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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, CSF

Specimen Type

CSF

Specimen 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-2  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 days

Test 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
Mayo Clinic Laboratories | Neurology Catalog Additional Information:

mml-neuroimmunology; mml-spinal-cord