Test Id : PCDEC

If the indirect immunofluorescence assay (IFA) pattern suggests antineuronal nuclear antibodies (ANNA)-1, then ANNA-1 immunoblot, ANNA-1 IFA titer and ANNA-2 immunoblot will be performed at an additional charge.

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

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

 If gamma-aminobutyric acid B receptor (GABA-B-R) antibody CBA is positive, then GABA-B-R IFA titer will be performed at an additional charge. 

If the IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX IFA titer will be performed at an additional charge. 

If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 antibody CBA and mGluR1 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 IFA titer will be performed at an additional charge. 

If the neuromyelitis optica/aquaporin-4-IgG (NMO/AQP4-IgG) fluorescence-activated cell sorting (FACS) screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay 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.

For more information, see the following:

Pediatric Autoimmune Encephalopathy/Central Nervous System Disorders Evaluation Algorithm-Spinal Fluid

Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm

Multiple neuroimmunology profile tests are available. For testing that is performed with each profile, see Autoimmune Neurology Antibody Matrix.

Provide the following information:

-Relevant clinical information

-Ordering provider name, phone number, mailing address, and e-mail address

Container/Tube: Sterile vial

Preferred: Vial number 1

Acceptable: Any vial

Specimen Volume: 4 mL

• Pediatric Autoimmune Encephalopathy/CNS Disorders Evaluation Algorithm-Spinal Fluid
• Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm

If not ordering electronically, complete, print, and send a Neurology Specialty Testing Client Test Request (T732) with the specimen.

2 mL

Evaluating children with autoimmune central nervous system disorders using spinal fluid specimens

If the indirect immunofluorescence assay (IFA) pattern suggests antineuronal nuclear antibodies (ANNA)-1, then ANNA-1 immunoblot, ANNA-1 IFA titer and ANNA-2 immunoblot will be performed at an additional charge.

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

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

 If gamma-aminobutyric acid B receptor (GABA-B-R) antibody CBA is positive, then GABA-B-R IFA titer will be performed at an additional charge. 

If the IFA pattern suggests dipeptidyl-peptidase-like protein-6 (DPPX) antibody, then DPPX antibody CBA and DPPX IFA titer will be performed at an additional charge. 

If the IFA pattern suggests metabotropic glutamate receptor 1 (mGluR1) antibody, then mGluR1 antibody CBA and mGluR1 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 IFA titer will be performed at an additional charge. 

If the neuromyelitis optica/aquaporin-4-IgG (NMO/AQP4-IgG) fluorescence-activated cell sorting (FACS) screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay 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.

For more information, see the following:

Pediatric Autoimmune Encephalopathy/Central Nervous System Disorders Evaluation Algorithm-Spinal Fluid

Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm

Autoimmune encephalitis and myelitis is increasingly recognized as a cause of central nervous system disease in children and adolescents. N-methyl-D-aspartate receptor antibody (NMDA-R) encephalitis and myelin oligodendrocyte glycoprotein (MOG) autoimmunity are most common, although other entities, including aquaporin-4 autoimmunity, contactin-associated protein-like 2 (CASPR2) autoimmunity, autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, and paraneoplastic encephalomyelopathies, may also occur in children.

Reflex Information:

*Methodology abbreviations:

Immunofluorescence assay (IFA)

Cell-binding assay (CBA)

Fluorescence activated cell sorting assay (FACS)

Radioimmunoassay (RIA)

Immunoblot (IB)

*Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1 or PCA-Tr may be reported as "unclassified antineuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."

This profile is consistent with an autoimmune central nervous system disorder.

Negative results do not exclude a diagnosis of an autoimmune central nervous system disorder.

1. Dubey D, Pittock SJ, Krecke KN, et al: Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 2019 Mar 1;76(3):301-309 doi: 10.1001/jamaneurol.2018.4053

2. McKeon A, Lennon VA, Lotze T, et al: CNS aquaporin-4 autoimmunity in children. Neurology. 2008 Jul 8;71(2):93-100

3. Dubey D, Hinson SR, Jolliffe EA, et al: Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year. J Neuroimmunol. 2018 Aug 15;321:157-163

4. Philipps G, Alisanski SB, Pranzatelli M, et al: Purkinje cell cytoplasmic antibody type 1 (anti-Yo) autoimmunity in a child with Down syndrome. JAMA Neurol. 2014 Mar;71(3):347-349

5. Lopez-Chiriboga AS, Klein C, Zekeridou A, et al: LGI1 and CASPR2 neurological autoimmunity in children. Ann Neurol. 2018 Sep;84(3):473-480

6. Lopez-Chiriboga AS, Majed M, Fryer J, et al: Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol. 2018 Nov 1;75(11):1355-1363

7. Clardy SL, Lennon VA, Dalmau J: Childhood onset of stiff-man syndrome. JAMA Neurol. 2013 Dec;70(12):1531-1536

8. Banwell B, Tenembaum S, Lennon VA, et al: Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Neurology. 2008 Jan 29;70(5):344-352

• Pediatric Autoimmune Encephalopathy/CNS Disorders Evaluation Algorithm-Spinal Fluid
• Pediatric Autoimmune Central Nervous System Demyelinating Disease Diagnostic Algorithm

Cell-Binding Assay:

