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Test Code CRMWS Collapsin Response-Mediator Protein-5-IgG, Western Blot, Serum

Useful For

Evaluation of cases of chorea, vision loss, cranial neuropathy and myelopathy

Method Name

Western Blot

Reporting Name

CRMP-5-IgG Western Blot, S

Specimen Type

Serum


Ordering Guidance


It is recommended an evaluation be ordered in conjunction with this testing if not previously performed. Multiple neurological phenotype-specific autoimmune/paraneoplastic evaluations are available. For more information as well as phenotype-specific testing options, see Autoimmune Neurology Test Ordering Guide.



Additional Testing Requirements


 



Necessary Information


Provide the following information:

-Relevant clinical information

-Ordering healthcare professional name, phone number, mailing address, and e-mail address



Specimen Required


Supplies: Sarstedt Aliquot Tube 5 mL (T914)

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL

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


Specimen Minimum Volume

1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
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

Autoantibodies specific for neurons and muscle are important serological markers of neurological autoimmunity. Most are highly predictive of specific neoplasms that are metastatic when diagnosed, but usually limited in spread to regional lymph nodes and adjacent structures.(1-4)

 

Collapsin response-mediator protein-5 (CRMP-5) is highly expressed in small-cell lung carcinomas (SCLC), in neurons throughout the adult central and peripheral nervous systems, and in a subset of glial cells.(1) In Western blot analyses the native antigen is a 62-kDa protein (recombinant human CRMP-5 is 68-kDa).(1) CRMP-5-IgG (also known as anti-CV-2)(4,5) is a more common autoantibody accompaniment of SCLC than antineuronal nuclear antibodies-1 (ANNA-1; anti-Hu) and sometimes occurs with thymoma.

 

The neurological presentation of CRMP-5 seropositive patients is usually multifocal, and can affect any level of the neuraxis. Neurological presentations that suggest a CRMP-5-IgG-related syndrome include subacute chorea or cranial neuropathy (particularly loss of vision, taste, or smell), dementia, myelopathy and gastrointestinal dysmotility in a patient with risk factors for lung cancer, or encephalopathy or neuromuscular hyperexcitability in a patient with serological or clinical evidence of myasthenia gravis.(1) Fourteen percent of patients have thromboembolic phenomena. Seropositive patients who have thymoma usually present with other myasthenia gravis neurological manifestations (eg, encephalopathy, disorders of continuous muscle fiber activity).(3)

 

CRMP-5-IgG is defined in serum or spinal fluid by its characteristic immunofluorescence (IF) staining pattern on a mixed tissue substrate of adult mouse central and peripheral neurons. However, CRMP-5-IgG is not detectable by standard IF screening if the titer is low (serum <1:240; CSF <1:2) or if coexisting autoantibodies, either neuron-specific or nonorgan-specific antinuclear and antimitochondrial antibodies, preclude identification of CRMP-5-IgG with certainty. In these situations, CRMP-5-IgG may be detected by Western blot analysis.

Reference Values

Negative

Interpretation

A positive result confirms that a patient's subacute neurological disorder has an autoimmune basis, and is likely to be associated with a small-cell lung carcinoma (SCLC) or thymoma, which may be occult.(1,2) A positive result has a predictive value of 90% for neoplasm (77% SCLC, 6% thymoma).(1) Seropositivity is found in approximately 3% of patients who have SCLC with limited metastasis without evidence of neurological autoimmunity.(6)

 

Clinical-serological correlations have not yet been established for children.

 

Western blot analysis is indicated when interfering nonorgan-specific or coexisting neuron-specific autoantibodies in serum or spinal fluid preclude unambiguous detection of CRMP-5-IgG by indirect immunofluorescence assay, or when the immunofluorescence assay is negative in a patient whose neurological presentation suggests a CRMP-5-IgG-related syndrome.

Cautions

Seronegativity does not exclude the presence of a neoplasm.

Day(s) Performed

Monday through Thursday

Report Available

5 to 10 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

84182

LOINC Code Information

Test ID Test Order Name Order LOINC Value
CRMWS CRMP-5-IgG Western Blot, S 47401-5

 

Result ID Test Result Name Result LOINC Value
83107 CRMP-5-IgG Western Blot, S 47401-5