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Paraneoplastic neurological syndromes

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Disease information

Paraneoplastic neurological syndromes (PNS) are diseases of the nervous system, both central and peripheral, that occur in relation to a developing tumor. However, they are not a direct product of the tumor or are caused by its metastasis. Instead, they bud from the antigens expressed by the tumor which then induce the formation of autoantibodies. Most malignant diseases (around 15%) especially a lung tumor, come with paraneoplastic neurological syndrome (PNS).

Tumor cells express a variety of antigens. These include:

  • Amphiphysin
  • CV2/CRMP5
  • PMNA2
  • Tr (DNER)

These antigens induce the formation of autoantibodies. Consequently, the antibodies then localize themselves to these antigens in the nervous tissue and cause neurological manifestations.

Two types of nomenclature are used to express the PNS-specific antibodies. One gets its basis from the first two letters of the patient’s name (such as Yo for Young and Ma for Margaret). The other stems from the immunohistochemical staining properties of the antibodies. We, however use the former since it’s independent of the test procedure.

Diagnostic Guidelines

According to the guidelines by European Network of paraneoplastic neurological diseases (PNS Euronetwork), the diagnostic criteria for these diseases is based around two foci: a definitive diagnosis and a possible diagnosis for paraneoplastic neurological syndromes. The diagnostic procedure involves both serological testing and clinical evaluation of the patients.

Serological testing

Serological diagnosis is based around the detection of antigen-specific antibodies using two unrelated methods. Techniques used are mostly various line blots (EUROLINE) and immunofluorescence test with different/special BIOCHIP mosaics for neurology. The results are compared for validity and reliability.

The absolute diagnosis is based upon the correlation of these tests with each other and with the clinical picture of the patients.

Paraneoplastic neurological syndromes products

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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