Test ID: MAC1 Myelopathy, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid
Ordering Guidance
Multiple neuroimmunology profile tests are available. For testing that is performed with each profile, see Autoimmune Neurology Antibody Matrix.
Necessary Information
Provide the following information:
-Relevant clinical information
-Ordering provider name, phone number, mailing address, and e-mail address
Specimen Required
Container/Tube: Sterile vial
Preferred: Vial number 1
Acceptable: Any vial
Specimen Volume: 4 mL
Forms
If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.
Useful For
Evaluating patients with suspected autoimmune myelopathy, myelitis, and paraneoplastic myelopathy using spinal fluid specimens
Profile Information
Test ID | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
MCI1 | Autoimmune Myelopathy Interp, CSF | No | Yes |
AMPHC | Amphiphysin Ab, CSF | No | Yes |
AGN1C | Anti-Glial Nuclear Ab, Type 1 | No | Yes |
ANN1C | Anti-Neuronal Nuclear Ab, Type 1 | No | Yes |
ANN2C | Anti-Neuronal Nuclear Ab, Type 2 | No | Yes |
ANN3C | Anti-Neuronal Nuclear Ab, Type 3 | No | Yes |
APBIC | AP3B2 IFA, CSF | No | Yes |
CRMWC | CRMP-5-IgG Western Blot, CSF | Yes | Yes |
DPPIC | DPPX Ab IFA, CSF | No | Yes |
GABCC | GABA-B-R Ab CBA, CSF | No | Yes |
GD65C | GAD65 Ab Assay, CSF | Yes | Yes |
GFAIC | GFAP IFA, CSF | No | Yes |
GL1IC | mGluR1 Ab IFA, CSF | No | Yes |
NCDIC | Neurochondrin IFA, CSF | No | Yes |
NIFIC | NIF IFA, CSF | No | Yes |
NMOFC | NMO/AQP4 FACS, CSF | Yes | Yes |
PCA1C | Purkinje Cell Cytoplasmic Ab Type 1 | No | Yes |
PCA2C | Purkinje Cell Cytoplasmic Ab Type 2 | No | Yes |
SP7IC | Septin-7 IFA, CSF | No | Yes |
Reflex Tests
Test ID | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
AGNBC | AGNA-1 Immunoblot, CSF | No | No |
AINCC | Alpha Internexin CBA, CSF | No | No |
AMIBC | Amphiphysin Immunoblot, CSF | No | No |
AN1BC | ANNA-1 Immunoblot, CSF | No | No |
AN2BC | ANNA-2 Immunoblot, CSF | No | No |
DPPCC | DPPX Ab CBA, CSF | No | No |
DPPTC | DPPX Ab IFA Titer, CSF | No | No |
GABIC | GABA-B-R Ab IF Titer Assay, CSF | No | No |
GFACC | GFAP CBA, CSF | No | No |
GFATC | GFAP IFA Titer, CSF | No | No |
GL1CC | mGluR1 Ab CBA, CSF | No | No |
GL1TC | mGluR1 Ab IFA Titer, CSF | No | No |
NFHCC | NIF Heavy Chain CBA, CSF | No | No |
NIFTC | NIF IFA Titer, CSF | No | No |
NFLCC | NIF Light Chain CBA, CSF | No | No |
NMOTC | NMO/AQP4 FACS Titer, CSF | No | No |
PC1BC | PCA-1 Immunoblot, CSF | No | No |
AGNTC | AGNA-1 Titer, CSF | No | No |
AN1TC | ANNA-1 Titer, CSF | No | No |
AN2TC | ANNA-2 Titer, CSF | No | No |
AN3TC | ANNA-3 Titer, CSF | No | No |
APBCC | AP3B2 CBA, CSF | No | No |
APBTC | AP3B2 IFA Titer, CSF | No | No |
APHTC | Amphiphysin Ab Titer, CSF | No | No |
CRMTC | CRMP-5-IgG Titer, CSF | No | No |
NCDCC | Neurochondrin CBA, CSF | No | No |
NCDTC | Neurochondrin IFA Titer, CSF | No | No |
PC1TC | PCA-1 Titer, CSF | No | No |
PC2TC | PCA-2 Titer, CSF | No | No |
SP7CC | Septin-7 CBA, CSF | No | No |
SP7TC | Septin-7 IFA Titer, CSF | No | No |
Testing Algorithm
If the indirect immunofluorescence assay (IFA) pattern suggests anti-glial nuclear antibody (AGNA-1), then AGNA-1 immunoblot and AGNA-1 IFA titer will be performed at an additional charge.
