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26149 Autoimmune Dysautonomia Evaluation (DYS2)

Autoimmune Dysautonomia Evaluation (DYS2)
Test Code: DYS2SO
Synonyms/Keywords
​ANNA (Antineuronal Nuclear Antibody)
Anti-CV2
Anti-Enteric Neuronal Antibody
Anti-Hu
Antineuronal
Cantoxin (Receptor Antibodies)
Cerebellar Antibodies
Chorea
Collapsin Response-Mediator Protein-5 Antibody (CRMP-5), Serum
Cramp-fasciculation
CRMP-5, IgG
Dorsal Root Ganglion Antibody
DPPX
dipeptidyl aminopeptidase-like protein 6
Hu Antibody
Isaacs disease
Motor End-Plate Antibody
Motor Nerve Terminal Antibodies
Myoid Antibody
Neuromuscular hyperexcitability
Neuromyotonia
Neuronal ganglionic acetylcholine receptor antibody
Neuronal Nuclear Antibody Panel
Neuronal Potassium Channel Ab
Neuronal-Anti
Paraneoplastic Antibodies
Paraneoplastic Autoantibody Evaluation
Paraneoplastic Neurological Autoimmunity
Stiff-man Syndrome
Potassium Channel Antibodies
Test Components

Testing Algorithm:

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

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

If IFA pattern suggests antineuronal nuclear antibodies (ANNA)-1 antibody, then ANNA-1 and 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 PCA type 1or type trace, then the appropriate antibody specific IFA is performed at an additional charge.

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

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

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

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

Useful For

Investigating idiopathic dysautonomic symptoms

Directing a focused search for cancer in patients with idiopathic dysautonomia

Investigating autonomic symptoms that appear in the course or wake of cancer therapy and are not explainable by recurrent cancer or metastasis (detection of autoantibodies in this profile helps differentiate autoimmune dysautonomia from the effects of chemotherapy)

Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
N​o​ ​Serum Red Top Tube (RTT) ​Serum Separator Tube (SST) ​4 mL ​2.5 mL
Collection Processing Instructions

Patient Preparation:

1. For optimal antibody detection, specimen collection is recommended before 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 medications in the previous 24 hours.

Necessary Information: 

Provide the following information:

-Relevant clinical information

-Ordering provider name, phone number, mailing address, and e-mail address

Specimen Stability Information
Specimen Type Temperature Time
​Serum ​ ​ ​Refrigerated (preferred) ​28 days
​Ambient ​72 hours
​Frozen ​28 days
Rejection Criteria
Gross Hemolysis
​Gross Icterus
​Gross Lipemia
Interference

Negative results do not exclude autoimmune dysautonomia or cancer.

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

Performing Laboratory Information
Performing Location Day(s) Test Performed Report Available Methodology/Instrumentation
​Mayo Clinic Laboratories ​Monday through Sunday ​7 to 10 days Indirect lmmunofluorescence (IFA), Cell Binding Assay (CBA), Western Blot (WB), Immunoblot (IB), Radioimmunoassay (RIA)
Reference Lab
Reference Range Information
Test IDReporting nameMethodologyReference value
ADEIDysautonomia, Interpretation, SInterpretationNA
GANGAChR Ganglionic Neuronal Ab, SRadioimmunoassay (RIA)< or =0.02 nmol/L
ANN1SAnti-Neuronal Nuclear Ab, Type 1Indirect immunofluorescence (IFA)<1:240*
CS2CSCASPR2-IgG CBA, SCell-Binding Assay (CBA)Negative
CRMSCRMP-5-IgG, SIFA<1:240*
DPPISDPPX Ab IFA, SIFANegative
LG1CSLGI1-IgG CBA, SCBANegative
PCAB2Purkinje Cell Cytoplasmic Ab Type 2IFA<1:240*

Reflex Information:

