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HelpTest Code PC1TS Purkinje Cell Cytoplasmic Antibody Type 1 (PCA-1) Titer, Serum
Specimen Required
Only orderable as a reflex. For further information see:
PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
Useful For
Identifying female patients whose subacute cerebellar degeneration or peripheral neuropathy is due to a remote (autoimmune) effect of gynecologic or breast carcinoma
Reporting an end titer result from serum specimens
Testing Algorithm
If the indirect immunofluorescence pattern suggests Purkinje cell cytoplasmic antibody type 1 (PCA-1), then this test will be performed at an additional charge.
Method Name
Only orderable as a reflex. For further information see:
PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
Indirect Immunofluorescence Assay (IFA)
Reporting Name
PCA-1 Titer, SSpecimen Type
SerumSpecimen Minimum Volume
0.6 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 28 days | |
| Frozen | 28 days | ||
| Ambient | 72 hours |
Reject Due To
| Gross hemolysis | Reject |
| Gross lipemia | Reject |
| Gross icterus | Reject |
Clinical Information
Purkinje cell antibody type 1 (PCA-1), also known as anti-Yo, binds to Purkinje cell cytoplasm in a characteristic pattern by indirect immunofluorescence. It is found in the serum, and usually cerebrospinal fluid, of patients with paraneoplastic cerebellar degeneration associated with gynecological or breast carcinoma. It is also found in some patients with sensory, sensorimotor neuropathy, or motor neuropathy with gynecologic cancer. Almost all (99%) seropositive patients are women.
Reference Values
Only orderable as a reflex. For further information see:
PAVAL / Paraneoplastic, Autoantibody Evaluation, Serum
ENS2 / Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum
MDS2 / Movement Disorder, Autoimmune/Paraneoplastic Evaluation, Serum
MAS1 / Myelopathy, Autoimmune/Paraneoplastic Evaluation, Serum
AIAES / Axonal Neuropathy, Autoimmune/Paraneoplastic Evaluation, Serum
<1:240
Neuron-restricted patterns of IgG staining that do not fulfill criteria for Purkinje cell cytoplasmic antibody type 1 may be reported as "unclassified antineuronal IgG." Complex patterns that include nonneuronal elements may be reported as "uninterpretable."
Interpretation
Purkinje cell antibody type 1 (PCA-1) has not been found in any healthy subject. It is rarely found in patients with neurologic diseases (including cerebellar disorders) without gynecologic or breast cancer. The ovarian cancers found in these patients are typically limited in metastatic spread and may not be detected by imaging procedures. If mammography is negative, exploratory laparotomy is advisable (as a "second look" in management of ovarian carcinoma). Breast carcinoma may coexist with a Mullerian cancer. PCA-1 is rarely found in patients with gynecologic cancer without neurologic dysfunction (<2%). PCA-1 is readily distinguished from PCA-Tr (a marker of Hodgkin lymphoma) and PCA-2 (a marker of small-cell lung carcinoma) by standardized staining criteria. PCA-1 rarely, if ever, has accompanying neuronal cytoplasmic or nuclear antibodies.
Cautions
Purkinje cell cytoplasmic antibody type 1 (PCA-1) antibody is rarely found in male patients (1%, usually with intra-abdominal adenocarcinoma) and never in patients with cerebellar ataxia associated with lung cancer.
Seven different IgG autoantibodies are currently recognized as accompaniments of paraneoplastic neurologic disorders occurring with small-cell lung carcinoma (SCLC):
-Antineuronal nuclear antibody-type 1 (ANNA-1, sometimes called anti-Hu) is found most often with sensory, autonomic, and sensorimotor neuropathies, and encephalomyeloradiculopathies in the context of SCLC.
-ANNA-2 is found most often with midbrain/brainstem encephalitis, cerebellar ataxia, myelopathy associated with breast cancer, or SCLC; peripheral neuropathy may be a presenting sign.
-ANNA-3 is found with multifocal autoimmune neurologic manifestations of aerodigestive carcinomas (usually SCLC).
-PCA-2 is found with multifocal autoimmune neurologic manifestations of SCLC.
-PCA-Tr is found in patients with cerebellar ataxia related to Hodgkin's lymphoma.
-CRMP-5-IgG is found in patients with multifocal autoimmune neurologic manifestations of SCLC or neuromuscular or encephalopathic manifestations of thymoma.
-Antiglial neuronal antibody (AGNA-1) is found in patients with multifocal autoimmune neurologic manifestations of SCLC, but particularly with Lambert-Eaton syndrome, peripheral neuropathy, limbic encephalitis, and dysautonomia.
Clinical Reference
1. Hetzel DJ, Stanhope CR, O'Neill BP, Lennon VA: Gynecologic cancer in patients with subacute cerebellar degeneration predicted by anti-Purkinje cell antibodies and limited in metastatic volume. Mayo Clin Proc. 1990 Dec;65(12):1558-1563
2. McKeon A, Tracy JA, Pittock SJ, Parisi JE, Klein CJ, Lennon VA. Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond. Arch Neurol. 2011 Oct;68(10):1282-9. doi: 10.1001/archneurol.2011.128
3. Vernino S, Lennon VA: New Purkinje cell antibody (PCA-2): Marker of lung cancer-related neurological autoimmunity. Ann Neurol. 2000 Mar;47(3):297-305
4. Yu Z, Kryzer TJ, Griesmann GE, et al: CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol 2001 Feb;49(2):146-154
5. Pittock SJ, Kryzer TJ, Lennon VA: Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol 2004 Nov;56(5):715-719
6. Horta ES, Lennon VA, Lachance DH, et al: Neural autoantibody clusters aid diagnosis of cancer. Clin Cancer Res 2014 July;20(4):3862-3869
Method Description
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)
Day(s) Performed
Monday through Sunday
Report Available
6 to 8 daysSpecimen Retention Time
4 weeksPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
86256
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PC1TS | PCA-1 Titer, S | 94350-6 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 43437 | PCA-1 Titer, S | 94350-6 |