If the IFA pattern suggests amphiphysin antibody, then amphiphysin immunoblot and amphiphysin IFA titer will be performed at an additional charge.
If the IFA pattern suggests anti-neuronal nuclear antibody type 1 (ANNA-1), then ANNA-1 immunoblot, ANNA-1 IFA titer, and ANNA-2 immunoblot will be performed at an additional charge.
If the IFA pattern suggests ANNA-2 antibody, then ANNA-2 immunoblot, ANNA-2 IFA titer, and ANNA-1 immunoblot will be performed at an additional charge.
If client requests or the IFA pattern suggests ANNA-3 antibodies, then ANNA-3 IFA titer will be performed at an additional charge.
If the IFA pattern suggests adaptor protein 3 beta 2 (AP3B2) antibodies, then AP3BC cell-binding assay (CBA) and AP3BC IFA titer will be performed at an additional charge.
If collapsin response-mediator protein 5 (CRMP-5)-IgG Western blot is positive, then CRMP-5-IgG IFA titer is performed at an additional charge.
If the IFA pattern suggests Purkinje cell cytoplasmic antibody type 1 (PCA-1), then PCA-1 immunoblot and PCA-1 IFA titer will be performed at an additional charge.
If the IFA pattern suggests PCA-2 antibody, then PCA-2 IFA titer will be performed at an additional charge.
If the IFA pattern suggests N-methyl-D-aspartate (NMDA) receptor antibody, then NMDA-receptor antibody CBA and NMDA-receptor antibody IFA titer will be performed at an additional charge.
If gamma-aminobutyric acid B (GABA-B) receptor antibody CBA is positive, then GABA-B-receptor antibody IFA titer will be performed at an additional charge.
If the IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX antibody IFA titer will be performed at an additional charge.
If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 antibody CBA and mGluR1 antibody IFA titer will be performed at an additional charge.
If the IFA pattern suggests glial fibrillary acidic protein (GFAP) antibody, then GFAP antibody CBA and GFAP antibody IFA titer will be performed at an additional charge.
If the neuromyelitis optica/aquaporin-4-IgG (NMO/AQP4-IgG) fluorescence-activated cell sorting (FACS) screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay will be performed at an additional charge.
If the IFA pattern suggests neuronal intermediate filament (NIF) antibody, then alpha internexin CBA, NIF heavy chain CBA, NIF light chain CBA, and NIF antibody IFA titer will be performed at an additional charge.
If the IFA pattern suggests neurochondrin antibody, then neurochondrin antibody CBA and neurochondrin IFA titer will be performed at an additional charge.
If the IFA pattern suggests septin 7 antibody, then septin 7 CBA and septin 7 IFA titer will be performed at an additional charge.
For more information see Autoimmune/Paraneoplastic Myelopathy Evaluation Algorithm-Spinal Fluid.
Method Name
MCI1: Medical Interpretation
AGN1C, AGNTC, AMPHC, APHTC, ANN1C, AN1TC, ANN2C, AN2TC, ANN3C, AN3TC, APBIC, APBTC, CRMTC, DPPIC, DPPTC, GABIC, GFAIC, GFATC, GL1IC, GL1TC, NCDIC, NCDTC, NIFIC, NIFTC, NMDIC, PCA1C, PC1TC, PCA2C, PC2TC, SP7IC, SP7TC: Indirect Immunofluorescence Assay (IFA)
GD65C: Radioimmunoassay (RIA)
CRMWC: Western Blot (WB)
AGNBC, AMIBC, AN1BC, AN2BC, PC1BC: Immunoblot (IB)
NMOFC, NMOTC: Flow Cytometry (FCM)
APBCC, DPPCC, GFACC, GL1CC, NCDCC, AINCC, NMDCC, NFLCC, NFHCC, SP7CC: Cell Binding Assay (CBA)
Reporting Name
Myelopathy, Autoimm/Paraneo, CSFSpecimen Type
CSFSpecimen Minimum Volume
2 mL
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
CSF | Refrigerated (preferred) | 28 days | |
Frozen | 28 days | ||
Ambient | 72 hours |
Clinical Information
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.
