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Test Code DYS2 Dysautonomia, Autoimmune/Paraneoplastic Evaluation, Serum


Ordering Guidance


Multiple neurological phenotype-specific autoimmune/paraneoplastic evaluations are available. For more information as well as phenotype-specific testing options, refer to 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 should not be requested for 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 for 1 week and assayed if sufficiently decayed or canceled if radioactivity remains.



Necessary Information


Provide the following information:

-Relevant clinical information

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



Specimen Required


Patient Preparation: For optimal antibody detection, collect specimen before starting immunosuppressant medication or intravenous immunoglobulin treatment.

Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 4 mL Serum

Collection Instructions: Centrifuge and aliquot serum into a plastic vial.


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)

Profile Information

Test ID Reporting Name Available Separately Always Performed
ADEI Dysautonomia, Interpretation, S No Yes
AAGFS AChR Alpha3 Ganglionic Flow, S No Yes
ANN1S Anti-Neuronal Nuclear Ab, Type 1 No Yes
APBIS AP3B2 IFA, S No Yes
CRMS CRMP-5-IgG, S No Yes
CS2CS CASPR2-IgG CBA, S No Yes
DPPCS DPPX Ab CBA, S No Yes
LG1CS LGI1-IgG CBA, S No Yes
PCAB2 Purkinje Cell Cytoplasmic Ab Type 2 No Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AN1BS ANNA-1 Immunoblot, S No No
AN2BS ANNA-2 Immunoblot, S No No
AN1TS ANNA-1 Titer, S No No
APBCS AP3B2 CBA, S No No
AAGTS AChR Alpha3 Gang Flow Titer, S No No
CRMTS CRMP-5-IgG Titer, S No No
DPPTS DPPX Ab IFA Titer, S No No
MP1BS MAP1B Immunoblot, S No No
PC2TS PCA-2 Titer, S No No
CRMWS CRMP-5-IgG Western Blot, S No No
APBTS AP3B2 IFA Titer, S No No

Method Name

ANN1S, APBIS, CRMS, PCAB2, AN1TS, APBTS, CRMTS, DPPTS, PC2TS: Indirect Immunofluorescence Assay (IFA)

CS2CS, DPPCS, LG1CS, APBCS: Cell Binding Assay (CBA)

CRMWS: Western Blot (WB)

AN1BS, AN2BS, MP1BS: Immunoblot (IB)

AAGFS, AAGTS: Flow Cytometry

ADEI: Technical Interpretation

Reporting Name

Dysautonomia, Autoimm/Paraneo, S

Specimen Type

Serum

Specimen Minimum Volume

Serum: 2.5 mL

Specimen Stability Information

Specimen Type Temperature Time
Serum Refrigerated (preferred) 28 days
  Frozen  28 days
  Ambient  72 hours

Reject Due To

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus Reject

Clinical Information

Autoimmune dysautonomia encompasses disorders of peripheral autonomic synapses, ganglionic neurons, autonomic nerve fibers, and central autonomic pathways mediated by neural-specific IgG or effector T cells. These disorders may be idiopathic or paraneoplastic, subacute or insidious in onset, and may present as a limited disorder or generalized pandysautonomia. Pandysautonomia is usually subacute in onset and severity and includes impaired pupillary light reflex, anhidrosis, orthostatic hypotension, cardiac arrhythmias, gastrointestinal dysmotility, sicca manifestations, and bladder dysfunction. Limited dysautonomia is confined to one or just a few domains, is often mild, and may include sicca manifestations, postural orthostatism and cardiac arrhythmias, bladder dysfunction, or gastrointestinal dysmotilities. Diagnosis of limited dysautonomia requires documentation of objective abnormalities by autonomic reflex testing, thermoregulatory sweat test, or gastrointestinal motility studies.

 

The most frequently encountered autoantibody marker of autoimmune dysautonomia is the neuronal ganglionic alpha-3-acetylcholine receptor (AChR) autoantibody. This autoantibody to date is the only proven effector of autoimmune dysautonomia.

 

Importantly, cancer is detected in 30% of patients with alpha-3-AChR autoantibody. Cancers recognized include small-cell lung carcinomas, thymoma, lymphoma, and adenocarcinomas of breast, lung, prostate, and gastrointestinal tract. Cancer risk factors include a previous or family history of cancer, history of smoking, or social or environmental exposure to carcinogens. Early diagnosis and treatment of the neoplasm favor neurologic improvement and lessen morbidity.

 

Autoantibodies to other onconeural proteins shared by neurons, glia, or muscle (eg, antineuronal nuclear antibody-type 1 [ANNA-1], collapsin response-mediator protein-5 neuronal [CRMP-5-IgG]) serve as additional markers of paraneoplastic or idiopathic dysautonomia. A specific neoplasm is often predictable by the individual patient's autoantibody profile.

Reference Values

Test ID

Reporting name

Methodology*

Reference value

ADEI

Dysautonomia, Interpretation, S

Technical interpretation

Interpretive report

AAGFS

AChR Alpha3 Ganglionic Flow, S

FC

Negative

ANN1S

Anti-Neuronal Nuclear Ab, Type 1

IFA

Negative

APBIS

AP3B2 IFA, S

IFA

Negative

CS2CS

CASPR2-IgG CBA, S

CBA

Negative

CRMS

CRMP-5-IgG, S

IFA

Negative

DPPCS

DPPX Ab CBA, S

CBA

Negative

LG1CS

LGI1-IgG CBA, S

CBA

Negative

PCAB2

Purkinje Cell Cytoplasmic Ab Type 2

IFA

Negative

 Reflex Information:

Test ID

Reporting name

Methodology*

Reference value

AAGTS

AChR Alpha3 Gang Flow Titer, S

FC

<1:10

AN1BS

ANNA-1 Immunoblot, S

IB

Negative

AN1TS

ANNA-1 Titer, S

IFA

<1:240

AN2BS

ANNA-2 Immunoblot, S

IB

Negative

APBCS

AP3B2 CBA, S

CBA

Negative

APBTS

AP3B2 IFA Titer, S

IFA

<1:240

CRMTS

CRMP-5-IgG Titer, S

IFA

<1:240

CRMWS

CRMP-5-IgG Western Blot, S

WB

Negative

DPPTS

DPPX Ab IFA Titer, S

IFA

<1:240

MP1BS

MAP1B Immunoblot, S

IB

Negative

PC2TS

PCA-2 Titer, S

IFA

<1:240

 

*Methodology abbreviations:

Cell-binding assay (CBA)

Flow cytometry (FC)

Immunoblot (IB)

Immunofluorescence assay (IFA)

Western Blot (WB)

Report Available

8 to 12 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. It has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

86255 x 8

86256 AAGTS (if appropriate)

84182 AN1BS (if appropriate)

86256 AN1TS (if appropriate)

84182 AN2BS (if appropriate)

86255 APBCS (if appropriate)

86256 APBTS (if appropriate)

86256 CRMTS (if appropriate)

84182 CRMWS (if appropriate)

86256 DPPTS (if appropriate)

84182 MP1BS (if appropriate)

86256 PC2TS (if appropriate)