Patient specimen is applied to a composite slide containing transfected and nontransfected HEK-293 cells. After incubation and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the presence of patient IgG binding.(Package insert: IIFT: Neurology Mosaics, Instructions for the indirect immunofluorescence test. EUROIMMUN; FA_112d-1_A_UK_C13, 02/2019)

Fluorescence-Activated Cell Sorting Assay:

Human embryonic kidney cells (HEK 293) are transfected transiently with a plasmid (pIRES2- Aequorea coerulescens green fluorescent protein [AcGFP]) encoding both green fluorescent protein (AcGFP) and AQP4-M1. After 36 hours, a mixed population of cells (transfected expressing AQP4 or MOG on the surface and AcGFP in the cytoplasm and nontransfected lacking AQP4 or MOG and AcGFP) are lifted and resuspended in live cell-binding buffer. Cells are incubated with patient serum and an AlexaFluor 647-labeled secondary antibody is added. Two populations are gated on the basis of AcGFP expression: positive (high AQP4 or MOG expression) and negative (low or no AQP4 or MOG expression). Positivity is based on the ratio (Positive >2.0) of the average median fluorescence intensity (MFI) of each cell population (MFI GFP positive:MFI GFP negative).(Unpublished Mayo method)

Indirect Immunofluorescence Assay:

The patient's sample is tested by a standardized immunofluorescence assay that uses a composite frozen section of mouse cerebellum, kidney, and gut tissues. After incubation with sample and washing, fluorescein-conjugated goat-antihuman IgG is applied. Neuron-specific autoantibodies are identified by their characteristic fluorescence staining patterns. Samples that are scored positive for any neuronal nuclear or cytoplasmic autoantibody are titrated to an endpoint. Interference by coexisting non-neuron-specific autoantibodies can usually be eliminated by serologic absorption.(Honorat JA, Komorowski L, Josephs KA, et al: IgLON5 antibody: neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017 Jul 18;4(5):e385. doi: 10.1212/NXI.0000000000000385)

Radioimmunoassay:

Duplicate aliquots of patient specimen are incubated with I(125)-labeled antigen. Immune complexes, formed by adding secondary (goat)-antihuman immunoglobulin, are pelleted by centrifugation and washed. Gamma emission from the washed pellet is counted, and mean counts per minute (cpm) are compared with results yielded by high-positive and -negative control sera. Specimens yielding cpm higher than the background cpm yielded by normal human specimen are retested to confirm positivity and titrated as necessary to obtain a value in the linear range of the assay. The antigen binding capacity (nmol per liter) is calculated from the cpm precipitated at a dilution yielding a linear range value.(Griesmann GE, Kryzer TJ, Lennon VA: Autoantibody profiles of myasthenia gravis and Lambert-Eaton myasthenic syndrome. In: Rose NR, Hamilton RG, et al, eds. Manual of Clinical and Laboratory Immunology. 6th ed ASM Press; 2002:1005-1012; Walikonis JE, Lennon VA: Radioimmunoassay for glutamic acid decarboxylase [GAD65] autoantibodies as a diagnostic aid for stiff-man syndrome and a correlate of susceptibility to type 1 diabetes mellitus. Mayo Clin Proc 1998 Dec;73[12]:1161-1166; Jones AL, Flanagan EP, Pittock SJ, et al: Responses to and Outcomes of Treatment of Autoimmune Cerebellar Ataxia in Adults. JAMA Neurol 2015 Nov;72[11]:1304-1312. doi: 10.1001/jamaneurol.2015.2378)

Immunoblot:

All steps are performed at ambient temperature (18-28 degrees C) utilizing the EUROBlot One instrument. 

Diluted patient serum (1:101) is added to test strips (strips containing recombinant antigen manufactured and purified using biochemical methods) in individual channels and incubated for 30 minutes. Positive serums will bind to the purified recombinant antigen and negative serums will not bind. Strips are washed to remove unbound antibodies and then incubated with antihuman IgG antibodies (alkaline phosphatase-labeled) and incubated for 30 minutes. The strips are again washed to remove unbound antihuman IgG antibodies and nitroblue tetrazolium chloride/5-bromo-4-chloro-3-indolyl phosphate (NBT/BCIP) substrate is added. Alkaline phosphatase enzyme converts the soluble substrate into a colored insoluble product on the membrane to produce a black band. Strips are digitized via picture capture on the EUROBlot One instrument and evaluated with the EUROLineScan software. (O'Connor K, Waters P, Komorowski L, et al: GABAA receptor autoimmunity: A multicenter experience. Neurol Neuroimmunol Neuroinflamm. 2019 Apr 4;6[3]:e552 doi: 10.1212/NXI.0000000000000552)

Profile tests: Monday through Sunday; Reflex tests: Varies

• Authorized users can sign in to Test Prices for detailed fee information.
• Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
• Prospective clients should contact their Regional Manager. For assistance, contact Customer Service.

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.

86255 x 10

86341

86053

84182 AN1BC (if appropriate)

86256 AN1TC (if appropriate)

84182 AN2BC (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 NCDCC (if appropriate)

86256 NCDTC (if appropriate)

86256 NMDIC (if appropriate)

86053 NMOTC (if appropriate)

84182 PCTBC (if appropriate)

86256 PCTTC (if appropriate)