Reference Values
Test ID |
Reporting name |
Methodology* |
Reference value |
MCI1 |
Autoimmune Myelopathy Interp, CSF |
Medical interpretation |
N/A |
AMPHC |
Amphiphysin Ab, CSF |
IFA |
Negative |
AGN1C |
Anti-Glial Nuclear Ab, Type 1 |
IFA |
Negative |
ANN1C |
Anti-Neuronal Nuclear Ab, Type 1 |
IFA |
Negative |
ANN2C |
Anti-Neuronal Nuclear Ab, Type 2 |
IFA |
Negative |
ANN3C |
Anti-Neuronal Nuclear Ab, Type 3 |
IFA |
Negative |
APBIC |
AP3B2 IFA, CSF |
IFA |
Negative |
CRMWC |
CRMP-5-IgG Western Blot, CSF |
WB |
Negative |
DPPIC |
DPPX Ab IFA, CSF |
IFA |
Negative |
GABCC |
GABA-B-R Ab CBA, CSF |
CBA |
Negative |
GD65C |
GAD65 Ab Assay, CSF |
RIA |
≤0.02 nmol/L Reference values apply to all ages. |
GFAIC |
GFAP IFA, CSF |
IFA |
Negative |
GL1IC |
mGluR1 Ab IFA, CSF |
IFA |
Negative |
NCDIC |
Neurochondrin IFA, CSF |
IFA |
Negative |
NIFIC |
NIF IFA, CSF |
IFA |
Negative |
NMOFC |
NMO/AQP4 FACS, CSF |
FCM |
Negative |
PCA1C |
Purkinje Cell Cytoplasmic Ab Type 1 |
IFA |
Negative |
PCA2C |
Purkinje Cell Cytoplasmic Ab Type 2 |
IFA |
Negative |
SP7IC |
Septin-7 IFA, CSF |
IFA |
Negative |
Reflex Information:
Test ID |
Reporting name |
Methodology* |
Reference value |
AGNBC |
AGNA-1 Immunoblot, CSF |
IB |
Negative |
AGNTC |
AGNA-1 Titer, CSF |
IFA |
<1:2 |
AINCC |
Alpha Internexin CBA, CSF |
CBA |
Negative |
AMIBC |
Amphiphysin Immunoblot, CSF |
IB |
Negative |
AN1BC |
ANNA-1 Immunoblot, CSF |
IB |
Negative |
AN1TC |
ANNA-1 Titer, CSF |
IFA |
<1:2 |
AN2BC |
ANNA-2 Immunoblot, CSF |
IB |
Negative |
AN2TC |
ANNA-2 Titer, CSF |
IFA |
<1:2 |
AN3TC |
ANNA-3 Titer, CSF |
IFA |
<1:2 |
APBCC |
AP3B2 CBA, CSF |
CBA |
Negative |
APBTC |
AP3B2 IFA Titer, CSF |
IFA |
<1:2 |
APHTC |
Amphiphysin Ab Titer, CSF |
IFA |
<1:2 |
CRMTC |
CRMP-5-IgG Titer, CSF |
IFA |
<1:2 |
DPPCC |
DPPX Ab CBA, CSF |
CBA |
Negative |
DPPTC |
DPPX Ab IFA Titer, CSF |
IFA |
<1:2 |
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 |
NCDCC |
Neurochondrin CBA, CSF |
CBA |
Negative |
NCDTC |
Neurochondrin 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 |
FCM |
<1:2 |
PC1BC |
PCA-1 Immunoblot, CSF |
IB |
Negative |
PC1TC |
PCA-1 Titer, CSF |
IFA |
<1:2 |
PC2TC |
PCA-2 Titer, CSF |
IFA |
<1:2 |
SP7CC |
Septin-7 CBA, CSF |
CBA |
Negative |
SP7TC |
Septin-7 IFA Titer, CSF |
IFA |
<1:2 |
*Methodology Abbreviations:
Immunofluorescence assay (IFA)
Cell-binding assay (CBA)
Flow Cytometry (FCM)
Radioimmunoassay (RIA)
Immunoblot (IB)
Western blot (WB)
Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, CRMP-5-IgG, PCA-1, or PCA-2Â may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."
