Ordering Recommendation

Use to evaluate stiffness, spasms, hyperekplexia, limb rigidity, myoclonus, or limb dystonia in the presence or absence of encephalopathy. For individuals who present with a broader phenotype suggestive of immune-mediated movement disorder, refer to Autoimmune Movement Disorder Panel, Serum (3006206).

Testing in both serum and CSF is recommended to maximize diagnostic yield; refer to Autoimmune Stiff-Person Disorders, CSF (3006235) for CSF testing.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

N/A

Collect

Serum separator tube (SST)

Specimen Preparation

Separate from cells ASAP or within 2 hours of collection. Transfer three 1 mL serum aliquots to ARUP standard transport tubes. (Min: 0.5 mL/aliquot)

Storage/Transport Temperature

Frozen

Unacceptable Conditions

Amniotic fluid, ocular fluid, peritoneal fluid, synovial fluid, CSF, or plasma. Contaminated, hemolyzed, icteric, or lipemic specimens.

Remarks
Stability

After separation from cells: Ambient: 24 hours; Refrigerated: 1 week; Frozen: 30 days (avoid repeated freeze/thaw cycles)

Methodology

Semi-Quantitative Cell-Based Indirect Fluorescent Antibody/Qualitative Immunoblot/Semi-Quantitative Enzyme-Linked Immunosorbent Assay (ELISA)

Performed

Varies

Reported

3-10 days

Reference Interval

Test Number
Components
Reference Interval
  Neuronal Antibody (Amphiphysin) Negative
  Glutamic Acid Decarboxylase Antibody 0.0-5.0 IU/mL
  DPPX Ab IgG CBA-IFA Screen, Serum Less than 1:10

Interpretive Data

Refer to report.

Compliance Category

Laboratory Developed Test (LDT)

Note

If DPPX antibody IgG by IFA is positive, then titer will be added. Additional charges apply.

Hotline History

N/A

CPT Codes

86341; 84182; 86255; if reflexed, add 86256

Components

Component Test Code* Component Chart Name LOINC
0051709 Neuronal Antibody (Amphiphysin) 94385-2
2001772 Glutamic Acid Decarboxylase Antibody 56540-8
3004361 DPPX Ab IgG CBA-IFA Screen, Serum 94676-4
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.

Aliases

  • DPPX
  • Amphiphysin
  • Dipeptidyl aminopeptidase-like protein 6
  • dystonia
  • GAD65
  • Glutamic acid decarboxylase
  • hyperekplexia
  • PERM
  • Progressive encephalomyelitis
  • Progressive encephalomyelitis with rigidity and myoclonus
  • rigidity
  • spasms
  • Spinal interneuronitis
  • SPS
  • Stiff man
  • Stiff person
  • Stiff-man
  • Stiff-person
  • Stiff-person syndrome
Autoimmune Stiff-Person Disorders, Serum