Dementia, Autoimmune/Paraneoplastic Evaluation, CSF (Sendout)

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General Information

Lab Name
Lab Code
993
External Test Id
DMC2
Description

Useful For: Investigating new onset dementia and cognitive impairment plus 1 or more of the following accompaniments using cerebrospinal fluid specimens:

  • Rapid onset and progression
  • Fluctuating course
  • Psychiatric accompaniments (psychosis, hallucinations)
  • Movement disorder (myoclonus, tremor, dyskinesias
  • Headache
  • Autoimmune stigmata (personal history or family history or signs of diabetes mellitus, thyroid disorder, vitiligo, poliosis [premature graying], myasthenia gravis, rheumatoid arthritis, systemic lupus erythematosus)
  • Smoking history (20+ pack years) or other cancer risk factors
  • History of cancer
  • Inflammatory cerebrospinal fluid
  • Neuroimaging findings atypical for degenerative etiology

Refer to Autoimmune Encephalitis Testing Recommendations for additional ordering guidance.

Test Algorithm: Reflex testing may be performed at additional charge. Refer to the Mayo algorithm below for a full list of tests and potential reflex tests included in this panel.

Synonyms
AMPA-R Antibody CBA, Amphiphysin Antibody, ANNA-1, ANNA-2, ANNA-3, Anti-Glial Nuclear Antibody Type 1, Anti-Neuronal Nuclear Antibody Type 1, Anti-Neuronal Nuclear Antibody Type 2, Anti-Neuronal Nuclear Antibody Type 3, CASPR2 IgG, Cognitive decline, Cognitive impairment, Collapsin Response-Mediator Protein-5 IgG, Contactin-Associated Protein-Like-2 (CASPR2) IgG, CRMP-5 IgG, DEMEC, Dementia Autoimmune Paraneoplastic Evaluation, Dipeptidyl aminopeptidase-like protein 6, Dipeptidyl-peptidase-like protein-6 (DPPX) Antibody, DMC2, DPPX, GABA-B-R Antibody CBA, Gamma Aminobutyric Acid Receptor Type B, GFAP IFA, Glial Fibrillary Acidic Protein Antibody, Glutamic Acid Decarboxylase (GAD65) Antibody, IgLON Family Member 5 Antibody, IgLON5, Leucine-Rich Glioma Inactivated Protein-1 IgG, LGI1 IgG, metabotropic glutamate receptor 1, mGluR1, N-methyl-D-Aspartate Receptor Antibody, Neurochondrin IFA, Neuronal Intermediate Filament Antibody, NIF, NMDA-R Antibody CBA, PCA-2, PCA-Tr, PDE10A Antibody, Phosphodiesterase 10A Antibody, Purkinje Cell Cytoplasmic Antibody Type 2, Purkinje Cell Cytoplasmic Antibody Type Tr, TRIM46 Antibody, Tripartite Motif-containing Protein 46 Antibody

Interpretation

Method

Panel and reflex tests include the following methods: Indirect Immunofluorescence Assay (IFA), Cell Binding Assay (CBA), Radioimmunoassay (RIA), Immunoblot (IB), Western Blot (WB).

Ref. Range Notes

Reference Values:

Alpha-amino-3-hydroxy-5-methyl-4-isoxazole Propionic Acid Receptor (AMPA-R) Antibody CBA, CSF: Negative
Amphiphysin Antibody, CSF: Negative
Anti-Glial Nuclear Antibody, Type 1 (AGNA-1), CSF: Negative
Anti-Neuronal Nuclear Antibody, Type 1 (ANNA-1), CSF: Negative
Anti-Neuronal Nuclear Antibody, Type 2 (ANNA-2), CSF: Negative
Anti-Neuronal Nuclear Antibody, Type 3 (ANNA-3), CSF: Negative
Contactin-Associated Protein-Like-2 (CASPR2) IgG Antibody CBA, CSF: Negative
Collapsin Response-Mediator Protein-5 (CRMP-5) IgG Antibody, CSF: Negative
Dipeptidyl Aminopeptidase-Like Protein 6 (DPPX) Antibody CBA, CSF: Negative
Gamma Aminobutyric Acid Receptor Type B (GABA-B-R) Antibody CBA, CSF: Negative
Glutamic Acid Decarboxylase (GAD65) Antibody Assay, CSF: < or =0.02 nmol/L
Glial Fibrillary Acidic Protein (GFAP) Antibody IFA, CSF: Negative
IgLON Family Member 5 (IgLON5) Antibody CBA, CSF: Negative
Leucine-Rich Glioma Inactivated Protein-1 IgG (LGI1) Antibody CBA, CSF: Negative
Neurochondrin IFA, CSF: Negative
Metabotropic Glutamate Receptor 1 (mGluR1) Antibody IFA, CSF: Negative
Neuronal Intermediate Filament (NIF) Antibody IFA, CSF: Negative
N-methyl-D-Aspartate Receptor (NMDA-R) Antibody CBA, CSF: Negative
Purkinje Cell Cytoplasmc Antibody Type Tr, CSF: Negative
Purkinje Cell Cytoplasmic Antibody Type 2, CSF: Negative
Phosphodiesterase 10A (PDE10A) Antibody IFA, CSF: Negative
Tripartite Motif-containing Protein 46 (TRIM46) Antibody IFA, CSF: Negative

