA | <1:240 |
| ANN3S | Anti-Neuronal Nuclear Ab, Type 3 | IFA | <1:240 |
| CS2CS | CASPR2-IgG CBA, S | CBA | Negative |
| CRMS | CRMP-5-IgG, S | IFA | <1:240 |
| DPPIS | DPPX Ab IFA, S | IFA | Negative |
| GABCS | GABA-B-R Ab CBA, S | CBA | Negative |
| GD65S | GAD65 Ab Assay, S | Radioimmunoassay (RIA) | < or =0.02 nmol/L Reference values apply to all ages. |
| GFAIS | GFAP IFA, S | IFA | Negative |
| IG5IS | IgLON5 IFA, S | IFA | Negative |
| LG1CS | LGI1-IgG CBA, S | CBA | Negative |
| GL1IS | mGluR1 Ab IFA, S | IFA | Negative |
| NIFIS | NIF IFA, S | IFA | Negative |
| NMDCS | NMDA-R Ab CBA, S | CBA | Negative |
| PCABP | Purkinje Cell Cytoplasmic Ab Type 1 | IFA | <1:240 |
| PCAB2 | Purkinje Cell Cytoplasmic Ab Type 2 | IFA | <1:240 |
| PCATR | Purkinje Cell Cytoplasmic Ab Type Tr | IFA | <1:240 |
Reflex Information:
| Test ID | Reporting name | Methodology | Reference value |
| ARBI | ACh Receptor (Muscle) Binding Ab | RIA | < or =0.02 nmol/L |
| AGNBS | AGNA-1 Immunoblot, S | Immunoblot (IB) | Negative |
| AINCS | Alpha Internexin CBA, S | CBA | Negative |
| AMPIS | AMPA-R Ab IF Titer Assay, S | IFA | <1:120 |
| AMIBS | Amphiphysin Immunoblot, S | IB | Negative |
| AN1BS | ANNA-1 Immunoblot, S | IB | Negative |
| AN2BS | ANNA-2 Immunoblot, S | IB | Negative |
| CRMWS | CRMP-5-IgG Western Blot, S | Western blot (WB) | Negative |
| DPPCS | DPPX Ab CBA, S | CBA | Negative |
| DPPTS | DPPX Ab IFA Titer, S | IFA | <1:240 |
| GABIS | GABA-B-R Ab IF Titer Assay, S | IFA | <1:120 |
| GFACS | GFAP CBA, S | CBA | Negative |
| GFATS | GFAP IFA Titer, S | IFA | <1:240 |
| IG5CS | IgLON5 CBA, S | CBA | Negative |
| IG5TS | IgLON5 IFA Titer, S | IFA | <1:240 |
| GL1CS | mGluR1 Ab CBA, S | CBA | Negative |
| GL1TS | mGluR1 Ab IFA Titer, S | IFA | <1:240 |
| NFHCS | NIF Heavy Chain CBA, S | CBA | Negative |
| NIFTS | NIF IFA Titer, S | IFA | <1:240 |
| NFLCS | NIF Light Chain CBA, S | CBA | Negative |
| NMDIS | NMDA-R Ab IF Titer Assay, S | IFA | <1:120 |
| PC1BS | PCA-1 Immunoblot, S | IB | Negative |
| PCTBS | PCA-Tr Immunoblot, S | IB | Negative |
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."
Note: 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
Provides information to assist in interpretation of the test results
Provides information to assist in interpretation of the test results
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
Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Negative results do not exclude autoimmune encephalopathy or cancer.
This test does not detect Ma1 or Ma2 antibodies (alias 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.
Intravenous immunoglobulin (IVIg) treatment prior to the serum collection may cause a false-positive result.
