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HelpTest Code SPPCI Stiff-Person Spectrum Disorders including Progressive Encephalomyelitis with Rigidity and Myoclonus, Interpretation, Spinal Fluid
Useful For
Interpretation for the evaluation of stiff-person spectrum disorders, including the classical or focal forms, such as stiff-limb or stiff-trunk, and progressive encephalomyelitis with rigidity and myoclonus, using spinal fluid specimens
Method Name
Only orderable as part of profile. For more information see SPPC / Stiff-Person Spectrum Disorders Evaluation, including Progressive Encephalomyelitis with Rigidity and Myoclonus, Spinal Fluid.
Medical Interpretation
Reporting Name
Stiff-Person/PERM Interp, CSFSpecimen Type
CSFSpecimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| CSF | Refrigerated (preferred) | 28 days | |
| Frozen | 28 days | ||
| Ambient | 72 hours |
Reference Values
Only orderable as part of profile. For more information see SPPC / Stiff-Person Spectrum Disorders Evaluation, including Progressive Encephalomyelitis with Rigidity and Myoclonus, Spinal Fluid.
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.
Clinical Reference
1. Hinson SR, Lopez-Chiriboga AS, Bower JH, et al: Glycine receptor modulating antibody predicting treatable stiff-person spectrum disorders. Neurol Neuroimmunol Neuroinflamm. 2018 Jan;5:e438
2. Hutchinson M, Waters P, McHugh J, et al: Progressive encephalomyelitis, rigidity, and myoclonus: a novel glycine receptor antibody. Neurology. 2008 Oct;71(16):1291-1292
3. Martinez-Hernandez E, Arino H, McKeon A, et al: Clinical and immunologic investigations in patients with stiff-person spectrum disorder. JAMA Neurol. 2016 Jun;73(6):714-720
4. McKeon A, Martinez-Hernandez E, Lancaster E, et al: Glycine receptor autoimmune spectrum with stiff-man syndrome phenotype. JAMA Neurol. 2013 Jan;70(1):44-50
5. McKeon A, Robinson MT, McEvoy KM, et al: Stiff-man syndrome and variants: clinical course, treatments, and outcomes. Arch Neurol. 2012 Feb;69(2):230-238
6. Pittock SJ, Lucchinetti CF, Parisi JE, et al: Amphiphysin autoimmunity: paraneoplastic accompaniments. Ann Neurol. 2005 Jun;58(1):96-107
7. Pittock SJ, Yoshikawa H, Ahlskog JE, et al: Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction. Mayo Clin Proc. 2006 Sep;81(9):1207-1214
8. Tobin WO, Lennon VA, Komorowski L, et al: DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients. Neurology. 2014 Nov;83(20):1797-1803
9. Walikonis JE, Lennon VA: Radioimmunoassay for glutamic acid decarboxylase (GAD65) autoantibodies as a diagnostic aid for stiff-man syndrome and a correlate of susceptibility to type 1 diabetes mellitus. Mayo Clin Proc. 1998 Dec;73(12):1161-1166
Day(s) Performed
Varies
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
Not ApplicableLOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| SPPCI | Stiff-Person/PERM Interp, CSF | 69048-7 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 614602 | Stiff-Person/PERM Interp, CSF | 69048-7 |
| 618909 | IFA Notes | 48767-8 |