Paraneoplastic and autoimmune encephalitis when to consider and how to diagnose it

Abstract

The field of limbic paraneoplastic and autoimmune encephalitis has been greatly enhanced during the last decade by the discovery of neuronal cell-surface directed autoimmunity in the serum and/or CSF of limbic encephalitis patients. The main antibodies target the glutamate NMDA receptor and the voltage-gated potassium channel (VGKC), now known to be mainly LGI-1 and CASPR2 proteins. The recent discovery that a number of acute encephalitis of unknown cause are due to anti-NMDAR or anti-VGKC encephalitis has induced a great interest in these pathologies from critical care physicians because they display specific clinical patterns and can be cured by surgical removal of a tumour, if applicable, and immunotherapy. Neurological paraneoplastic syndromes, classic limbic encephalitis, cellsurface antibody-associated encephalitis are described and the most recent literature is reviewed.