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, CSF (3006207).

Testing in both serum and CSF is recommended to maximize diagnostic yield; refer to Autoimmune Stiff-Person Disorders, Serum (3006234) for serum testing.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

N/A

Collect

CSF

Specimen Preparation

Transfer three 1 mL CSF aliquots to ARUP standard transport tubes. (Min: 0.5 mL/aliquot)

Storage/Transport Temperature

Frozen

Unacceptable Conditions

Fluid other than CSF. Grossly hemolyzed 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
  Glutamic Acid Decarboxylase Antibody CSF 0.0-5.0 IU/mL
  DPPX Ab IgG CBA-IFA Screen, CSF Less than 1:1
  Amphiphysin Antibody, CSF Negative

Interpretive Data

Refer to report.

Compliance Category

Laboratory Developed Test (LDT)

Note

If DPPX CSF 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
3002789 Glutamic Acid Decarboxylase Antibody CSF 42501-7
3004511 Amphiphysin Antibody, CSF 57750-2
3004513 DPPX Ab IgG CBA-IFA Screen, CSF 94283-9
* 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

  • Amphiphysin
  • Dipeptidyl aminopeptidase-like protein 6
  • DPPX
  • 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, CSF