Clinical Reference
Recommendations for in-depth reading of a clinical nature
Recommendations for in-depth reading of a clinical nature
1. McKeon A, Lennon, VA, Pittock, SJ: Immunotherapy responsive dementias and encephalopathies. Continuum (Minneap Minn). 2010 Apr;16(2):80-101
2. Lucchinetti CF, Kimmel DW, Lennon VA: Paraneoplastic and oncological profiles of patients seropositive for type 1 anti-neuronal nuclear autoantibodies. Neurology. 1998;50:652-657
3. Pittock SJ, Yoshikawa H, Ahlskog JE, et al: Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal and spinal cord dysfunction. Mayo Clin Proc. 2006 Sep;81(9):1207-1214
4. Lancaster E, Martinez-Hernandez E, Dalmau J: Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011 Jul 12;77(2):179-189
5. Klein CJ, Lennon VA, Aston PA, et al: Insights from LGI1 and CASPR2 potassium channel complex autoantibody subtyping. JAMA Neurol. 2013 Feb;70(2):229-234
Special Instructions
Library of PDFs including pertinent information and forms related to the test
Library of PDFs including pertinent information and forms related to the test
Method Description
Describes how the test is performed and provides a method-specific reference
Describes how the test is performed and provides a method-specific reference
Indirect Immunofluorescence Assay:
The patient's sample is tested by a standardized indirect immunofluorescence assay (IFA) that uses a composite frozen section of mouse cerebellum, kidney, and gut tissues. After incubation with sample and washing, fluorescein-conjugated goat-antihuman IgG is applied. Neuron-specific autoantibodies are identified by their characteristic fluorescence staining patterns. Samples that are scored positive for any neuronal nuclear or cytoplasmic autoantibody are titrated to an endpoint. Interference by coexisting non-neuron-specific autoantibodies can usually be eliminated by serologic absorption.(Honorat JA, Komorowski L, Josephs KA, et al: IgLON5 antibody: Neurological accompaniments and outcomes in 20 patients. Neruol Neruoimmunol Neruoinflamm. 2017 Jul 18;4(5):e385. doi: 10.1212/NXI.0000000000000385)
Western Blot:
Neuronal antigens extracted aqueously from adult rat cerebellum, full-length recombinant human collapsin response-mediator protein-5 (CRMP-5), or full-length recombinant human amphiphysin protein is denatured, reduced, and separated by electrophoresis on 10% polyacrylamide gel. IgG is detected autoradiographically by enhanced chemiluminescence.(Yu Z, Kryzer TJ, Griesmann GE, et al: CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol. 2001 February;49[2]:146-154; Dubey D, Jitprapaikulsan J, Bi H, et al: Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome. Neurology. 2019 Nov 12;93(20):e1873-e1880. doi: 10.1212/WNL.0000000000008472)
Immunoblot:
All steps are performed at room temperature (18 to 28 degrees C) utilizing the EUROBlot One instrument. Diluted patient serum (1:101) is added to test strips (strips containing recombinant antigen manufactured and purified using biochemical methods) in individual channels and incubated for 30 minutes. Positive specimens will bind to the purified recombinant antigen and negative specimens will not bind. Strips are washed to remove unbound serum antibodies and then incubated with anti-human IgG antibodies (alkaline phosphatase-labelled) for 30 minutes. The strips are again washed to remove unbound anti-human IgG antibodies and nitroblue tetrazolium chloride/5-bromo-4-chloro-3-indolylphosphate (NBT/BCIP) substrate is added. Alkaline phosphatase enzyme converts the soluble substrate into a colored insoluble product on the membrane to produces a black band. Strips are digitized via picture capture on the EUROBlot One instrument and evaluated with the EUROLineScan software.(O'Connor K, Waters P, Komorowski L, et al: GABAA receptor autoimmunity: A multicenter experience. Neurol Neuroimmunol Neuroinflamm. 2019 Apr 4;6[3]:e552. doi: 10.1212/NXI.0000000000000552)
Cell-Binding Assay:
Patient specimen is applied to a composite slide containing transfected and nontransfected HEK-293 cells. After incubation and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the presence of patient IgG binding.(Package insert: IIFT: Neurology Mosaics, Instructions for the indirect immunofluorescence test. EUROIMMUN; FA_112d-1_A_UK_C13; 02/2019)
