Autoimmune encephalitis

Clinical information

Patients with autoimmune encephalopathies exhibit autoantibodies against neuronal cell surface antigens. The antibodies are directed against glutamate receptors (type NMDA or type AMPA), GABAB receptors, DPPX, voltage-gated potassium channels (VGKC) or VGKC-associated proteins (LGI1, CASPR2, TAG-1/ contactin-2). Since these antigens play a direct or indirect role in synaptic signal transduction, the associated autoimmunities manifest with seizures and neuropsychiatric symptoms. The resulting conditions include special forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. These severe, potentially lethal syndromes can have a non-paraneoplastic or paraneoplastic aetiology. The frequency of underlying tumours ranges from 10 to 70%, depending on the type of antibody. The antibodies most likely play a causal role in the pathogenesis. Since appropriate therapy (immunomodulatory intervention, tumour resection) results in considerable regression of symptoms in most patients, early diagnosis is important for a favourable prognosis.

Diagnostics

The diagnosis of autoimmune encephalitides is generally based on a combination of the characteristic clinical picture, supporting findings from brain MRT, EEG and CSF analysis if necessary, and antibody determination in serum/CSF. Monospecific recombinant assays are the method of choice for serological diagnostics and can be combined with conventional immunohistochemical detection procedures. The following conditions must be excluded by differential diagnostics: infectious encephalitides (especially HSV), other autoimmune aetiologies (e.g. limbic encephalitis with autoantibodies against Hu, Ma2, CV2, amphiphysin) and clinically similar diseases of the central and/or peripheral nervous system. A diagnostic discrimination from atypical encephalitides should also be taken into consideration. It should be taken into account that overlap syndromes and combinations of different syndromes can also occur. When a positive serological result is obtained, a comprehensive tumour investigation should be undertaken.

Techniques

Method
Parameter
Substrate
Species
IIFT
antibodies against glutamate receptor (type NMDA)
IIFT
RC-IIFT Neurology Mosaic 14A
glutamate receptor (type NMDA)
contactin-associated protein 2 (CASPR2)
IgLON family member 5 (IgLON5)
dipeptidyl aminopeptidase-like protein 6 (DPPX)
leucine-rich glioma-inactivated protein 1 (LGI1)
GABA B receptor
6 BIOCHIPs per field:
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells

EU 90
EU 90
EU 90
EU 90
EU 90
EU 90
IIFT
glutamate receptor (type NMDA)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
glutamate receptor (type NMDA) EUROPattern
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
Autoimmune Encephalitis Mosaic 6
glutamate receptor (type NMDA)
contactin-associated protein 2 (CASPR2)
glutamate receptors (type AMPA1/2)
leucine-rich glioma-inactivated protein 1 (LGI1)
dipeptidyl aminopeptidase-like protein 6 (DPPX)
GABA B receptor
6 BIOCHIPs per field:
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells

EU 90
EU 90
EU 90
EU 90
EU 90
EU 90
IIFT
Autoimmune Encephalitis Mosaic 6 EUROPattern
glutamate receptor (type NMDA)
contactin-associated protein 2 (CASPR2)
glutamate receptors (type AMPA1/2)
leucine-rich glioma-inactivated protein 1 (LGI1)
dipeptidyl aminopeptidase-like protein 6 (DPPX)
GABA B receptor
6 BIOCHIPs per field:
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells
transfected cells

EU 90
EU 90
EU 90
EU 90
EU 90
EU 90
IIFT
Glutamate Receptor (Type AMPA1/2)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
antibodies against GABA B receptor
IIFT
GABA B receptor
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
antibodies against dipeptidyl aminopeptidase-like protein 6
(DPPX ab control)
IIFT
dipeptidyl aminopeptidase-like protein 6
(DPPX)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
dipeptidyl aminopeptidase-like protein 6
(DPPX) EUROPattern
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
antibodies against IgLON family member 5 (IgLON5)
IIFT
IgLON family member 5 (IgLON5)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
Anti-VGKC-Ass. Proteins Mosaic 1
leucine-rich glioma-inact. prot. 1 (LGI1)
contactin-associated protein 2 (CASPR2)
3 BIOCHIPs per field:
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
IIFT
antibodies against leucine-rich glioma-inactivated protein 1
(LGI1 ab control)
IIFT
Anti-VGKC-Ass. Proteins Mosaic 1 EUROPattern
leucine-rich glioma-inact. prot. 1 (LGI1)
contactin-associated protein 2 (CASPR2)
3 BIOCHIPs per field:
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
IIFT
antibodies against contactin-associated protein 2
(CASPR2 ab control)
IIFT
contactin-associated protein 2 (CASPR2)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
leucine-rich glioma-inactivated protein 1 (LGI1)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
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