Note: Includes reference values for panel tests only.

Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, CRMP-5-IgG, PCA-2, or PCA-Tr may be reported as "unclassified anti-neuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

CRMP-5 Note: CRMP-5 titers lower than 1:2 are detectable by recombinant CRMP-5 Western blot analysis. CRMP-5 Western blot analysis will be done on request on stored spinal fluid (held 4 weeks). This supplemental testing is recommended in cases of chorea, vision loss, cranial neuropathy, and myelopathy.

Intepretation: Antibodies specific for neuronal, glial, or muscle proteins are valuable serological markers of autoimmune epilepsy and of a patient's immune response to cancer. These autoantibodies are not found in healthy subjects and are usually accompanied by subacute neurological symptoms and signs. It is not uncommon for more than 1 of the following autoantibodies to be detected in patients with autoimmune dementia:

  • Plasma membrane antibodies (N-methyl-D-aspartate [NMDA] receptor; 2-amino-3-[5-methyl-3-oxo-1,2- oxazol-4-yl] propanoic acid [AMPA] receptor; gamma-amino butyric acid [GABA]-B receptor). These autoantibodies are all potential effectors of dysfunction.
  • Neuronal nuclear autoantibody type 1 (ANNA-1) or type 3 (ANNA-3)
  • Neuronal or muscle cytoplasmic antibodies (amphiphysin, Purkinje cell antibody-type 2 [PCA-2], collapsin response-mediator protein-5 neuronal [CRMP-5-IgG], or glutamic acid decarboxylase [GAD65] antibody).
Interferences and Limitations

Cautions:

Negative results do not exclude autoimmune dementia or cancer.

This evaluation does not detect Ma1 or Ma2 antibodies (also known as MaTa). Ma2 antibody has been described in patients with brainstem and limbic encephalitis in the context of testicular germ cell neoplasms. Scrotal ultrasound is advisable in men who present with unexplained subacute encephalitis.

Guidelines

Ordering & Collection

Specimen Type
CSF
Collection

Collect 4mL CSF in a sterile vial

Approval Required
LMR approval is not required.
Handling Instructions

Outside Laboratories: Refrigerate 4 mL (2 mL minimum) CSF in a sterile vial.

Stability: Refrigerated (Preferred): 28 days; Frozen: 28 days; Ambient: 72 hours.

Reject Due To: Gross hemolysis, lipemia, or icterus.

Quantity
requested: 4 mL CSF
minimum: 2 mL CSF

Processing

Processing

Refrigerate CSF in a sterile vial.

Login: SEND1-;REFRIG

  • RSNDT1: MAYO
  • RSTYP1: CSF
  • RSTRQ1: ;Dementia Autoimmune Evaluation, CSF (Mayo Test DMC2)

Sendouts:

  • Order Mayo test: DMC2.

Stability: Refrigerated (Preferred): 28 days; Frozen: 28 days; Ambient: 72 hours.

Reject Due To: Gross hemolysis, lipemia, or icterus.

Performance

LIS Dept Code
Performing Location(s)
Sendout Mayo Clinic Laboratories
800-533-1710

200 First Street Southwest
Rochester, MN 55901
Frequency
Performed: Monday through Sunday (Panel Tests). Report Available: 8-12 days from sample receipt at the performing lab.
Available STAT?
No

Billing & Coding

CPT codes
Billing Comments

CPTs: 86255x21, 86341x1

  • Note: Includes panel tests only. Reflex testing may be added at additional charge.
LOINC