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
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
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

Special Instructions

Movement Disorder Autoimmune 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