Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid - Mayo Clinic Laboratories

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
AMPCC	AMPA-R Ab CBA, 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
CS2CC	CASPR2-IgG CBA, 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
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
NCDIC	Neurochondrin 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
SP5IC	Septin-5 IFA, CSF	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
AMPIC	AMPA-R Ab IF Titer Assay, 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
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
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
GFACC	GFAP CBA, CSF	No	No
GFATC	GFAP IFA 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
PCTTC	PCA-Tr Titer, CSF	No	No
SP5CC	Septin-5 CBA, CSF	No	No
SP5TC	Septin-5 IFA Titer, CSF	No	No
SP7CC	Septin-7 CBA, CSF	No	No
SP7TC	Septin-7 IFA Titer, CSF	No	No
AGNTC	AGNA-1 Titer, CSF	No	No
K11TC	KLHL11 Ab IFA Titer, CSF	No	No

Testing Algorithm

If the immunofluorescence assay (IFA) patterns suggest amphiphysin antibody, then amphiphysin immunoblot and amphiphysin IFA titer will be performed at an additional charge.

If the IFA pattern suggests antiglial nuclear antibody (AGNA)-1, then AGNA-1 immunoblot (IB) and AGNA-1 IFA titer will be performed at an additional charge.

If the IFA pattern suggests antineuronal nuclear antibody type 1 (ANNA-1), then ANNA-1 IB, ANNA-1 IFA titer, and ANNA-2 IB will be performed at an additional charge.

If the IFA pattern suggests ANNA-2, then ANNA-2 IB, ANNA-2 IFA titer, and ANNA-1 IB 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 AP3B2 cell-binding assay (CBA) and AP3B2 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 will be performed at an additional charge.

If the IFA pattern suggests Purkinje cytoplasmic antibody type 1 (PCA-1), then PCA-1 IB and PCA-1 IFA titer will be performed at an additional charge.

If the IFA pattern suggests PCA-Tr, then PCA-Tr IB and PCA-Tr 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 alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA)-receptor CBA is positive, then AMPA-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 CBA and DPPX antibody IFA titer will be performed at an additional charge.

If gamma-aminobutyric acid B (GABA-B)-receptor CBA is positive, then GABA-B-receptor 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 IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 CBA and mGluR1 antibody IFA titer will be performed at an additional charge.

If N-methyl-D-aspartate (NMDA)-receptor CBA is positive, then NMDA-receptor antibody IFA titer will be performed at an additional charge.

If the IFA pattern suggests GTPase regulator associated with focal adhesion kinase-1 (GRAF1) antibody, then GRAF1 CBA and GRAF1 antibody IFA titer will be performed at an additional charge.

If the IFA pattern suggests IgLON5 antibody, then IgLON5 CBA and IgLON5 antibody IFA titer will be performed at an additional charge.

If the IFA pattern suggests inositol 1,4,5-trisphosphate receptor (ITPR1) antibody, then ITPR1 CBA and ITPR1 antibody IFA titer 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 Kelch-like protein 11 (KLHL11) CBA is reactive, then KLHL11 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-5 antibody, then septin-5 CBA and septin-5 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 Movement Disorder Evaluation Algorithm-Spinal Fluid.

Method Name

GRFIC, GRFTC, K11TC, AGN1C, AGNTC, AMPIC, AMPHC, APHTC, ANN1C, AN1TC, ANN2C, AN2TC, ANN3C, AN3TC, APBIC, APBTC, CRMTC, DPPIC, DPPTC, GABIC, GFAIC, GFATC, IG5IC, IG5TC, ITPIC, ITPTC, GL1IC, GL1TC, NCDIC, NCDTC, NIFIC, NIFTC, NMDIC, PCA1C, PC1TC, PCA2C, PC2TC, PCTRC, PCTTC, SP5IC, SP5TC, SP7IC, SP7TC: Indirect Immunofluorescence Assay (IFA)

GRFCC, K11CC, AMPCC, APBCC, CS2CC, DPPCC, GABCC, GFACC, IG5CC, ITPCC, LG1CS, LG1CC, GL1CC, NCDCC, AINCC, NFLCC, NFHCC, NMDCC, SP5CC, SP7CC: 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
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
AMPCC	AMPA-R Ab CBA, CSF	CBA	Negative
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
CS2CC	CASPR2-IgG CBA, CSF	CBA	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.
GRFIC	GRAF1 IFA, CSF	IFA	Negative
GFAIC	GFAP 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
NCDIC	Neurochondrin 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	Negative
PCA1C	Purkinje Cell Cytoplasmic Ab Type 1	IFA	Negative
PCA2C	Purkinje Cell Cytoplasmic Ab Type 2	IFA	Negative
SP5IC	Septin-5 IFA, CSF	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
AMPIC	AMPA-R Ab IF Titer Assay, CSF	IFA	<1:2
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
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
GFACC	GFAP CBA, CSF	CBA	Negative
GFATC	GFAP IFA Titer, CSF	IFA	<1:2
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
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
NMDIC	NMDA-R Ab IF Titer Assay, CSF	IFA	<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
PCTTC	PCA-Tr Titer, CSF	IFA	<1:2
PCTBC	PCA-Tr Immunoblot, CSF	IB	Negative
SP5CC	Septin-5 CBA, CSF	CBA	Negative
SP5TC	Septin-5 IFA 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)

Western blot (WB)

Radioimmunoassay (RIA)

Immunoblot (IB)

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."

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 12 days

CPT Code Information

86255 x25

84182

86341

84182-AGNBC (if appropriate)

86256-GNTC (if appropriate)

86255-AINCC (if appropriate)

84182-AMIBC (if appropriate)

86256-AMPIC (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-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-NCDCC (if appropriate)

86256-NCDTC (if appropriate)

86255-NFHCC (if appropriate)

86255-NFLCC (if appropriate)

86256-NIFTC (if appropriate)

86256-NMDIC (if appropriate)

84182-PC1BC (if appropriate)

86256-PC1TC (if appropriate)

86256-PC2TC (if appropriate)

84182-PCTBC (if appropriate)

86256-PCTTC (if appropriate)

86255-SP5CC (if appropriate)

86256-SP5TC (if appropriate)

86255-SP7CC (if appropriate)

86256-SP7TC (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
61514	AMPA-R Ab CBA, CSF	93491-9
61515	GABA-B-R Ab CBA, CSF	93426-5
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
615862	AP3B2 IFA, CSF	In Process
615866	Neurochondrin IFA, CSF	101451-3
615874	Septin-7 IFA, CSF	In Process
615870	Septin-5 IFA, CSF	In Process
605156	GFAP IFA, CSF	94360-5
606953	ITPR1 IFA, CSF	96467-6
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
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
21631	PCA-Tr, CSF	94362-1
21747	CRMP-5-IgG Western Blot, CSF	53707-6
21702	GAD65 Ab Assay, CSF	94359-7
618902	IFA Notes	48767-8

Special Instructions

Autoimmune/Paraneoplastic Movement Disorder Evaluation Algorithm-Spinal Fluid

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.

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Test ID: MDC2 Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Spinal Fluid