| dc.description.abstract | Autoimmune encephalitis associated with antibodies to neuronal cell surface or synaptic proteins was first described in 2001. They include anti-NMDAR, anti-VGKC-complex associated proteins (primarily LGI1 and CASPAR2), anti-AMPAR, anti-GABABR, anti-GlyR, anti-DPPX, anti-mGluR5, anti-D2R and anti-GABAAR encephalitis. Their presentations are rather similar and usually consist of a combination of neuropsychiatric symptoms, seizures and movement disorders. However, some clinical distinctions can often be made for some of the subtypes, like autonomic dysfunction and hypoventilation in anti-NMDAR encephalitis and severe hyponatremia and fasciobrachial dystonic seizures in anti-LGI1 encephalitis. This spectrum of autoimmune neurological diseases can be paraneoplastic, and/or mimic infectious encephalitis or other CNS diseases. The diagnostics should include comprehensive patient history, testing for autoantibodies in CFS and serum and cerebral imaging (MRI) and EEG. First line treatment consists of tumour removal (if present), intravenous corticosteroids, immunoglobulins (IVIg) and/or plasmapheresis. Eventually combined with second line treatment with biological cytostatic medication (rituximab and/or cyclophosphamide) and long-term treatment with oral corticosteroids, immunosuppression (mycophenolate mofetil) and/or cytostatic treatment (azathioprine). Despite the dramatic clinical picture for the subset, prompt recognition and treatment with immunotherapy results in full recovery or mild sequelae in 3 out of 4 patients. This review will focus on the clinical recognition, treatment and follow-up on some of the main types of extracellular autoimmune encephalitis. | nor |