Test ID: MDC2 Movement Disorder, 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
Patient Preparation:
For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication, or corticosteroid or intravenous immunoglobulin (IVIg) treatment.
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 paraneoplastic or other autoimmune movement disorders including patients with ataxia, brainstem encephalitis, chorea, dyskinesias, myoclonus, and parkinsonism using spinal fluid specimens
Profile Information
Test ID | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
MDCI | Movement Disorder 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 |
CS2CC | CASPR2-IgG CBA, CSF | No | Yes |
CRMWC | CRMP-5-IgG Western Blot, CSF | Yes | Yes |
CRMC | CRMP-5-IgG, CSF | No | Yes |
DPPIC | DPPX Ab IFA, CSF | No | Yes |
GD65C | GAD65 Ab Assay, CSF | Yes | Yes |
GRFIC | GRAF1 IFA, CSF | No | Yes |
IG5IC | IgLON5 IFA, CSF | No | Yes |
ITPIC | ITPR1 IFA, CSF | No | Yes |
K11CC | KLHL11 Ab CBA, CSF | Yes | Yes |
LG1CC | LGI1-IgG CBA, CSF | No | Yes |
GL1IC | mGluR1 Ab IFA, CSF | No | Yes |
NIFIC | NIF IFA, CSF | No | Yes |
NMDCC | NMDA-R Ab CBA, CSF | No | Yes |
PCTRC | Purkinje Cell Cytoplasmc Ab Type Tr | No | Yes |
PCA1C | Purkinje Cell Cytoplasmic Ab Type 1 | No | Yes |
PCA2C | Purkinje Cell Cytoplasmic Ab Type 2 | 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 |
AMPIC | AMPA-R Ab IF Titer Assay, CSF | No | No |
AMPCC | AMPA-R Ab CBA, CSF | No | No |
AMIBC | Amphiphysin Immunoblot, CSF | No | No |
AN1BC | ANNA-1 Immunoblot, CSF | No | No |
AN2BC | ANNA-2 Immunoblot, CSF | No | No |
DPPTC | DPPX Ab IFA Titer, CSF | No | No |
DPPCC | DPPX Ab CBA, CSF | No | No |
GABIC | GABA-B-R Ab IF Titer Assay, CSF | No | No |
GABCC | GABA-B-R Ab CBA, CSF | No | No |
GRFCC | GRAF1 CBA, CSF | No | No |
GRFTC | GRAF1 IFA Titer, CSF | No | No |
IG5CC | IgLON5 CBA, CSF | No | No |
IG5TC | IgLON5 IFA Titer, CSF | No | No |
ITPCC | ITPR1 CBA, CSF | No | No |
ITPTC | ITPR1 IFA Titer, CSF | No | No |
GL1TC | mGluR1 Ab IFA Titer, CSF | No | No |
GL1CC | mGluR1 Ab CBA, 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 |
NMDIC | NMDA-R Ab IF Titer Assay, CSF | No | No |
PC1BC | PCA-1 Immunoblot, CSF | No | No |
PCTBC | PCA-Tr Immunoblot, CSF | No | No |
K11TC | KLHL11 Ab IFA Titer, CSF | No | No |
Testing Algorithm
If immunofluorescence assay (IFA) pattern suggests amphiphysin antibody, then amphiphysin immunoblot is performed at an additional charge.
If IFA pattern suggests antiglial nuclear antibody (AGNA)-1, then AGNA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests antineuronal nuclear antibody (ANNA)-1, then ANNA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests ANNA-2, then ANNA-2 immunoblot is performed at an additional charge.
If IFA pattern suggests Purkinje cytoplasmic antibody (PCA)-1, then PCA-1 immunoblot is performed at an additional charge.
If IFA pattern suggests PCA-Tr ,then PCA-Tr immunoblot is performed at an additional charge.
If IFA pattern suggests alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA)-receptor antibody, then AMPA-receptor cell-binding assay (CBA) and AMPA-receptor antibody IFA titer are performed at an additional charge.
If IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX CBA and DPPX antibody IFA titer are performed at an additional charge.
If IFA pattern suggests gamma-aminobutyric acid B (GABA-B)-receptor antibody, then GABA-B-receptor CBA and GABA-B-receptor antibody IFA titer are performed at an additional charge.
If IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 CBA and mGluR1 antibody IFA titer are performed at an additional charge.
If IFA pattern suggests N-methyl-D-aspartate (NMDA)-receptor antibody and NMDA-receptor CBA is positive, then NMDA-receptor antibody IFA titer is performed at an additional charge.
If IFA pattern suggests GTPase regulator associated with focal adhesion kinase-1 (GRAF1) antibody, then GRAF1 CBA and GRAF1 antibody IFA titer are performed at an additional charge.
If IFA pattern suggests IgLON5 antibody, then IgLON5 CBA and IgLON5 antibody IFA titer are performed at an additional charge.
