Products - Autoimmune - Neurology - Paraneoplastic neurological syndromes

Paraneoplastic neurological syndromes

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Information

Paraneoplastic neurological syndromes (PNS) are diseases of the central and peripheral nervous system that occur in direct association with tumors. However, they are not directly caused by the tumor or by its metastases, nor are they a side-effect of treatment with cytostatic drugs or radiotherapy.

Depending on the type of tumor, tumor cells express antigens such as amphiphysin, CV2/CRMP5, PNMA2 (Ma2/Ta), Ri, Yo, Hu, ZIC4 or Tr (DNER), which can induce the formation of specific autoantibodies. These autoantibodies bind to the respective antigens found in nerve tissue and can thus cause neurological disorders.

Diagnostic Guidelines

Approx. 15% of patients with malignant diseases, especially lung tumors, have PNS.
New diagnostic criteria for PNS have recently been established (Graus et al., 2020). What is new is the subdivision into high-risk phenotypes (encephalomyelitis, limbic encephalitis, rapidly progressive cerebellar syndrome, opsoclonus-myoclonus syndrome, sensory neuronopathy, gastrointestinal pseudoobstruction (enteric neuropathy), Lambert-Eaton myasthenic syndrome) and intermediate-risk phenotypes for PNS (including encephalitis, anti-NMDA receptor encephalitis, brainstem encephalitis, Morvan syndrome, isolated myelopathy, stiff-person syndrome, polyradiculoneuropathy).

The term “onconeural antibodies” has been replaced by “high-risk antibodies” for cancer (tumor in >70% of patients with these antibodies) and “intermediate-risk antibodies” for cancer (tumor in 30%–70% of patients with these antibodies). The guidelines classify three levels of evidence for PNS: “definite”, “probable” and “possible”. Each level can be identified using the PNS-care score, which assesses the clinical phenotype, the antibody type, the presence or absence of cancer and the follow-up time. With the exception of opsoclonus-myoclonus syndrome, the diagnosis of “definite PNS” requires the presence of high- or intermediate-risk antibodies.

Neuronal antibodies have been established as useful biomarkers and their detection is of paramount importance in the diagnosis of PNS. For a reliable diagnosis, the guidelines recommend that autoantibodies in PNS should always be determined using two independent methods.

Gold standard methods include indirect immunofluorescence (IFA or immunohistochemistry) based on brain sections, for example, using specific BIOCHIP Mosaics from EUROIMMUN for neurology. For confirmation, immunoblots (e.g. EUROLINE profiles) for the monospecific detection of autoantibodies, mainly against intracellular antigens, or cell-based assays (CBAs) for the detection of antibodies against cell surface or synaptic proteins are used. Neuronal autoantibodies should be tested in both serum and cerebrospinal fluid.

Paraneoplastic neurological syndromes products

For Research Use Only. Not For Use In Diagnostic Procedures.
The individual product regulatory statements may vary, please refer to the instructions for use for more information.

wdt_ID Method Parameter Substrate Species/ Antigen
626 IFA brain: grey and white matter,
Purkinje cell cytoplasm (Yo),
Hu and Ri antigen
CV2, Ma, amphiphysin
cerebellum monkey
627 EUROLINE positive control serum: IgG, human, 100x concentrated
for DL 1111-1 G, DL 1111-2 G, DL 1111-4 G and DL 1111-7 G
628 IFA Neurology Mosaic 1 EUROPattern
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
3 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue
monkey
monkey
monkey
629 IFA Neurology Mosaic 1
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
3 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue
monkey
monkey
monkey
630 IFA Neurology Mosaic14
cerebellum antigens
non-medullated nerves
2 BIOCHIPs per field:
cerebellum
non-medullated nerves
monkey
monkey
631 IFA Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
cell nuclei (ANA)
cerebellum
nerves
intestinal tissue
HEp-2 cells
(4 BIOCHIPs per field)
monkey
monkey
monkey
human
632 IFA Neurology Mosaic 17
Yo, Hu, Ri, CV2, Ma, amphiphysin, NMO
cerebrum antigens, NMO
NMO, optic nerve antigens
aquaporin-4
5 BIOCHIPs per field
cerebellum
cerebrum
optic nerve
transfected cells
control transfection
monkey
monkey
monkey
EU 90
EU 90
633 IFA Neurology Mosaic 2
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
2 BIOCHIPs per field:
cerebellum
nerves
monkey
monkey
634 EUROLINE Neuronal Antigens Profile 2
(amphiphysin, CV2, PNMA2
(Ma-2/Ta), Ri, Yo, Hu separately)
EUROLINE
635 EUROLINE Neuronal Antigens Profile PLUS RST
(amphiphysin, CV2, PNMA2
(Ma-2/Ta), Ri, Yo, Hu,
recoverin, SOX1, titin separately)
EUROLINE
636 EUROLINE Neuronal Antigens Profile
SOX1, titin
EUROLINE
638 EUROLINE positive control serum: IgG, human, 100x concentrated
for DL 1111-6 G
639 EUROLINE Paraneoplastic Neurologic Syndromes - 12 Ag
(amphiphysin, CV2, PNMA2 (Ma-2/Ta), Ri, Yo, Hu,
recoverin, SOX1, titin, Zic4, GAD65, Tr (DNER) separately)
EUROLINE
640 IFA Neurology Mosaic 8 EUROPattern
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
pancreas islets
4 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue
pancreas
monkey
monkey
monkey
monkey
641 IFA Neurology Mosaic 8
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
pancreas islets
4 BIOCHIPs per field
cerebellum
nerves
intestinal tissue
pancreas
monkey
monkey
monkey
monkey
642 IFA antibodies against Delta and Notch-like epidermal growth factor-related receptor
(DNER CBA control)
643 IFA Delta and Notch-like epidermal
growth factor-related receptor (DNER)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
644 IFA antibodies against Purkinje cell cytoplasm
(Yo ab control)
645 IFA Purkinje Cell Mosaic 1
Yo/CDR2
DNER
ITPR1
CARP
4 BIOCHIPs per field:
transfected cells
transfected cells
transfected cells
transfected cells
EU 90
EU 90
EU 90
EU 90
646 IFA antibodies against neuronal nuclei: Ri antigen
(Ri ab control)
647 IFA antibodies against neuronal nuclei: Hu antigen
(Hu ab control)
648 IFA zinc finger protein ZIC4 transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
Method Parameter Substrate Species/ Antigen
Contact our Autoimmune Product Team for further details and information
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