Test Id : PVLE
Paraneoplastic Vision Loss Evaluation, Serum
Useful For
Suggests clinical disorders or settings where the test may be helpful
Evaluating patients with rapidly progressive vision loss where a paraneoplastic cause for vision loss (retinopathy or optic neuritis with other findings [eg, retinitis] is suspected)
Evaluating patients with small-cell carcinoma who develop vision loss
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.
| Test Id | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| PVLEI | Paraneoplas Vision Loss Interp, S | No | Yes |
| CRMS | CRMP-5-IgG, S | No | Yes |
| RCVBS | Recoverin Immunoblot, S | Yes | 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.
| Test Id | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| CRMWS | CRMP-5-IgG Western Blot, S | Yes | No |
| CRMTS | CRMP-5-IgG Titer, S | No | No |
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If the indirect immunofluorescence assay (IFA) patterns suggest collapsin response-mediator protein-5 (CRMP-5) antibody, then CRMP-5 IFA titer and CRMP-5 Western blot will be performed at an additional charge.
Method Name
A short description of the method used to perform the test
PVLEI: Medical Interpretation
CRMTS, CRMS: Indirect Immunofluorescence Assay (IFA)
CRMWS: Western Blot (WB)
RCVBS: Immunoblot (IB)
NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test
Aliases
Lists additional common names for a test, as an aid in searching
CRMP-5
Anti-CV-2
Recoverin
Retinopathy
Ophthalmitis
Anti-CAR
CAR
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If the indirect immunofluorescence assay (IFA) patterns suggest collapsin response-mediator protein-5 (CRMP-5) antibody, then CRMP-5 IFA titer and CRMP-5 Western blot will be performed at an additional charge.
Specimen Type
Describes the specimen type validated for testing
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.
For a list of antibodies performed with each evaluation, see Autoimmune Neurology Antibody Matrix.
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
Patient Preparation:
1. For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication.
2. 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 1 week and assayed if sufficiently decayed or canceled if radioactivity remains.
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
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
Forms
If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.
Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the testing laboratory. The minimum volume is sufficient for one attempt at testing.
2 mL
Reject Due To
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
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 28 days | |
| Ambient | 72 hours | ||
| Frozen | 28 days |
Useful For
Suggests clinical disorders or settings where the test may be helpful
Evaluating patients with rapidly progressive vision loss where a paraneoplastic cause for vision loss (retinopathy or optic neuritis with other findings [eg, retinitis] is suspected)
Evaluating patients with small-cell carcinoma who develop vision loss
Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If the indirect immunofluorescence assay (IFA) patterns suggest collapsin response-mediator protein-5 (CRMP-5) antibody, then CRMP-5 IFA titer and CRMP-5 Western blot will be performed at an additional charge.
Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
There are 2 recognized forms of paraneoplastic vision loss: paraneoplastic autoimmune optic neuropathy with retinopathy accompanying collapsin response-mediator protein-5 (CRMP-5)-IgG and cancer associated retinopathy (CAR) accompanying recoverin antibody. Both occur in the setting of occult small-cell carcinoma of the lung or other body region.
Patients with CRMP-5-IgG associated optic neuropathy commonly present with painless bilateral visual loss over weeks to months. At onset, there is typically bilateral optic disc edema without evidence of enhancement of the optic nerve on magnetic resonance imaging or elevated opening pressure on lumbar puncture. Visual acuity can range from 20/20 to hand motion. In most cases, patients have coexisting vitritis or retinitis. In addition, patients can have diplopia, usually from cerebellar involvement. The majority of patients with CRMP-5 associated optic neuropathy will have other neurologic deficits from CRMP-5 autoimmunity, such as asymmetric axonal polyradiculoneuropathy. CAR presents with subacute painless progressive bilateral (although asymmetry has been described) progressive vision loss over weeks to months, reflecting both rod and cone retinal dysfunction in most patients. Accordingly, symptoms often include nyctalopia (inability to see in dim light or at night), impaired dark adaptation, photopsia (flashes of light in the field of vision), photosensitivity, dyschromatopsia, and, ultimately, severe visual acuity loss.
Patients with CRMP-5-IgG-related ophthalmitis may have improvements with intra-ocular or systemic corticosteroid treatment. Patients with recoverin-related retinopathy are unlikely to have vision improvement with treatment.
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.
COLLAPSIN RESPONSE-MEDIATOR PROTEIN-5-IgGNegative
RECOVERIN IMMUNOBLOT
Negative
COLLAPSIN RESPONSE-MEDIATOR PROTEIN-5 TITER
<1:240
COLLAPSIN RESPONSE-MEDIATOR PROTEIN-5 WESTERN BLOT
Negative
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 1-800-533-1710 to request CRMP-5 Western blot. Neuron-restricted patterns of IgG staining that do not fulfill criteria for CRMP-5-IgG may be reported as "unclassified antineuronal IgG." Complex patterns that include non-neuronal elements may be reported as "uninterpretable."
Interpretation
Provides information to assist in interpretation of the test results
Recoverin IgG:
Seropositivity is consistent with a diagnosis of paraneoplastic retinopathy. Considerations include small-cell carcinoma, pulmonary, or extrapulmonary.
Collapsin response-mediator protein-5 IgG:
Seropositivity is consistent with a diagnosis of paraneoplastic retinitis or ophthalmitis. Considerations include small-cell carcinoma, pulmonary, or extrapulmonary.
Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Negative results do not exclude the diagnosis of paraneoplastic eye disease.
Clinical Reference
Recommendations for in-depth reading of a clinical nature
1. Cross SA, Salomao DR, Parisi JE, et al: Paraneoplastic autoimmune optic neuritis with retinitis defined by CRMP-5-IgG. Ann Neurol. 2003 Jul;54(1):38-50 doi: 10.1002/ana.10587
2. Lopez A, McKeon A, Lachance D, et al: Recoverin antibody: Ophthalmologic and oncologic significance. Neurology. 2016 Apr 5;86(16 Supplement)P6.131
Method Description
Describes how the test is performed and provides a method-specific reference
Indirect Immunofluorescence Assay:
The patient's sample is tested by a standardized immunofluorescence assay 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. Neurol Neuroimmunol Neuroinflamm 2017 Jul 18;4(5):e385. doi: 10.1212/NXI.0000000000000385)
Immunoblot:
All steps are performed at ambient temperature (18-28 degrees C) utilizing the EUROBlot One instrument. Diluted patient specimen (1:12.5) 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 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)
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;49[2]:145-154; 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)
PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information
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.
Profile tests: Monday through Sunday; Reflex tests: Varies
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.
Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location
Indicates the location of the laboratory that performs the test
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.
- 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 account representative. 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.
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
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 x1
84182 x1
84182 CRMWS (if appropriate)
86256 CRMTS (if appropriate)
LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.
| Test Id | Test Order Name | Order LOINC Value |
|---|---|---|
| PVLE | Paraneoplastic Vision Loss Eval, S | 104992-3 |
| Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
|---|---|---|
| 83077 | CRMP-5-IgG, S | 72504-4 |
| 610009 | Recoverin Immunoblot, S | 83003-4 |
| 607411 | Paraneoplas Vision Loss Interp, S | 104993-1 |
| 618908 | IFA Notes | 48767-8 |