Referred Tests
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| Email: | mcl@mayo.edu |
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Test ID: PCDEC
Pediatric Autoimmune Central Nervous System Disorders Evaluation, Spinal Fluid
Useful For 
Suggests clinical disorders or settings where the test may be helpful
Evaluating children with autoimmune central nervous system disorders using spinal fluid specimens
Testing Algorithm Delineates situations when tests are added to the initial order. This includes reflex and additional tests.
If indirect immunofluorescence assay (IFA) patterns suggest ANNA-1 antibody, then ANNA-1 immunoblot and ANNA-2 immunoblot are performed at an additional charge.
If IFA patterns suggest ANNA-2 antibody, then ANNA-2 immunoblot, ANNA-1 immunoblot, and ANNA-2 antibody IFA are performed at an additional charged.
If IFA patterns suggest ANNA-3 antibody, then ANNA-3 IFA is performed at an additional charge.
If IFA patterns suggest PCA-1 antibody, then PCA-1 immunoblot and PCA-1 IFA are performed at an additional charge.
If IFA patterns suggest PCA-2 antibody, then PCA-2 IFA is performed at an additional charge.
If IFA patterns suggest PCA-Tr antibody, then PCA-Tr immunoblot is performed at an additional charge.
If IFA pattern suggests amphiphysin antibody, then amphiphysin immunoblot and amphiphysin antibody IFA titer are performed at an additional charge.
If IFA pattern suggests AGNA antibody, then AGNA immunoblot and AGNA antibody IFA titer are performed at an additional charge.
If IFA pattern suggests NMDA-R antibody, then NMDA-R IFA titer is performed at an additional charge.
If IFA pattern suggests AMPA-R antibody, then AMPA-R antibody cell-binding assay (CBA) and AMPA-R IFA are performed at an additional charge.
If IFA pattern suggests GABA-B-R antibody, then GABA-B-R IFA titer is performed at an additional charge.
If IFA pattern suggests DPPX antibody, then DPPX antibody CBA and DPPX IFA titer are performed at an additional charge.
If IFA pattern suggests mGluR1 antibody, then mGluR1antibody CBA and mGluR1 IFA titer are performed at an additional charge.
If IFA pattern suggests GFAP antibody, then GFAP antibody CBA and GFAP IFA titer are performed at an additional charge.
If NMO/AQP4-IgG FACS screen assay requires further investigation, then NMO/AQP4-IgG FACS titration assay is performed at an additional charge.
See Pediatric Autoimmune Central Nervous System Disorders Evaluation Algorithm-Spinal Fluid in Special Instructions.
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
Autoimmune encephalitis and myelitis is increasingly recognized as a cause of central nervous system (CNS) disease in children and teens. 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.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
| Test ID | Reporting Name | Methodology | Reference Value |
| PCCI | Peds Autoimmune CNS Interp, CSF | ||
| ANN1C | Anti-Neuronal Nuclear Ab, Type 1 | Indirect Immunofluorescence Assay (IFA) | Negative at <1:2* |
| CS2CC | CASPR2-IgG CBA, CSF | Cell Binding Assay (CBA) | Negative |
| DPPIC | DPPX Ab IFA, CSF | IFA | Negative |
| GABCC | GABA-B-R Ab CBA, CSF | CBA | Negative |
| GD65C | GAD65 Ab Assay, CSF | Radioimmunoassay (RIA) | <0.02 nmol/L Reference values apply to all ages. |
| GFAIC | GFAP IFA, CSF | IFA | Negative |
| GL1IC | mGluR1 Ab IFA, CSF | IFA | Negative |
| LG1CC | LGI1-IgG CBA, CSF | CBA | Negative |
| NMDCC | NMDA-R Ab CBA, CSF | CBA | Negative |
| NMOFC | NMO/AQP4 FACS, CSF | Fluorescence-Activated Cell Sorting Assay (FACS) | Negative |
| PCTRC | Purkinje Cell Cytoplasmc Ab Type Tr | IFA | <1:2* |
Reflex Information:
| Test ID | Reporting Name | Methodology | Reference Value |
| AGN1C | Anti-Glial Nuclear Ab Type 1 | IFA | <1:2 |
| AGNBC | AGNA-1 Immunoblot, CSF | Immunoblot (IB) | Negative |
| AMIBC | Amphiphysin Immunoblot, CSF | IB | Negative |
| AMPCC | AMPA-R Ab CBA, CSF | CBA | Negative |
| AMPHC | Amphiphysin Ab, CSF | IFA | <1:2 |
| AMPIC | AMPA-R Ab IF Titer Assay, CSF | IFA | <1:2 |
| ANN2C | Anti-Neuronal Nuclear Ab, Type 2 | IFA | <1:2* |
| AN1BC | ANNA-1 Immunoblot, CSF | IB | Negative |
| AN2BC | ANNA-2 Immunoblot, CSF | IB | Negative |
| ANN3C | Anti-Neuronal Nuclear Ab, Type 3 | IFA | <1:2* |
| DPPCC | DPPX Ab CBA, CSF | CBA | Negative |
| DPPTC | DPPX Ab IFA Titer, CSF | IFA | <1:2 |
| 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 |
| GL1CC | mGluR1 Ab CBA, CSF | CBA | Negative |
| GL1TC | mGluR1 Ab IFA Titer, CSF | IFA | <1:2 |
| NMDIC | NMDA-R Ab IF Titer Assay, CSF | IFA | <1:2 |
| NMOTC | NMO/AQP4 FACS Titer, CSF | FACS | <1:2 |
| PC1BC | PCA-1 Immunoblot, CSF | IB | Negative |
| PCA1C | Purkinje Cell Cytoplasmic Ab Type 1 | IFA | <1:2* |
| PCA2C | Purkinje Cell Cytoplasmic Ab Type 2 | IFA | <1:2* |
| PCTBC | PCA-Tr Immunoblot, CSF | IB | Negative |
*Neuron-restricted patterns of IgG staining that do not fulfill criteria for ANNA-1, ANNA-2, ANNA-3, PCA-1, PCA-2, or PCA-Tr may be reported as "unclassified antineuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."
Interpretation Provides information to assist in interpretation of the test results
This profile is consistent with an autoimmune central nervous system disorder.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Negative results do not exclude a diagnosis of an autoimmune central nervous system disorder.
Clinical Reference Recommendations for in-depth reading of a clinical nature
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