Test IDReporting nameMethodologyReference value
AMPCSAMPA-R Ab CBA, SCBANegative
AMPISAMPA-R Ab IF Titer Assay, SIFA<1:120
AMPHSAmphiphysin Ab, SIFA<1:240
AMIBSAmphiphysin Immunoblot, SImmunoblot (IB)Negative
AN1BSANNA-1 Immunoblot, SIBNegative
AN2BSANNA-2 Immunoblot, SIBNegative
CRMWSCRMP-5-IgG Western Blot, SWestern Blot (WB)Negative
DPPCSDPPX Ab CBA, SCBANegative
DPPTSDPPX Ab IFA Titer, SIFA<1:120
GABCSGABA-B-R Ab CBA, SCBANegative
GABISGABA-B-R Ab IF Titer Assay, SIFA<1:120
NMDCSNMDA-R Ab CBA, SCBANegative
NMDISNMDA-R Ab IF Titer Assay, SIFA<1:120
PC1BSPCA-1 Immunoblot, SIBNegative
PCTBSPCA-Tr Immunoblot, SIBNegative
PCABPPurkinje Cell Cytoplasmic Ab Type 1IFA<1:240*
PCATRPurkinje Cell Cytoplasmic Ab Type TrIFA<1:240*

 

*Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, 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 800-533-1710 to request CRMP-5 Western blot.

Interpretation

Antibodies directed at onconeural proteins shared by neurons, muscle, and glia are valuable serological markers of a patient's immune response to cancer. These autoantibodies are not found in healthy subjects and are usually accompanied by subacute neurological symptoms and signs. It is not uncommon for more than one autoantibody to be detected in patients with autoimmune dysautonomia. These include:

-Plasma membrane cation channel antibodies (neuronal ganglionic [alpha-3]). All of these autoantibodies are potential effectors of autonomic dysfunction.

-Antineuronal nuclear autoantibody-type 1

-Neuronal and muscle cytoplasmic antibodies (CRMP-5 IgG)

A rising autoantibody titer in previously seropositive patients suggests cancer recurrence.

Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​83519 1
​86255 ​6
​86255 ​7 ​If needed
​86256 ​4 ​If needed
​84182 ​6 ​If needed
Synonyms/Keywords
​ANNA (Antineuronal Nuclear Antibody)
Anti-CV2
Anti-Enteric Neuronal Antibody
Anti-Hu
Antineuronal
Cantoxin (Receptor Antibodies)
Cerebellar Antibodies
Chorea
Collapsin Response-Mediator Protein-5 Antibody (CRMP-5), Serum
Cramp-fasciculation
CRMP-5, IgG
Dorsal Root Ganglion Antibody
DPPX
dipeptidyl aminopeptidase-like protein 6
Hu Antibody
Isaacs disease
Motor End-Plate Antibody
Motor Nerve Terminal Antibodies
Myoid Antibody
Neuromuscular hyperexcitability
Neuromyotonia
Neuronal ganglionic acetylcholine receptor antibody
Neuronal Nuclear Antibody Panel
Neuronal Potassium Channel Ab
Neuronal-Anti
Paraneoplastic Antibodies
Paraneoplastic Autoantibody Evaluation
Paraneoplastic Neurological Autoimmunity
Stiff-man Syndrome
Potassium Channel Antibodies
Test Components

Testing Algorithm:

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

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

If IFA pattern suggests antineuronal nuclear antibodies (ANNA)-1 antibody, then ANNA-1 and 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 PCA type 1or type trace, then the appropriate antibody specific IFA is performed at an additional charge.

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

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

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

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

Ordering Applications
Ordering Application Description
​Cerner ​Autoimmune Dysautonomia Evaluation (DYS2)
​Clinical Order Manager ​Autoimmune Dysautonomia Evaluation (DYS2)
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
N​o​ ​Serum Red Top Tube (RTT) ​Serum Separator Tube (SST) ​4 mL ​2.5 mL
Collection Processing

Patient Preparation:

1. For optimal antibody detection, specimen collection is recommended before 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 medications in the previous 24 hours.

Necessary Information: 

Provide the following information:

-Relevant clinical information

-Ordering provider name, phone number, mailing address, and e-mail address

Specimen Stability Information
Specimen Type Temperature Time
​Serum ​ ​ ​Refrigerated (preferred) ​28 days
​Ambient ​72 hours
​Frozen ​28 days
Rejection Criteria
Gross Hemolysis
​Gross Icterus
​Gross Lipemia
Interference

Negative results do not exclude autoimmune dysautonomia or cancer.

