Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum - Mayo Clinic Laboratories

Ordering Guidance

Multiple neuroimmunology profile tests are available. 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:

1. For optimal antibody detection, specimen collection is recommended prior to initiation of immunosuppressant medication or intravenous immunoglobulin (IVIg) treatment.

2. This test should not be requested in patients who have recently received radioisotopes, therapeutically or diagnostically, because of potential assay interference. The specific waiting period before specimen collection will depend on the isotope administered, the dose given, and the clearance rate in the individual patient. Specimens will be screened for radioactivity prior to analysis. Radioactive specimens received in the laboratory will be held 1 week and assayed if sufficiently decayed or canceled if radioactivity remains.

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic 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 in serum specimens

Profile Information

Test ID	Reporting Name	Available Separately	Always Performed
MDSI	Movement Disorder Interp, S	No	Yes
AMPHS	Amphiphysin Ab, S	No	Yes
AGN1S	Anti-Glial Nuclear Ab, Type 1	No	Yes
ANN1S	Anti-Neuronal Nuclear Ab, Type 1	No	Yes
ANN2S	Anti-Neuronal Nuclear Ab, Type 2	No	Yes
ANN3S	Anti-Neuronal Nuclear Ab, Type 3	No	Yes
CS2CS	CASPR2-IgG CBA, S	No	Yes
CRMS	CRMP-5-IgG, S	No	Yes
CRMWS	CRMP-5-IgG Western Blot, S	Yes	Yes
DPPIS	DPPX Ab IFA, S	No	Yes
GD65S	GAD65 Ab Assay, S	Yes	Yes
GRFIS	GRAF1 IFA, S	No	Yes
IG5IS	IgLON5 IFA, S	No	Yes
ITPIS	ITPR1 IFA, S	No	Yes
K11CS	KLHL11 Ab CBA, S	Yes	Yes
LG1CS	LGI1-IgG CBA, S	No	Yes
GL1IS	mGluR1 Ab IFA, S	No	Yes
NIFIS	NIF IFA, S	No	Yes
NMDCS	NMDA-R Ab CBA, S	No	Yes
CCPQ	P/Q-Type Calcium Channel Ab	No	Yes
PCABP	Purkinje Cell Cytoplasmic Ab Type 1	Yes	Yes
PCAB2	Purkinje Cell Cytoplasmic Ab Type 2	Yes	Yes
PCATR	Purkinje Cell Cytoplasmic Ab Type Tr	No	Yes

Reflex Tests

Test ID	Reporting Name	Available Separately	Always Performed
AGNBS	AGNA-1 Immunoblot, S	No	No
AINCS	Alpha Internexin CBA, S	No	No
AMPCS	AMPA-R Ab CBA, S	No	No
AMPIS	AMPA-R Ab IF Titer Assay, S	No	No
AMIBS	Amphiphysin Immunoblot, S	No	No
AN1BS	ANNA-1 Immunoblot, S	No	No
AN2BS	ANNA-2 Immunoblot, S	No	No
DPPCS	DPPX Ab CBA, S	No	No
DPPTS	DPPX Ab IFA Titer, S	No	No
GABCS	GABA-B-R Ab CBA, S	No	No
GABIS	GABA-B-R Ab IF Titer Assay, S	No	No
GRFCS	GRAF1 CBA, S	No	No
GRFTS	GRAF1 IFA Titer, S	No	No
IG5CS	IgLON5 CBA, S	No	No
IG5TS	IgLON5 IFA Titer, S	No	No
ITPCS	ITPR1 CBA, S	No	No
ITPTS	ITPR1 IFA Titer, S	No	No
GL1CS	mGluR1 Ab CBA, S	No	No
GL1TS	mGluR1 Ab IFA Titer, S	No	No
NFHCS	NIF Heavy Chain CBA, S	No	No
NIFTS	NIF IFA Titer, S	No	No
NFLCS	NIF Light Chain CBA, S	No	No
NMDIS	NMDA-R Ab IF Titer Assay, S	No	No
PC1BS	PCA-1 Immunoblot, S	No	No
PCTBS	PCA-Tr Immunoblot, S	No	No
K11TS	KLHL11 Ab IFA Titer, S	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-1 (AGNA)-1, then AGNA-1 immunoblot is performed at an additional charge.

