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Email: | mcl@mayo.edu |
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Values are valid only on day of printing. |
Evaluating patients with suspected autoimmune myelopathy, myelitis, paraneoplastic myelopathy using spinal fluid specimens
If indirect immunofluorescence assay (IFA) patterns suggest AGNA-1 antibody, then AGNA-1 immunoblot is performed at an additional charge.
If IFA patterns suggest amphiphysin antibody, then amphiphysin immunoblot is performed at an additional charge.
If IFA patterns suggest ANNA-1 antibody, then ANNA-1 immunoblot is performed at an additional charge.
If IFA patterns suggest ANNA-2 antibody, then ANNA-2 immunoblot is performed at an additional charge.
If IFA patterns suggest PCA-1 antibody, then PCA-1 immunoblot is performed at an additional charge.
If IFA patterns suggest PCA-Tr antibody, then PCA-Tr immunoblot is performed at an additional charge.
If IFA pattern suggest NMDA-receptor antibody, then NMDA- receptor antibody cell-binding assay (CBA) and NMDA- receptor titer are performed at an additional charge.
If IFA pattern suggest AMPA- receptor antibody, then AMPA- receptor antibody CBA and AMPA- receptor titer are performed at an additional charge.
If IFA pattern suggest GABA-B- receptor antibody, then GABA-B- receptor antibody CBA and GABA-B- receptor titer are performed at an additional charge.
If IFA pattern suggest DPPX antibody, then DPPX antibody CBA and DPPX IFA titer are performed at an additional charge.
If IFA pattern suggest mGluR1 antibody, then mGluR1antibody CBA and mGluR1 IFA titer are performed at an additional charge.
If IFA pattern suggest GFAP antibody, then GFAP antibody CBA and GFAP IFA titer are performed at an additional charge.
If NMO/AQP4-IgG FACS screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay is performed at an additional charge.
If IFA pattern suggests NIF antibody, then alpha internexin CBA, NIF heavy chain CBA, NIF light chain CBA, and NIF titer are performed at an additional charge.
See Autoimmune Myelopathy Evaluation Algorithm-Spinal Fluid in Special Instructions.
Patients with autoimmune myelopathy present with subacute onset and rapid progression of spinal cord symptoms with one or more of the following: weakness, gait difficulties, loss of sensation, neuropathic pain, and bowel and bladder dysfunction. Clinical history and examination, spinal cord magnetic resonance imaging and cerebrospinal fluid (CSF) testing may provide clues to an autoimmune diagnosis. Autoimmune myelopathy evaluation of both serum and CSF can assist in the diagnosis (paraneoplastic or idiopathic autoimmune), and aid distinction from other causes of myelopathy (multiple sclerosis, sarcoidosis, vascular disease). Early testing may assist in early diagnosis of occult cancer, prompt initiation of immune therapies, or both.
Test ID | Reporting name | Methodology | Reference value |
MCI1 | Autoimmune Myelopathy Interp, CSF |
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AMPHC | Amphiphysin Ab, CSF | Indirect immunofluorescence assay (IFA) | <1:2 |
AGN1C | Anti-Glial Nuclear Ab, Type 1 | IFA | <1:2 |
ANN1C | Anti-Neuronal Nuclear Ab, Type 1 | IFA | <1:2 |
ANN2C | Anti-Neuronal Nuclear Ab, Type 2 | IFA | <1:2 |
ANN3C | Anti-Neuronal Nuclear Ab, Type 3 | IFA | <1:2 |
CRMWC | CRMP-5-IgG Western Blot, CSF | Western blot | Negative |
CRMC | CRMP-5-IgG, CSF | IFA | <1:2 |
DPPIC | DPPX Ab IFA, CSF | IFA | Negative |
GD65C | GAD65 Ab Assay, CSF | Radioimmunoassay | < or =0.02 nmol/L Reference values apply to all ages. |
GFAIC | GFAP IFA, CSF | IFA | Negative |
GL1IC | mGluR1 Ab IFA, CSF | IFA | Negative |
NIFIC | NIF IFA, CSF | IFA | Negative |
NMOFC | NMO/AQP4 FACS, CSF | Flow cytometry | Negative |
PCTRC | Purkinje Cell Cytoplasmic Ab Type Tr | IFA | <1:2 |
PCA1C | Purkinje Cell Cytoplasmic Ab Type 1 | IFA | <1:2 |
PCA2C | Purkinje Cell Cytoplasmic Ab Type 2 | IFA | <1:2 |
Reflex Information:
Test ID | Reporting name | Methodology | Reference value |
AGNBC | AGNA-1 Immunoblot, CSF | Immunoblot (IB) | Negative |
AINCC | Alpha Internexin CBA, CSF | Cell-binding assay (CBA) | Negative |
AMPCC | AMPA-R Ab CBA, CSF | CBA | Negative |
AMPIC | AMPA-R Ab IF Titer Assay, CSF | IFA | <1:2 |
AMIBC | Amphiphysin Immunoblot, CSF | IB | Negative |
AN1BC | ANNA-1 Immunoblot, CSF | IB | Negative |
AN2BC | ANNA-2 Immunoblot, CSF | IB | Negative |
DPPCC | DPPX Ab CBA, CSF | CBA | Negative |
DPPTC | DPPX Ab IFA Titer, CSF | IFA | <1:2 |
GABCC | GABA-B-R Ab CBA, CSF | CBA | Negative |
GABIC | GABA-B-R Ab IF Titer Assay, CSF | IFA | <1:2 |
GFACC | GFAP CBA, CSF | CBA | Negative |
GFATC | GFAP IFA Titer, CSF | IFA | <1:2 |
GL1CC | mGluR1 Ab CBA, CSF | CBA | Negative |
GL1TC | mGluR1 Ab IFA Titer, CSF | IFA | <1:2 |
NFHCC | NIF Heavy Chain CBA, CSF | CBA | Negative |
NIFTC | NIF IFA Titer, CSF | IFA | <1:2 |
NFLCC | NIF Light Chain CBA, CSF | CBA | Negative |
NMDCC | NMDA-R Ab CBA, CSF | CBA | Negative |
NMDIC | NMDA-R Ab IF Titer Assay, CSF | IFA | <1:2 |
NMOTC | NMO/AQP4 FACS Titer, CSF | Flow cytometry | <1:2 |
PC1BC | PCA-1 Immunoblot, CSF | IB | Negative |
PCTBC | PCA-Tr Immunoblot, CSF | IB | Negative |
Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, CRMP-5-IgG, PCA-1, PCA-2, or PCA-Tr may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."
A positive result is consistent with a diagnosis of autoimmune myelopathy in the appropriate clinical context.
Negative results do not exclude a diagnosis of autoimmune myelopathy.
1. Dubey D, Pittock SJ, Krecke KN: Clinical, Radiologic, and Prognostic Features of Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Autoantibody. JAMA Neurol 2018 Dec 21. doi: 10.1001/jamaneurol.2018.4053. Epub ahead of print
2. Zalewski NL, Flanagan EP: Autoimmune and Paraneoplastic Myelopathies. Semin Neurol 2018 Jun;38(3):278-289
3. Flanagan EP, Hinson SR, Lennon VA: Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients. Ann Neurol 2017;81:298-309
4. Keegan BM, Pittock SJ, Lennon VA: Autoimmune myelopathy associated with collapsin response-mediator protein-5 immunoglobulin G. Ann Neurol 2008;63:531-534
5. Weinshenker BG, Wingerchuk DM, Vukusic S: Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006;59:566-569