DIAGNOSTIC TEST PANEL 199
Paraneoplastic Syndrome (IIF and Immunoblot)
Diagnostic test panel for antibodies against GAD, Hu, Amphiphysin, Recoverin, SOX1, Yo, Zic4, Ri, Tr, Ma, CV2/CRMP5, and Titin. For suspicion of paraneoplastic syndrome, autoimmune encephalitis, and neuropathy.
Indication
Suspicion of paraneoplastic syndrome, autoimmune encephalitis and neuropathy.
Sample material
Serum
- Minim. volume: 0,5 mL
CSF
- Minim. volume: 2,0 mL
Transport
Within Sweden
- room temperature
International
- cold
Clinical background
Paraneoplastic syndromes occur secondary to malignancy. All parts of the nervous system can be involved and the symptoms are often subacute, such as encephalopathy, cerebellar degeneration, and polyneuropathy. In most of the paraneoplastic syndromes, the neurological symptoms appear before the cancer has been identified. Identification of paraneoplastic antibodies should direct the search to an underlying cancer.
Small cell lung cancer (SCLC) is the most common underlying cause, followed by breast and ovarian tumors, but many different malignancies may be associated with paraneoplastic syndromes. Symptom onset is usually sub-acute and can precede the cancer diagnosis by up to 5 years.
Generally, immunomodulatory drugs used in autoimmunity are not effective in these cases and instead, treatment of the underlying cancer is most important. If the cancer can be successfully treated the expression of the intracellular antigen and activity of the immune system decrease and the neurological symptoms may improve.
Antibodies against Amphiphysin
occur in patients with paraneoplastic variant Stiff person syndrome but can also be seen in autoimmune encephalitis. Antibodies against Amphiphysin are associated with breast cancer and small cell lung cancer (SCLC).Antibodies against CV2/CRMP5
are primarily associated with SCLC, but also occurs with thymoma, and other tumor types. Clinical symptoms may be chorea, sensorimotor neuropathy, limbic encephalitis, and cerebellar degeneration.Antibodies against Hu (ANNA-1)
occur in paraneoplastic subacute sensory neuronopathy and/or paraneoplastic encephalomyelitis. The antibody prevalence is strongly associated to SCLC but can also be seen in other tumor types (e.g. breast cancer, Hodgkin's lymphoma, melanoma, ovarian cancer, testicular cancer, thymoma).Antibodies against Ma (Ma-2/Ta)
are associated with paraneoplastic cerebellar degeneration, limbic encephalitis, and brain stem encephalitis. These antibodies can occur in many different forms of cancer, such as breast cancer, testis cancer and SCLC.Antibodies against Ri (Nova 1/ANNA-2)
occur less often than antibodies against Hu, but can be seen in paraneoplastic sensory neuronopathy, cerebellar ataxia and/or paraneoplastic encephalomyelitis. Prevalence is strongly associated to SCLC.Antibodies against Yo (anti-PCA-1)
are strongly associated with subacute cerebral ataxia and ovarian or breast cancer. Neuropathy may also occur.Antibodies against GAD
occur in 60% of patients with Stiff Person Syndrome and can then be detected in both serum and CSF. Anti-GAD antibodies may also be associated with diabetes, limbic encephalitis, epilepsy, and cerebellar ataxia.Antibodies against Recoverin
occur almost exclusively in SCLC with paraneoplastic retinopathy.Antibodies against SOX1 (AGNA)
are unusual but have been reported to occur in paraneoplastic neuropathy, Lambert-Eaton syndrome, and in unclear forms of neuropathy and ataxia. SOX1 antibodies are often associated with antibodies against VGCC, Hu, CV2/CRMP5 or amphiphysin. The most common cancer form associated with SOX1 is SCLC, but other lung cancer forms have also been reported.Antibodies against Titin
can be detected in patients with myasthenia gravis and are associated with thymoma.Antibodies against Trotter antigen (Tr/DNER)
can be seen in breast or ovarian cancer, SCLC, thymoma, Hodgkin's lymphoma and are associated with paraneoplastic encephalomyelitis or cerebellar degeneration.Antibodies against Zic4
may occur with paraneoplastic neurological disease associated with SCLC with or without neurological symptoms. Most Zic4 positive patients also produce antibodies against Hu and CV2. Patients who have only Zic4 antibodies usually display symptom of cerebellar degeneration. Patients with a combination of Zic4 and other autoantibodies suffer from a broader spectrum of symptoms.
References
Read "Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes" (open access)
Tests included in panel
- 089. GAD Antibodies
- 220. Hu (ANNA-1) Antibodies (IgG)
- 730. Amphiphysin Antibodies (IgG)
- 736. Recoverin Antibodies (IgG)
- 737. SOX1 Antibodies (IgG)
- 740. Yo (Purkinje cell) Antibodies (IgG)
- 742. Zic4 Antibodies (IgG)
- 750. Ri (Nova 1/ ANNA-2) Antibodies (IgG)
- 760. Tr Antibodies (IgG)
- 790. Ma (Ma-2/Ta) Antibodies (IgG)
- 870. CV2/CRMP5 Antibodies (IgG)
- 965. Titin Antibodies (IgG)
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How to order
This test panel is available worldwide for hospitals, clinics, and physicians.
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Print and complete the request form
Download the request form. Clearly state the name and phone number of the referring hospital, clinic, or physician. -
Prepare your samples
Serum: At least 0.5 mL serum (plain serum tubes without additives).
CSF: At least 2 mL CSF (polypropylene tubes).
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Send samples and request form
Within Sweden
Samples can be sent at room temperature to:
Envelopes and smaller boxes:
Wieslab AB, Box 50117, 20211 Malmö, Sweden
Larger boxes and frozen samples:
Wieslab AB, Lundavägen 151, 21224 Malmö, Sweden
International
Send samples cold to:
Wieslab AB, Lundavägen 151, 21224 Malmö, Sweden
Last updated: 2025-08-18