If IFA pattern suggests antineuronal nuclear antibodies (ANNA)-1, then ANNA-1 immunoblot is performed at an additional charge.

If IFA pattern suggests ANNA-2 antibody, 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 antibody, then PCA-Tr immunoblot is performed at an additional charge.

If IFA pattern suggests IgLON5 antibody, then IgLON5 cell-binding assay (CBA) and IgLON5 antibody IFA titer are 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 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 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 antibody (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 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.

For more information see Movement Disorder Autoimmune Evaluation Algorithm-Serum.

Method Name

AGN1S, AMPHS, AMPIS, ANN1S, ANN2S, ANN3S, CRMS, DPPIS, DPPTS, GABIS, GL1IS, GL1TS, GRFIS, GRFTS, IG5IS, IG5TS, ITPIS, ITPTS, K11TS, NIFIS, NIFTS, NMDIS, PCAB2, PCABP, PCATR: Indirect Immunofluorescence Assay (IFA)

AINCS, AMPCS, CS2CS, DPPCS, GABCS, GL1CS, GRFCS, IG5CS, ITPCS, K11CS, LG1CS, NFHCS, NFLCS, NMDCS: Cell Binding Assay (CBA)

CRMWS: Western Blot (WB)

AGNBS, AMIBS, AN1BS, AN2BS, PC1BS, PCTBS: Immunoblot (IB)

CCPQ, GD65S: Radioimmunoassay (RIA)

Reporting Name

Movement, Autoimm/Paraneo, S

Specimen Type

Serum

Specimen Minimum Volume

3 mL

Specimen Stability Information

Specimen Type	Temperature	Time
Serum	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 65kDa 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
MDSI	Movement Disorder Interp, S	Medical interpretation	N/A
AMPHS	Amphiphysin Ab, S	IFA	<1:240
AGN1S	Anti-Glial Nuclear Ab, Type 1	IFA	<1:240
ANN1S	Anti-Neuronal Nuclear Ab, Type 1	IFA	<1:240
ANN2S	Anti-Neuronal Nuclear Ab, Type 2	IFA	<1:240
ANN3S	Anti-Neuronal Nuclear Ab, Type 3	IFA	<1:240
CS2CS	CASPR2-IgG CBA, S	CBA	Negative
CRMS	CRMP-5-IgG, S	IFA	<1:240
CRMWS	CRMP-5-IgG Western Blot, S	WB	Negative
DPPIS	DPPX Ab IFA, S	IFA	Negative
GD65S	GAD65 Ab Assay, S	RIA	≤0.02 nmol/L Reference values apply to all ages.
GRFIS	GRAF1 IFA, S	IFA	Negative
IG5IS	IgLON5 IFA, S	IFA	Negative
ITPIS	ITPR1 IFA, S	IFA	Negative
K11CS	KLHL11 Ab CBA, S	CBA	Negative
LG1CS	LGI1-IgG CBA, S	CBA	Negative
GL1IS	mGluR1 Ab IFA, S	IFA	Negative
NIFIS	NIF IFA, S	IFA	Negative
NMDCS	NMDA-R Ab CBA, S	CBA	Negative
CCPQ	P/Q-Type Calcium Channel Ab	RIA	≤0.02 nmol/L
PCABP	Purkinje Cell Cytoplasmic Ab Type 1	IFA	<1:240
PCAB2	Purkinje Cell Cytoplasmic Ab Type 2	IFA	<1:240
PCATR	Purkinje Cell Cytoplasmic Ab Type Tr	IFA	<1:240

Reflex Information:

