Sign in →

Test Code SPPC Stiff-Person Spectrum Disorders Evaluation, including Progressive Encephalomyelitis with Rigidity and Myoclonus, Spinal Fluid


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


Container/Tube: Sterile vial

Preferred: Vial number 1

Acceptable: Any vial

Specimen Volume: 4 mL


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 spinal fluid specimens

Profile Information

Test ID Reporting Name Available Separately Always Performed
SPPCI Stiff-Person/PERM Interp, CSF No Yes
AMPHC Amphiphysin Ab, CSF No Yes
DPPCC DPPX Ab CBA, CSF No Yes
GD65C GAD65 Ab Assay, CSF Yes Yes
GLYCC Glycine Alpha1 LCBA, CSF Yes Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
AMIBC Amphiphysin Immunoblot, CSF No No
DPPTC DPPX Ab IFA Titer, CSF No No
APHTC Amphiphysin Ab Titer, CSF 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

AMPHC, APHTC, DPPTC: Indirect Immunofluorescence Assay (IFA)

AMIBC: Immunoblot

DPPCC: Cell-Binding Assay

GD65C: Radioimmunoassay (RIA)

GLYCC: Live Cell-Binding Assay

Reporting Name

Stiff-Person/PERM Eval, CSF

Specimen Type

CSF

Specimen Minimum Volume

2 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
CSF 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

SPPCI

Stiff-Person/PERM Interp, CSF

Medical interpretation

N/A

AMPHC

Amphiphysin Ab, CSF

IFA

Negative

DPPCC

DPPX Ab CBA, CSF

CBA

Negative

GD65C

GAD65 Ab Assay, CSF

RIA

≤0.02 nmol/L

GLYCC

Glycine Alpha1 LCBA, CSF

LCBA

Negative

Reflex information

Test ID

Reporting Name

Methodology

Reference Value

AMIBC

Amphiphysin Immunoblot, CSF

IB

Negative

APHTC

Amphiphysin Ab Titer, CSF

IFA

<1:2

DPPTC

DPPX Ab IFA Titer, CSF

IFA

<1:2

 

*Methodology abbreviations:

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

Spinal fluid antibody positivity 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
SPPC Stiff-Person/PERM Eval, CSF 94712-7

 

Result ID Test Result Name Result LOINC Value
5906 Amphiphysin Ab, CSF 90815-2
21702 GAD65 Ab Assay, CSF 94359-7
64934 DPPX Ab CBA, CSF 94283-9
606973 Glycine Alpha1 LCBA, CSF 96497-3
614602 Stiff-Person/PERM Interp, CSF 69048-7
618909 IFA Notes 48767-8

Forms

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