If IFA pattern suggests inositol 1,4,5-trisphosphate receptor (ITPR1) antibody, then ITPR1 CBA and ITPR1 antibody IFA titer are performed at an additional charge.
If 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 are performed at an additional charge.
If Kelch-like protein 11 (KLHL11) CBA is reactive, then KLHL11 antibody IFA titer is performed at an additional charge.
See Movement Disorder Autoimmune Evaluation Algorithm-Spinal Fluid
Method Name
AGN1C, AMPHC, AMPIC, ANN1C, ANN2C, ANN3C, CRMC, DPPIC, DPPTC, GABIC, GL1IC, GL1TC, GRFIC, GRFTC, IG5IC, IG5TC, ITPIC, ITPTC, K11TC, NIFIC, NIFTC, NMDIC, PCA1C, PCA2C, PCTRC: Indirect Immunofluorescence Assay (IFA)
AINCC, AMPCC, CS2CC, DPPCC, GABCC, GL1CC, GRFCC, IG5CC, ITPCC, K11CC, LG1CC, NFHCC, NFLCC, NMDCC: Cell-Binding Assay (CBA)
CRMWC: Western Blot (WB)
AGNBC, AMIBC, AN1BC, AN2BC, PC1BC, PCTBC: Immunoblot (IB)
GD65C: Radioimmunoassay (RIA)
MDCI: Medical Interpretation
Reporting Name
Movement, Autoimm/Paraneo, CSFSpecimen Type
CSFSpecimen Minimum Volume
3.5 mL
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
CSF | Refrigerated (preferred) | 28 days | |
Frozen | 28 days | ||
Ambient | 72 hours |
Clinical Information
Autoimmune movement disorders encapsulate a large and diverse group of neurologic disorders occurring either in isolation or accompanying more diffuse autoimmune encephalitic illnesses.
The full range of movement phenomena has been described and, as they often occur in adults, many of the presentations can mimic neurodegenerative disorders, such as autoimmune chorea mimicking Huntington disease. Disorders may be ataxic, hypokinetic (parkinsonism), or hyperkinetic (myoclonus, chorea other dyskinetic disorders). Associated disorders may fall under the rubric of brainstem encephalitis.
The autoantibody targets are diverse and include neuronal surface proteins such as leucine-rich, glioma-inactivated 1 (LGI1), as well as antibodies reactive with intracellular antigens (such as Purkinje cell cytoplasmic antibody-1 [PCA-1]) that are markers of a central nervous system process mediated by CD8+ cytotoxic T cells.
In some instances (such as PCA-1 autoimmunity), antibodies detected in serum and cerebrospinal fluid can be indicative of a paraneoplastic cause and may direct the cancer search. In other instances (such as 65 kDa isoform of glutamic acid decarboxylase [GAD65] autoimmunity), a paraneoplastic cause is very unlikely, and early treatment with immunotherapy may promote improvement or recovery.
Reference Values
Test ID |
Reporting name |
Methodology* |
Reference value |
MDCI |
Movement Disorder Interp, CSF |
Medical interpretation |
N/A |
AMPHC |
Amphiphysin Ab, CSF |
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 |
CS2CC |
CASPR2-IgG CBA, CSF |
CBA |
Negative |
CRMWC |
CRMP-5-IgG Western Blot, CSF |
WB |
Negative |
CRMC |
CRMP-5-IgG, CSF |
IFA |
<1:2 |
DPPIC |
DPPX Ab IFA, CSF |
IFA |
Negative |
GD65C |
GAD65 Ab Assay, CSF |
RIA |
≤0.02 nmol/L Reference values apply to all ages. |
GRFIC |
GRAF1 IFA, CSF |
IFA |
Negative |
IG5IC |
IgLON5 IFA, CSF |
IFA |
Negative |
ITPIC |
ITPR1 IFA, CSF |
IFA |
Negative |
K11CC |
KLHL11 Ab CBA, CSF |
CBA |
Negative |
LG1CC |
LGI1-IgG CBA, CSF |
CBA |
Negative |
GL1IC |
mGluR1 Ab IFA, CSF |
IFA |
Negative |
NIFIC |
NIF IFA, CSF |
IFA |
Negative |
NMDCC |
NMDA-R Ab CBA, CSF |
CBA |
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 |
IB |
Negative |
AINCC |
Alpha Internexin CBA, CSF |
CBA |
Negative |
AMPIC |
AMPA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
AMPCC |
AMPA-R Ab CBA, CSF |
CBA |
Negative |
AMIBC |
Amphiphysin Immunoblot, CSF |
IB |
Negative |
AN1BC |
ANNA-1 Immunoblot, CSF |
IB |
Negative |
AN2BC |