Radioimmunoassay:
Duplicate aliquots of patient specimen are incubated with I(125)-labeled antigen. Immune complexes, formed by adding secondary (goat)-antihuman immunoglobulin, are pelleted by centrifugation and washed. Gamma emission from the washed pellet is counted, and mean counts per minute (cpm) are compared with results yielded by high-positive and -negative control sera. Specimen yielding cpm higher than the background cpm yielded by normal human specimen are retested to confirm positivity and titrated as necessary to obtain a value in the linear range of the assay. The antigen binding capacity (nmol per liter) is calculated from the cpm precipitated at a dilution yielding a linear range value.(Griesmann GE, Kryzer TJ, Lennon VA: Autoantibody profiles of myasthenia gravis and Lambert-Eaton myasthenic syndrome. In: Rose NR, Hamilton RG, et al, eds. Manual of Clinical and Laboratory Immunology. 6th ed. Washington, DC, ASM Press; 2002:1005-1012; Jones AL, Flanagan EP, Pittock SJ, et al: Responses to and Outcomes of Treatment of Autoimmune Cerebellar Ataxia in Adults. JAMA Neurol. 2015 Nov;72[11]:1304-1312. doi: 10.1001/jamaneurol.2015.2378)
PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information
Indicates whether the report includes an additional document with charts, images or other enriched information
No
Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.
AGN1S, AMPHS, AMPIS, ANN1S, ANN2S, ANN3S, ARBI, CRMS, DPPIS, DPPTS, GABIS, GD65S, GFAIS, GFATS, GL1IS, GL1TS, IG5IS, IG5TS, NIFIS, NIFTS, NMDIS, PCAB2, PCABP, PCATR:
Monday through Sunday
AMPCS, CS2CS, DDPCS, GABCS, LG1CS, NMDCS:
Monday through Friday, Sunday
GL1CS, IG5CS:
Monday, Thursday
AINCS, NFHCS, NFLCS:
Tuesday, Thursday
GFACS:
Monday, Wednesday, Friday
CRMWS:
Monday through Thursday
AGNBS, AMIBS, AN1BS, AN2BS, PC1BS, PCTBS:
Monday through Friday
Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.
10 to 13 days
Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
28 days
Performing Laboratory Location
Indicates the location of the laboratory that performs the test
Indicates the location of the laboratory that performs the test
Rochester
Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.
- Authorized users can sign in to Test Prices for detailed fee information.
- Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
- Prospective clients should contact their Regional Manager. For assistance, contact Customer Service.
Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.
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
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
CPT codes are provided by the performing laboratory.
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
CPT codes are provided by the performing laboratory.
CPT codes are provided by the performing laboratory.
86255 x 19
86341 x 1
83519-ARBI (if appropriate)
84182-AGNBS (if appropriate)
86255-AINCS (if appropriate)
86256-AMPIS (if appropriate)
84182-AMIBS (if appropriate)
84182-AN1BS (if appropriate)
84182-AN2BS (if appropriate)
84182-CRMWS (if appropriate)
86255-DPPCS (if appropriate)
86256-DPPTS (if appropriate)
86256-GABIS (if appropriate)
86255-GFACS (if appropriate)
86256-GFATS (if appropriate)
86255-IG5CS (if appropriate)
86256-IG5TS (if appropriate)
86255-GL1CS (if appropriate)
86256-GL1TS (if appropriate)
86255-NFHCS (if appropriate)
86256-NIFTS (if appropriate)
86255-NFLCS (if appropriate)
86256-NMDIS (if appropriate)
84182-PC1BS (if appropriate)
84182-PCTBS (if appropriate)
LOINC® Information
| Test Id | Test Order Name | Order LOINC Value |
|---|---|---|
| ENS2 | Encephalopathy-Autoimmune Eval, S | 94697-0 |
| Result Id | Test Result Name |
Result LOINC Value
Result LOINC Value Tooltip
|
|---|---|---|
Test Setup Resources
Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.
Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.
Normal and Abnormal sample reports are provided as references for report appearance.
SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.