Interpretation
A positive result is consistent with a diagnosis of autoimmune myelopathy in the appropriate clinical context.
Clinical Reference
1. Dubey D, Pittock SJ, Krecke KN, et al: Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 2019 Mar 1;76(3):301-309. doi: 10.1001/jamaneurol.2018.4053
2. Zalewski NL, Flanagan EP: Autoimmune and paraneoplastic myelopathies. Semin Neurol. 2018 Jun;38(3):278-289. doi: 10.1055/s-0038-1660856
3. Flanagan EP, Hinson SR, Lennon VA, et al: Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients. Ann Neurol. 2017 Feb;81(2):298-309. doi: 10.1002/ana.24881
4. Keegan BM, Pittock SJ, Lennon VA: Autoimmune myelopathy associated with collapsin response-mediator protein-5 immunoglobulin G. Ann Neurol. 2008 Apr;63(4):531-534. doi: 10.1002/ana.21324
5. Weinshenker BG, Wingerchuk DM, Vukusic S, et al: Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006 Mar;59(3):566-569. doi: 10.1002/ana.20770
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
8 to 12 daysTest Classification
This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86255 x 15
86053
86341
84182
84182 AGNBC (if appropriate)
86256 AGNTC (if appropriate)
86255 AINCC (if appropriate)
84182 AMIBC (if appropriate)
84182 AN1BC (if appropriate)
86256 AN1TC (if appropriate)
84182 AN2BC (if appropriate)
86256 AN2TC (if appropriate)
86256 AN3TC (if appropriate)
86255 APBCC (if appropriate)
86256 APBTC (if appropriate)
86256 APHTC (if appropriate)
86256 CRMTC (if appropriate)
86255 DPPCC (if appropriate)
86256 DPPTC (if appropriate)
86256 GABIC (if appropriate)
86255 GFACC (if appropriate)
86256 GFATC (if appropriate)
86255 GL1CC (if appropriate)
86256 GL1TC (if appropriate)
86255 NCDCC (if appropriate)
86256 NCDTC (if appropriate)
86255 NFHCC (if appropriate)
86255 NFLCC (if appropriate)
86256 NIFTC (if appropriate)
86255-NMDCC (if appropriate)
86256-NMDIC (if appropriate)
86053 NMOTC (if appropriate)
84182 PC1BC (if appropriate)
86256 PC1TC (if appropriate)
86256 PC2TC (if appropriate)
86255 SP7CC (if appropriate)
86256 SP7TC (if appropriate)
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
MAC1 | Myelopathy, Autoimm/Paraneo, CSF | 94353-0 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
61515 | GABA-B-R Ab CBA, CSF | 93426-5 |
38325 | NMO/AQP4-IgG FACS, CSF | 46718-3 |
64929 | DPPX Ab IFA, CSF | 82989-5 |
64927 | mGluR1 Ab IFA, CSF | 94361-3 |
605156 | GFAP IFA, CSF | 94360-5 |
605128 | Autoimmune Myelopathy Interp, CSF | 69048-7 |
606965 | NIF IFA, CSF | 96490-8 |
615862 | AP3B2 IFA, CSF | In Process |
615866 | Neurochondrin IFA, CSF | 101451-3 |
615874 | Septin-7 IFA, CSF | In Process |
89079 | AGNA-1, CSF | 94355-5 |
5906 | Amphiphysin Ab, CSF | 94354-8 |
3852 | ANNA-1, CSF | 94356-3 |
7472 | ANNA-2, CSF | 94357-1 |
21633 | ANNA-3, CSF | 94358-9 |
3988 | PCA-1, CSF | 94363-9 |
21632 | PCA-2, CSF | 94364-7 |
21747 | CRMP-5-IgG Western Blot, CSF | 53707-6 |
21702 | GAD65 Ab Assay, CSF | 94359-7 |
618901 | IFA Notes | 48767-8 |
Special Instructions
mml-neuroimmunology; mml-spinal-cord