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

Useful For

Investigating idiopathic dysautonomic symptoms

Directing a focused search for cancer in patients with idiopathic dysautonomia

Investigating autonomic symptoms that appear in the course or wake of cancer therapy and are not explainable by recurrent cancer or metastasis (detection of autoantibodies in this profile helps differentiate autoimmune dysautonomia from the effects of chemotherapy)

Test Components

Testing Algorithm:

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

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

If IFA pattern suggests antineuronal nuclear antibodies (ANNA)-1 antibody, then ANNA-1 and 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 PCA type 1or type trace, then the appropriate antibody specific IFA is performed at an additional charge.

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

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

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

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

Reference Range Information
Test IDReporting nameMethodologyReference value
ADEIDysautonomia, Interpretation, SInterpretationNA
GANGAChR Ganglionic Neuronal Ab, SRadioimmunoassay (RIA)< or =0.02 nmol/L
ANN1SAnti-Neuronal Nuclear Ab, Type 1Indirect immunofluorescence (IFA)<1:240*
CS2CSCASPR2-IgG CBA, SCell-Binding Assay (CBA)Negative
CRMSCRMP-5-IgG, SIFA<1:240*
DPPISDPPX Ab IFA, SIFANegative
LG1CSLGI1-IgG CBA, SCBANegative
PCAB2Purkinje Cell Cytoplasmic Ab Type 2IFA<1:240*

Reflex Information:

Test IDReporting nameMethodologyReference value
AMPCSAMPA-R Ab CBA, SCBANegative
AMPISAMPA-R Ab IF Titer Assay, SIFA<1:120
AMPHSAmphiphysin Ab, SIFA<1:240
AMIBSAmphiphysin Immunoblot, SImmunoblot (IB)Negative
AN1BSANNA-1 Immunoblot, SIBNegative
AN2BSANNA-2 Immunoblot, SIBNegative
CRMWSCRMP-5-IgG Western Blot, SWestern Blot (WB)Negative
DPPCSDPPX Ab CBA, SCBANegative
DPPTSDPPX Ab IFA Titer, SIFA<1:120
GABCSGABA-B-R Ab CBA, SCBANegative
GABISGABA-B-R Ab IF Titer Assay, SIFA<1:120
NMDCSNMDA-R Ab CBA, SCBANegative
NMDISNMDA-R Ab IF Titer Assay, SIFA<1:120
PC1BSPCA-1 Immunoblot, SIBNegative
PCTBSPCA-Tr Immunoblot, SIBNegative
PCABPPurkinje Cell Cytoplasmic Ab Type 1IFA<1:240*
PCATRPurkinje Cell Cytoplasmic Ab Type TrIFA<1:240*

 

*Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, 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 800-533-1710 to request CRMP-5 Western blot.

Interpretation

Antibodies directed at onconeural proteins shared by neurons, muscle, and glia are valuable serological markers of a patient's immune response to cancer. These autoantibodies are not found in healthy subjects and are usually accompanied by subacute neurological symptoms and signs. It is not uncommon for more than one autoantibody to be detected in patients with autoimmune dysautonomia. These include:

-Plasma membrane cation channel antibodies (neuronal ganglionic [alpha-3]). All of these autoantibodies are potential effectors of autonomic dysfunction.

-Antineuronal nuclear autoantibody-type 1

-Neuronal and muscle cytoplasmic antibodies (CRMP-5 IgG)

A rising autoantibody titer in previously seropositive patients suggests cancer recurrence.

For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Report Available Methodology/Instrumentation
​Mayo Clinic Laboratories ​Monday through Sunday ​7 to 10 days Indirect lmmunofluorescence (IFA), Cell Binding Assay (CBA), Western Blot (WB), Immunoblot (IB), Radioimmunoassay (RIA)
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​83519 1
​86255 ​6
​86255 ​7 ​If needed
​86256 ​4 ​If needed
​84182 ​6 ​If needed
For most current information refer to the Marshfield Laboratory online reference manual.