ANNA-2 Immunoblot, CSF |
IB |
Negative |
DPPTC |
DPPX Ab IFA Titer, CSF |
IFA |
<1:2 |
DPPCC |
DPPX Ab CBA, CSF |
CBA |
Negative |
GABIC |
GABA-B-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
GABCC |
GABA-B-R Ab CBA, CSF |
CBA |
Negative |
GRFCC |
GRAF1 CBA, CSF |
CBA |
Negative |
GRFTC |
GRAF1 IFA Titer, CSF |
IFA |
<1:2 |
IG5CC |
IgLON5 CBA, CSF |
CBA |
Negative |
IG5TC |
IgLON5 IFA Titer, CSF |
IFA |
<1:2 |
ITPCC |
ITPR1 CBA, CSF |
CBA |
Negative |
ITPTC |
ITPR1 IFA Titer, CSF |
IFA |
<1:2 |
K11TC |
KLHL11 Ab IFA Titer, CSF |
IFA |
<1:2 |
GL1TC |
mGluR1 Ab IFA Titer, CSF |
IFA |
<1:2 |
GL1CC |
mGluR1 Ab CBA, CSF |
CBA |
Negative |
NFHCC |
NIF Heavy Chain CBA, CSF |
CBA |
Negative |
NIFTC |
NIF IFA Titer, CSF |
IFA |
<1:2 |
NFLCC |
NIF Light Chain CBA, CSF |
CBA |
Negative |
NMDIC |
NMDA-R Ab IF Titer Assay, CSF |
IFA |
<1:2 |
PC1BC |
PCA-1 Immunoblot, CSF |
IB |
Negative |
PCTBC |
PCA-Tr Immunoblot, CSF |
IB |
Negative |
*Methodology abbreviations:
Immunofluorescence assay (IFA)
Cell-binding assay (CBA)
Western blot (WB)
Radioimmunoassay (RIA)
Immunoblot (IB)
Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, 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."
Interpretation
A positive antibody result is consistent with a diagnosis of an autoimmune movement disorder.
A search for cancer may be indicated, depending on the antibody profile.
A trial of immune therapy may bring about improvement in neurological symptoms.
Clinical Reference
1. Honorat JA, McKeon A: Autoimmune Movement Disorders: a Clinical and Laboratory Approach. Curr Neurol Neurosci Rep. 2017 Jan;17(1):4 doi: 10.1007/s11910-017-0709-2
2. Dubey D, Wilson MR, Clarkson B, et.al:.Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis. JAMA Neurol. 2020 Nov 1;77(11):1420-1429. doi: 10.1001/jamaneurol.2020.2231
Day(s) Performed
Profile tests: Monday through Sunday; Reflex tests: Varies
Report Available
8 to 11 daysCPT Code Information
86255 x19
84182
86341
84182 AGNBC (if appropriate)
86255 AINCC (if appropriate)
86255 AMPCC (if appropriate)
86256 AMPIC (if appropriate)
84182 AMIBC (if appropriate)
84182 AN1BC (if appropriate)
84182 AN2BC (if appropriate)
86255 DPPCC (if appropriate)
86256 DPPTC (if appropriate)
86255 GABCC (if appropriate)
86256 GABIC (if appropriate)
86255 GRFCC (if appropriate)
86256 GRFTC (if appropriate)
86255 IG5CC (if appropriate)
86256 IG5TC (if appropriate)
86255 ITPCC (if appropriate)
86256 ITPTC (if appropriate)
86256-K11TC (if appropriate)
86255 GL1CC (if appropriate)
86256 GL1TC (if appropriate)
86255 NFHCC (if appropriate)
86256 NIFTC (if appropriate)
86255 NFLCC (if appropriate)
86256 NMDIC (if appropriate)
84182 PC1BC (if appropriate)
84182 PCTBC (if appropriate)
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
MDC2 | Movement, Autoimm/Paraneo, CSF | 94712-7 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
61513 | NMDA-R Ab CBA, CSF | 93502-3 |
606959 | GRAF1 IFA, CSF | 96473-4 |
606965 | NIF IFA, CSF | 96490-8 |
606947 | IgLON5 IFA, CSF | 96479-1 |
610580 | KLHL11 Ab CBA, CSF | 99073-9 |
64280 | LGI1-IgG CBA, CSF | 94288-8 |
64282 | CASPR2-IgG CBA, CSF | 94286-2 |
64929 | DPPX Ab IFA, CSF | 82989-5 |
64927 | mGluR1 Ab IFA, CSF | 94361-3 |
601997 | Movement Disorder Interp, CSF | 69048-7 |
606953 | ITPR1 IFA, CSF | 96467-6 |
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 |
21650 | CRMP-5-IgG, CSF | 94706-9 |
3988 | PCA-1, CSF | 94363-9 |
21632 | PCA-2, CSF | 94364-7 |
21631 | PCA-Tr, CSF | 94362-1 |
21747 | CRMP-5-IgG Western Blot, CSF | 53707-6 |
21702 | GAD65 Ab Assay, CSF | 94359-7 |
36429 | Reflex Added | 77202-0 |
Special Instructions
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.mml-Movement-Disorders