Voltage-gated potassium channel associated proteins

VGKC antibodies were described few years ago and it has been established that the pathogenic targets are against the complexing proteins, mainly LGI1 and Caspr2 (see below). Testing for VGKC antibodies are no longer recommended to done in isolation, and the more specific LGI1 or Caspr2 antibodies should be checked, and correlated clinically.

LGI-1 (Leucine-rich glioma inactivated protein 1) antibody

Tumours: Thymoma

Syndrome: Limbic encephalitis with LE with Faciobrachial-dystonic seizures

CASPR2 (Contactin-associated protein 2)

Tumours: Thymoma

Syndrome: Limbic encephalitis, cerebellar ataxia, neuromyotonia, Morvan syndrome

DPPX (Dipeptidyl-Peptidase–Like Protein-6)

Tumours: Thymoma

Syndrome: Limbic encephalitis with psychiatric symptoms

GABARB1 (GABA receptors 1)

Tumours: B cell neoplasms

Syndrome: Gastrointestinal symptoms, weight loss, cognitive deficit CNS hyperexcitability

Diagnosis and treatment of autoimmune encephalitis

There are several antibodies which can give rise to autoimmune encephalitis (see diagram). In our lab this is done as a panel, helping to identify the antigenic target using a single assay. Antibodies are best identified by a combination of techniques (immunohistochemistry and immunofluorescence) to increase sensitivity and specificity. Patients often need immunosuppression, plasma exchange and in refractory patients B-cell depleting therapies like Rituximab (especially in NMDAR encephalitis).

Summary of Encephalitic antibodies