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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


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, CSF

Specimen Type

CSF

Specimen 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 days

CPT 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

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.
Mayo Clinic Laboratories | Neurology Catalog Additional Information:

mml-Movement-Disorders