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Test Code SPPS Stiff-Person Spectrum Disorders Evaluation, including Progressive Encephalomyelitis with Rigidity and Myoclonus, Serum


Ordering Guidance


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.

 

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 email 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: 2 mL

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


Useful For

Evaluating patients with suspected stiff-person syndrome (classical or focal forms, such as stiff-limb or stiff-trunk) and progressive encephalomyelitis with rigidity and myoclonus using serum specimens

Profile Information

Test ID Reporting Name Available Separately Always Performed
SPPSI Stiff-Person/PERM Interp, S No Yes
AMPHS Amphiphysin Ab, S No Yes
DPPCS DPPX Ab CBA, S No Yes
GD65S GAD65 Ab Assay, S Yes Yes
GLYCS Glycine Alpha1 LCBA, S Yes Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AMIBS Amphiphysin Immunoblot, S No No
DPPTS DPPX Ab IFA Titer, S No No
APHTS Amphiphysin Ab Titer, S No No

Testing Algorithm

If the indirect immunofluorescence assay (IFA) pattern suggests amphiphysin antibody, then amphiphysin immunoblot and amphiphysin IFA titer will be performed at an additional charge.

 

If the dipeptidyl-peptidase-like protein-6 (DPPX) cell bound assay is positive, then DPPX antibody IFA titer will be performed at an additional charge.

Method Name

AMPHS, APHTS, DPPTS: Indirect Immunofluorescence Assay (IFA)

AMIBS: Immunoblot

DPPCS: Cell-Binding Assay

GD65S: Radioimmunoassay (RIA)

GLYCS: Live Cell-Binding Assay

SPPSI: Medical Interpretation

Reporting Name

Stiff-Person/PERM Eval, S

Specimen Type

Serum

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

Reference Values

Test ID

Reporting Name

Methodology*

Reference Value

SPPSI

Stiff-Person/PERM Interp, S

Medical interpretation

N/A

AMPHS

Amphiphysin Ab, S

IFA

Negative

DPPCS

DPPX Ab CBA, S

CBA

Negative

GD65S

GAD65 Ab Assay, S

RIA

≤0.02 nmol/L

GLYCS

Glycine Alpha1 LCBA, S

LCBA

Negative

Reflex Information

Test ID

Reporting Name

Methodology

Reference Value

AMIBS

Amphiphysin Immunoblot, S

IB

Negative

APHTS

Amphiphysin Ab Titer, S

IFA

<1:240

DPPTS

DPPX Ab IFA Titer, S

IFA

<1:240

 

*Methodology abbreviations

Indirect Immunofluorescence Assay (IFA)

Cell-Binding Assay (CBA)

Radioimmunoassay (RIA)

Live Cell-Binding Assay (LCBA)

Immunoblot (IB)

 

Neuron-restricted patterns of IgG staining that do not fulfill criteria for amphiphysin antibody may be reported as "unclassified antineuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

Interpretation

Seropositivity supports the clinical diagnosis of stiff-person spectrum disorder (classical stiff-person, stiff-limb, stiff-trunk, or progressive encephalomyelitis with rigidity and myoclonus). A paraneoplastic basis should be considered.

Day(s) Performed

Profile tests: Monday through Sunday; Reflex tests: Varies

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

86255 x 2

86341

0431U

84182 (if appropriate)

86256 (if appropriate)

86256 (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
SPPS Stiff-Person/PERM Eval, S 94701-0

 

Result ID Test Result Name Result LOINC Value
81722 Amphiphysin Ab, S 72327-0
81596 GAD65 Ab Assay, S 30347-9
64933 DPPX Ab CBA, S 94676-4
606972 Glycine Alpha1 LCBA, S 96496-5
614601 Stiff-Person/PERM Interp, S 69048-7
618910 IFA Notes 48767-8

Forms

If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.