Test ID	Reporting Name	Methodology*	Reference Value
AGNBS	AGNA-1 Immunoblot, S	IB	Negative
AINCS	Alpha Internexin CBA, S	CBA	Negative
AMPIS	AMPA-R Ab IF Titer Assay, S	IFA	<1:120
AMPCS	AMPA-R Ab CBA, S	CBA	Negative
AMIBS	Amphiphysin Immunoblot, S	IB	Negative
AN1BS	ANNA-1 Immunoblot, S	IB	Negative
AN2BS	ANNA-2 Immunoblot, S	IB	Negative
DPPCS	DPPX Ab CBA, S	CBA	Negative
DPPTS	DPPX Ab IFA Titer, S	IFA	<1:240
GABCS	GABA-B-R Ab CBA, S	CBA	Negative
GABIS	GABA-B-R Ab IF Titer Assay, S	IFA	<1:120
GRFCS	GRAF1 CBA, S	CBA	Negative
GRFTS	GRAF1 IFA Titer, S	IFA	<1:240
IG5CS	IgLON5 CBA, S	CBA	Negative
IG5TS	IgLON5 IFA Titer, S	IFA	<1:240
ITPCS	ITPR1 CBA, S	CBA	Negative
ITPTS	ITPR1 IFA Titer, S	IFA	<1:240
K11TS	KLHL11 Ab IFA Titer, S	IFA	<1:240
GL1CS	mGluR1 Ab CBA, S	CBA	Negative
GL1TS	mGluR1 Ab IFA Titer, S	IFA	<1:240
NFHCS	NIF Heavy Chain CBA, S	CBA	Negative
NIFTS	NIF IFA Titer, S	IFA	<1:240
NFLCS	NIF Light Chain CBA, S	CBA	Negative
NMDIS	NMDA-R Ab IF Titer Assay, S	IFA	<1:120
PC1BS	PCA-1 Immunoblot, S	IB	Negative
PCTBS	PCA-Tr Immunoblot, S	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

10 to 13 days

CPT Code Information

86596

86255 x 19

84182

86341

84182-AGNBS (if appropriate)

86255-AINCS (if appropriate)

86255-AMPCS (if appropriate)

86256-AMPIS (if appropriate)

84182-AMIBS (if appropriate)

84182-AN1BS (if appropriate)

84182-AN2BS (if appropriate)

86255-DPPCS (if appropriate)

86256-DPPTS (if appropriate)

86255-GABCS (if appropriate)

86256-GABIS (if appropriate)

86255-GRFCS (if appropriate)

86256-GRFTS (if appropriate)

86255-IG5CS (if appropriate)

86256-IG5TS (if appropriate)

86255-ITPCS (if appropriate)

86256-ITPTS (if appropriate)

86256-K11TS (if appropriate)

86255-GL1CS (if appropriate)

86256-GL1TS (if appropriate)

86255-NFHCS (if appropriate)

86256-NIFTS (if appropriate)

86255-NFLCS (if appropriate)

86256-NMDIS (if appropriate)

84182-PC1BS (if appropriate)

84182-PCTBS (if appropriate)

LOINC Code Information

Test ID	Test Order Name	Order LOINC Value
MDS2	Movement, Autoimm/Paraneo, S	94701-0

Result ID	Test Result Name	Result LOINC Value
61516	NMDA-R Ab CBA, S	93503-1
64279	LGI1-IgG CBA, S	94287-0
64281	CASPR2-IgG CBA, S	94285-4
64930	DPPX Ab IFA, S	82976-2
64928	mGluR1 Ab IFA, S	94347-2
601998	Movement Disorder Interp, S	69048-7
606946	IgLON5 IFA, S	96476-7
606952	ITPR1 IFA, S	96464-3
606964	NIF IFA, S	96486-6
606958	GRAF1 IFA, S	96471-8
610581	KLHL11 Ab CBA, S	99072-1
80776	ANNA-2, S	94343-1
83137	ANNA-3, S	94344-9
81185	P/Q-Type Calcium Channel Ab	94349-8
83077	CRMP-5-IgG, S	94815-8
83107	CRMP-5-IgG Western Blot, S	47401-5
81596	GAD65 Ab Assay, S	94345-6
83138	PCA-2, S	94351-4
9477	PCA-1, S	94350-6
83076	PCA-Tr, S	94352-2
89080	AGNA-1, S	94341-5
81722	Amphiphysin Ab, S	94340-7
80150	ANNA-1, S	94342-3
36349	Reflex Added	77202-0

Special Instructions

Movement Disorder Autoimmune Evaluation Algorithm-Serum

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

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Test ID: MDS2 Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum